1

ACCSAP 10 Qs & As
1- Arrhythmia …………………………… 1
2- Valvular Heart Diseases………… 131
3- Congenital Heart Diseases……. 225
4- Pericardial Diseases……………… 274
5- HTN & Hypotension……………… 320
6- Vascular Diseases…………………. 380
7- Systemic Disorders affecting
the circulation system…………. 447

Collected & Arranged by:

Dr Hani Abdullah
Dr Fatma Hadi
Dr Salem Boresa
Dr Rami Elesali
Dr Naeif Almagal

King Khalid University Hospital

Riyadh- 1/1/2020

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Collected by:
Dr Hani Abdullah
Dr Fatma Hadi
Dr Salem Boresa
Dr Rami Elesali
Dr Naeif Almagal
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 2

Arrhythmias

Question 1
1.A 67-year-old man with coronary artery disease, hypertension, and sleep apnea presented to your
office wishing to discuss rhythm control. Two years ago, he experienced his first episode of atrial
fibrillation, for which he was cardioverted and placed on metoprolol and apixaban. Two weeks ago,
he noted the recurrence of an irregular heart rate and had an electrocardiogram (Figure 1). Since
that time, he has felt an irregular heart rate consistently and his fitness tracker device confirms this.
How would you characterize this patient's current arrhythmia?

(Figure 1)

B. Atrial flutter.
C. Persistent atrial fibrillation.
D. Paroxysmal atrial fibrillation.
E. Permanent atrial fibrillation.

This patient has atrial fibrillation. For his initial episode, this would be characterized as paroxysmal
atrial fibrillation. With the current episode lasting consistently for >7 days, this would be
characterized as persistent atrial fibrillation. Longstanding persistent atrial fibrillation is defined by
atrial fibrillation for >12 months in duration. Permanent atrial fibrillation is defined by cessation of
attempts to control rhythm and acceptance of atrial fibrillation as a permanent state.
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1. The correct answer: C

Key Point
AF is currently classified as paroxysmal, persistent, long-standing persistent, and permanent.
Permanent is a circumstance where further attempts at restoration of sinus rhythm are abandoned.

Question 2
2.A 74-year-old female with hypertension presented to the clinic for evaluation of new symptoms of
palpitations. She described this sensation as a feeling of her heart skipping a beat and occasionally
racing. She did not have chest pain or discomfort, but did have dyspnea with more prolonged
episodes. Episodes are not triggered by activity or exercise and most often occur after dinner. She
has not had syncope. Episodes occur several times a month and last for a few minutes to several
hours. Her blood pressure is managed with low-dose atenolol. She has a family history of stroke.
Her electrocardiogram was obtained (Figure 1).

Which of the following is the best next step in her management?

A. Stress test.
B. Reassurance.
C. Holter monitor.
D. Increase atenolol.
E. Event monitor.

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When evaluating palpitations, the history is fundamental because it enables assessment of

both the differential diagnosis of symptoms and the possibility of structural heart disease that
put the patient at arrhythmic risk. Palpitations can be of cardiac origin (supraventricular or
ventricular ectopy or arrhythmia, valvular heart disease or cardiomyopathy), psychosomatic
(anxiety or depression), neurologic (autonomic nervous system
dysfunction/neurocardiogenic), druginduced, or due to other pathology (thyroid disorder or
anemia). Evaluation of symptomatology requires assessment of the impact of symptoms on
the patient's life and the potential of risk due to structural heart disease or arrhythmia. In this
case, because the patient has risk factors for heart disease, correlation of symptoms with a
potential arrhythmia is critical, and reassurance without further evaluation would not be
optimal. Increasing atenolol would be difficult due to baseline bradycardia. When deciding
between using a 24-hour Holter and an event monitor to correlate symptoms with a potential
arrhythmia, the frequency of symptoms is important. Overall, ambulatory event monitoring of
a duration of 2-4 weeks has been shown to provide a higher yield of diagnosis than Holter.

2. The correct answer: E

Key Point
When evaluating patients with lesser degrees of bradycardia, symptom-rhythm correlation is
important. This can be achieved with ambulatory monitoring (Holter or similar). More
prolonged monitoring with an implantable subcutaneous monitor may be needed in some
cases.

Question 3

3.A 78-year-old male with a history of coronary artery bypass presented to the emergency
department with symptoms of dizziness and palpitations. He has a history of tobacco use,
diabetes mellitus, and hypertension. His home medications include metoprolol succinate,
lisinopril, aspirin, and atorvastatin. Upon arrival at the emergency department, the patient's
blood pressure was 80/45 mm Hg with a heart rate of 180 bpm. He appeared mildly
distressed. An electrocardiogram (ECG) was obtained (Figure 1).

What is the most likely cause of this patient's arrhythmia?

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(Figure 1)

A. Abnormal automaticity,
B. Triggered activity.
C. Scar-mediated re-entry.
D. Early afterdepolarizations.
E. Delayed afterdepolarizations.

This ECG shows monomorphic ventricular tachycardia (VT). Monomorphic VT is most commonly
due to a re-entry mechanism, typically from myocardial scar. Triggered activity encompasses both
delayed and early afterdepolarization and is considered the likely mechanism for right ventricular
outflow tract (RVOT) VT. Early afterdepolarizations are the trigger for torsades de pointes in the
setting of QT prolongation. Delayed afterdepolarizations are associated with catecholaminergic
polymorphic VT. Ischemia is commonly associated with enhanced normal automaticity and abnormal
automaticity.

3. The correct answer: C

Key Point
Monomorphic ventricular tachycardia (MVT) is most commonly due to a re-entry mechanism around
an infarcted scar tissue. Less commonly, MVT is due to a single focus in patients without structural
heart disease.

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 6

Question 4
4.A 30-year-old male presented to the cardiology clinic for evaluation after his father had an episode
of syncope and was found to have a Brugada pattern on his electrocardiogram (ECG). His father's
genetic testing revealed an SCN5A gene mutation. You obtained an ECG (Figure 1).

What is the best next step in his care?

(Figure 1)

A. No further evaluation.
B. Echocardiogram.
C. Implantable cardioverter defibrillator.
D. Exercise stress ECG.
E. Genetic testing.
This patient's ECG is normal. Nevertheless, first- and second-degree relatives of patients with
Brugada syndrome and positive genetic screen are recommended to undergo genetic testing even in
the setting of a normal baseline ECG. An echocardiogram may not demonstrate structural heart
disease in a patient with isolated Brugada syndrome. Implantable cardioverter-defibrillator (ICD)
implantation is not indicated for asymptomatic Brugada syndrome patients with a family history of
sudden cardiac death alone. Although an exercise stress test may induce changes in ST segments
and can be supportive of a diagnosis of Brugada syndrome, as this patient's father (the proband)
already has an identified causative mutation, proceeding with genetic testing of the patient would be
the best next step. A negative test would effectively exclude the diagnosis of Brugada syndrome in
him.

4. The correct answer: E

Key Point
Clinical and genetic testing of probands is recommended for family screening along with clinical and
genetic testing of all first- and second-degree relatives, even if phenotype negative, to assist with
counseling.
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 7

Question 5
5.A 38-year-old male presented for evaluation of syncope. He has a history of palpitations
associated with lightheadedness. He takes no home medications. His father died suddenly at age
35. An electrocardiogram (ECG) (Figure 1) and echocardiogram (Video 1) were obtained.

In this patient, which of the following measurements is most predicative of a positive genetic screen
for his condition?

(Figure 1)
Video 1

A. Right ventricular end-diastolic dimension.

B. QTc.
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C. R-R interval.
D. Left ventricular end-diastolic dimension.
E. Septal wall thickness.

This patient has long QT syndrome (LQTS) based on his ECG and history. The probability of a
positive genetic test for LQTS is greatest when the QTc is >480 msec, especially in the setting of a
positive family history. This diagnosis is not related to the underlying heart rate. The transthoracic
echocardiogram is normal and LQTS is not associated with cardiac structural abnormalities.

5. The correct answer: B

Key Point
The success of genetic testing in LQTS depends in large part on the robustness of the clinical
diagnosis with mutations identified in 70-80% of cases when the diagnosis is certain (QTc >480
msec), but dropping significantly in borderline cases.

Question 6
6.A 60-year-old man was referred by his primary care clinician for evaluation of bradycardia. He is an
avid marathon runner who exercises regularly without limitations. The patient's past medical history
is notable for hypertension, which has been well controlled with lisinopril.

On examination, his heart rate is 46 bpm and blood pressure 112/78 mm Hg. Cardiac auscultation
reveals a bradycardic, regular rhythm. Lungs are clear to auscultation, and he has no lower
extremity edema.

An electrocardiogram was recorded (Figure 1).

Which of the following is the most appropriate next test?

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(Figure 1)

A. Treadmill stress test.

B. Pharmacologic nuclear stress test.
C. Electrophysiology study.
D. Borrelia burgdorferi antibody.
E. Transthoracic echocardiogram.

Atrioventricular (AV) block may result from increased parasympathetic nervous system output or
disease of the conduction system. Enhanced vagal tone due to athletic training, sleep, pain, or other
causes may result in slowing of the sinus rate as well as AV conduction abnormalities. More
commonly, this results in first-degree AV block or Mobitz type I second-degree AV block. Higher-
degree AV block, Mobitz type II second-degree AV block, or third degree heart block are suggestive
of underlying infranodal pathology of the conduction system, i.e., below the level of the AV node.

This patient has 2:1 atrioventricular block, which may be due to either type I or type II second-
degree AV block. Given that this patient is able to participate in regular exercise, it is likely that the
AV block is a consequence of high vagal tone rather than due to disease of the conduction
system. An exercise treadmill stress test may help differentiate whether 2:1 atrioventricular block is
due to Mobitz type I or II AV block. Exercise causes withdrawal of vagal tone and increased
sympathetic tone leading to improved atrioventricular nodal conduction. If the baseline
atrioventricular block is infranodal (Mobitz type II), the atrioventricular block will not resolve and will
likely worsen as the sinus rate increases. If the latter ensues, a pacemaker would be indicated. If
the atrioventricular block is Mobitz type I, then increased sympathetic tone would facilitate
conduction through the AV node and may lead to resolution of the AV block.

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An echocardiogram would be unlikely to elucidate the cause of his conduction abnormality.

Testing for B. burgdorferi is not indicated without other signs or symptoms of Lyme disease such as
antecedent rash, flu-like symptoms, and joint pains. Later signs of B. burgdorferi infection can
include carditis with AV block.

An electrophysiology study is not indicated without noninvasive evidence of higher-degree AV block.

A pharmacologic nuclear stress test would not be expected to yield the diagnosis, as the patient
does not have any other symptoms of ischemic heart disease.
6. The correct answer: A

Key Point
In patients with block at the AV node level, only block will improve with exercise, but with infra-Hisian
block (i.e., below the AV node), block will get worse with exercise. An exercise test should be
recommended if there is a question regarding whether the bradycardia is intrinsic (heart rate will not
improve or may even worsen with exercise). In contrast, in patients in whom bradycardia is due to
high vagal tone (athletes, for example), there will be a normal and appropriate increase in heart rate
with exercise.

Question 7
A 71-year-old woman with an ischemic cardiomyopathy and permanent atrial fibrillation (AF)
underwent biventricular implantable cardioverter-defibrillator (ICD) implantation 6 months
prior. She has New York Heart Association class III symptoms and continues to complain of
very limited functional capacity, with no change since resynchronization therapy was
initiated.

She is currently taking carvedilol 25 mg BID, lisinopril 10 mg QD, furosemide 40 mg BID,

atorvastatin 40 mg QD, aspirin 81 mg QD, and warfarin 5 mg QD. Her vital signs in the office
were blood pressure 90/60 mm Hg, heart rate 111 bpm, respiration rate 20 breaths per
minute, and oxygen saturation 94% room air. She was afebrile. Her examination was
significant for bibasilar rales, irregular heartbeat, and 1+ bilateral lower extremity edema.

Interrogation of her biventricular ICD revealed good pacing and sensing thresholds. The
battery status and lead impedances were adequate. There had been two episodes of
nonsustained ventricular tachycardia, up to 15 beats, but no shocks had been required. She
was 25% paced over the past 3 months.

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In addition to treatment of her heart failure, which of the following should be the next step in her
management?
A. Add sotalol.
B. Referral for heart transplant evaluation.
C. Atrioventricular nodal ablation.
D. Increase carvedilol dose.

The benefit of cardiac resynchronization therapy is highest with maximal biventricular pacing. This
patient is paced only 25% of the time due to AF with rapid ventricular response. Restoration of sinus
rhythm is unlikely to be successful in a patient with permanent AF. Antiarrhythmic drugs for rhythm
control, such as sotalol, should not be continued when AF becomes permanent. Her blood pressure
is tenuous, so increasing her beta-blocker is not recommended. Transplant evaluation prior to
utilizing available guideline-directed therapy is not indicated.

7.The correct answer: C

Key Point
Catheter ablation of the AV junction for nonpharmacologic rate control is highly effective, but renders
the patient pacemaker dependent and is irreversible. It is most suitable for elderly patients with
associated bradycardia or intolerance/inefficacy of rate control agents.

Question 8
8.A 40-year-old female was referred for evaluation of palpitations. She has felt skipped and irregular
beats for the past 20 years, but in the past 6 months, she has found that her exercise tolerance has
decreased due to these symptoms. She denied any chest pain or dyspnea. Prior evaluation included
a Holter monitor, which demonstrated frequent runs of nonsustained ventricular tachycardia (NSVT).
An echocardiogram was technically limited due to body habitus and poor acoustic windows.

Her family history is notable for an uncle who died of a "rhythm problem" that occurred during a
hospitalization 20 years ago.

On physical examination, her blood pressure was 123/70 mm Hg, pulse was 60 bpm, and weight
was 170 pounds (body mass index 30). The jugular venous pulse was at 6 cm, with occasional
cannon A waves present. Lung sounds were clear. Auscultation showed a single S1, single S2, and
frequent ectopic beats.

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The remainder of the examination was normal. Her 12-lead electrocardiogram (ECG) rhythm strip is
shown in Figure 1.

Which of the following is the best next step in management?

A. Cardiac catheterization.
B. Cardiac magnetic resonance imaging.
C. Implantable loop recorder.
D. Stress nuclear perfusion study.
E. Thirty-day event monitoring.

This ECG shows premature ventricular contractions (PVCs) of multiple morphologies. PVCs show a
right bundle morphology (namely positive in V1), but with different amplitudes in the late precordial
leads. The PVCs are likely arising from the left ventricle, but from different locations. In a woman
with no risk factors for coronary disease and a longstanding history of symptoms likely related to
these PVCs and NSVT, one should first consider a cardiomyopathy. As an echocardiogram was
previously regarded as suboptimal, the best next imaging study is a cardiac magnetic resonance
image (MRI). This patient’s cardiac MRI revealed left ventricular noncompaction. An event monitor or
implantable loop recorder is not helpful in this case, as the frequent PVCs are already evident on her
office evaluation.
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8. The correct answer: B

Key Point
Distinction of ventricular tachycardia in patients with or without structural heart disease is
importantsince therapeutic approaches are very different. In patients with structural heart disease,
an implantable cardioverter-defibrillator is generally recommended (secondary prevention); in
patients without structural heart disease, drug therapy or catheter ablation may be effective in
reducing or eliminating the arrhythmia.

Question 9
9.A 60-year-old male came to the emergency department with palpitations. He described a racing
heart rate and shortness of breath. His past medical history includes a myocardial infarction (MI),
dyslipidemia, hypertension, and gastroesophageal reflux disease. His current medications are
aspirin 81 mg, metoprolol succinate 25 mg, chlorthalidone 50 mg, atorvastatin 80 mg, clopidogrel 75
mg, and omeprazole 20 mg. His pulse was 150 bpm and thready. His blood pressure was 90/60 mm
Hg. On examination, he was diaphoretic and uncomfortable. An electrocardiogram was obtained
(Figure 1).

Which of the following best describes the mechanism of his arrhythmia?

(Figure 1)
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A. Re-entry.
B. Early afterdepolarizations.
C. Enhanced automaticity.
D. Delayed afterdepolarizations.
E. Decremental conduction.

Mechanisms of ventricular arrhythmia (VA) include enhanced normal automaticity, abnormal

automaticity, triggered activity induced by early or late afterdepolarizations, and re-entry. Re-entry
requires a trigger to initiate the arrhythmia and a substrate to sustain it. The trigger may be a
premature ventricular complex (PVC), which may be due to automaticity. The substrate may be
structural remodeling secondary to an underlying disease process, and often includes a scar
secondary to a prior MI or surgical repair, or patchy fibrosis in the setting of cardiomyopathy or
hypertrophy. Changes in ion channel or transporter function and/or expression and cell-to-cell
coupling secondary to the underlying pathology may alter the initiation or propagation of the cardiac
action potential. The electrophysiological substrate is dynamically influenced by a variety of factors
including cardiac metabolism, electrolytes, signaling pathways, and autonomic effects.

Re-entry around a scar is the mechanism of monomorphic VAs in patients with ischemic heart
disease.

Decremental conduction is a normal behavior of the atrioventricular node to protect the ventricle
from high atrial rates.

Both early and delayed afterdepolarizations are types of triggered activity and are not expected to be
related to scar-mediated VAs, as seen with monomorphic ventricular tachycardia.

Enhanced automaticity is usually caused by electrolyte abnormalities or ischemia; although it may be

a factor in initiating monomorphic ventricular tachycardia, it is not responsible for sustaining the
arrhythmia.

9. The correct answer: A

Key Point
Monomorphic ventricular tachycardia (MVT) is most commonly due to a re-entry mechanism around
an infarcted scar tissue. Less commonly, MVT is due to a single focus in patients without structural
heart disease.

Question 10
10.A 38-year-old woman presented to the cardiology clinic for evaluation of tachycardia. She has no
prior medical history and is on no medications. She noted intermittent palpitations over the past few
months, usually while watching television. She was referred for an ambulatory electrocardiogram
(ECG) monitor by her primary care clinician, which showed an episode of wide complex tachycardia
at 178 bpm with an inferior QRS axis and left bundle branch block appearance. A transthoracic
echocardiogram showed normal biventricular size and systolic function.
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Her resting ECG was obtained (Figure 1).

What is the best next step?

(Figure 1)

A. Implantable cardioverter-defibrillator.
B. Exercise treadmill test.
C. Cardiac magnetic resonance imaging.
D. Catheter ablation.
E. Coronary computed tomography angiogram.

The wide complex tachycardia with a left bundle appearance and inferior axis described above is
suggestive of right ventricular outflow tract (RVOT) ventricular tachycardia (VT). RVOT VT can be
seen in structurally normal hearts and has a more benign course that can respond well to catheter
ablation. However, the patient's baseline ECG shows anterior T-wave inversions, which are
concerning for arrhythmogenic right ventricular cardiomyopathy (ARVC), and therefore she needs
further testing for ARVC.

In patients with suspected ARVC, there is a class I recommendation for cardiac magnetic resonance
imaging (MRI) to establish the diagnosis and for risk stratification. ARVC is characterized by
progressive ventricular myocyte loss with replacement by fatty or fibrous tissue, and is associated
with progressive ventricular dysfunction that may involve both ventricles. Ventricular arrhythmia,
syncope, and sudden cardiac death may occur at a relatively young age, particularly in the second
and third decades of life and often during physical activity. ARVC is often due to a mutation involving
a desmosomal protein, and it usually has autosomal dominant inheritance with variable penetrance.

Catheter ablation can be pursued for patients with ARVC and recurrent VT that is refractory to beta-
blockers. This patient is on no medications, and a beta-blocker should be started prior to ablation.

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This is in contrast to RVOT VT, for which catheter ablation is an acceptable first-line therapy.
However, because the diagnosis is not yet established, this is not the correct answer.

Therefore, a coronary computed tomography (CT) angiogram or stress test is not indicated.

Implantable cardioverter-defibrillators (ICDs) may be indicated in certain patients with ARVC, but the
diagnosis has not been established yet.

10. The correct answer: C

Key Point
The important differential diagnosis in right ventricular outflow tract ventricular tachycardia (VT) is VT
occurring in the situation of arrhythmogenic right ventricular cardiomyopathy (ARVC). One important
difference is that the resting 12-lead echocardiogram (ECG) shows marked T-wave inversion in the
right precordial leads (V1-V3/4) and there may be evidence of an epsilon wave. The 12-lead ECG in
patients with normal heart VT is normal. If ARVC is suspected, computed tomography or magnetic
resonance imaging should be performed to rule out this condition, which is a genetic abnormality.

Question 11
11.An 83-year-old male was admitted to the hospital with confusion and near-syncope. He has been
profoundly fatigued for several weeks and feels as though he is going to pass out whenever he tries
to walk any significant distance. His past medical history includes a remote (>5 years ago)
myocardial infarction, hypertension, and osteoarthritis. His current medications are chlorthalidone 25
mg daily, aspirin 81 mg daily, atorvastatin 80 mg daily, and diclofenac as needed. His vital signs
were pulse 62 bpm, blood pressure 126/78 mm Hg, and respiration 12 breaths per minute. A
physical examination revealed prominent A waves, soft s1 and normally split s2, and a soft (II/VI)
holosystolic murmur at the apex. There was 1+ bilateral ankle edema. His electrocardiogram (ECG)
was recorded (Figure 1). A treadmill stress test was performed; he exercised 6 minutes of modified
Bruce protocol, reaching a peak heart rate of 112 bpm and maximum blood pressure of 180/80 mm
Hg, then stopped due to lightheadedness and fatigue.

Which of the following most likely explains his symptoms?

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(Figure 1)

A. Complete heart block.

B. Chronotropic incompetence.
C. Cardio-inhibitory syncope.
D. First-degree atrioventricular block.
E. Autonomic dysfunction.

This patient has symptoms from lack of atrioventricular (AV) synchrony, attributable to a markedly
prolonged P-R interval ("marked first-degree AV block"). The diagnosis is made from the surface
ECG.

Although there is little evidence to suggest that pacemakers improve survival in patients with isolated
first-degree AV block, it is now recognized that marked (P-R more than 300 msec) first-degree AV
block can lead to symptoms even in the absence of higher degrees of AV block. When marked first-
degree AV block for any reason causes atrial systole in close proximity to the preceding ventricular
systole and produces hemodynamic consequences usually associated with retrograde
(ventriculoatrial) conduction, including cannon A waves, signs and symptoms similar to the
pacemaker syndrome may occur. With marked first-degree AV block, atrial contraction occurs before
complete atrial filling, ventricular filling is compromised, and an increase in pulmonary capillary
wedge pressure and a decrease in cardiac output follow. Small uncontrolled trials have suggested
some symptomatic and functional improvement by pacing of patients with P-R intervals more than
0.30 sec by decreasing the time for AV conduction.

He has normal heart rate and blood pressure responses to exercise, ruling out autonomic
dysfunction and chronotropic incompetence. The ECG does not show complete heart block. His
symptoms are not classic for cardio-inhibitory syncope, as they are exertional rather than vagally
triggered.
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11. The correct answer: D

Key Point
The diagnosis and treatment of SND and AV block includes a thorough history and symptom
evaluation, as well as examination of the electrocardiographic evidence correlating these symptoms
with electrical abnormalities.

Question 12
12.A 34-year-old male presented to the emergency department with nausea and lightheadedness
over the prior hour. He has no past medical history and takes no medications. His family history is
not known because he was adopted from Brazil as a young child.

On examination, his heart rate was 200 bpm and blood pressure was 80/60 mm Hg. He was pale
and fatigued. His lungs were clear and heart was tachycardic with no murmurs appreciated. His
abdomen was soft and extremities were without edema.

An electrocardiogram (ECG) was obtained (Figure 1). He underwent cardioversion and a

subsequent ECG was obtained (Figure 2).

Which of the following is the best next step?

(Figure 1)

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(Figure 2)

A. Cardiac magnetic resonance imaging.

B. Electrophysiology study.
C. Fluorodeoxyglucose-positron emission tomography.
D. Trypanosoma cruzi antibody.
E. Treadmill stress testing.

This patient presented with sustained monomorphic ventricular tachycardia (VT) with a left bundle
branch block (LBBB) morphology and inferior axis, suggesting that it originates in the right
ventricular outflow tract (RVOT).

The differential diagnosis of VT arising from the RVOT includes idiopathic VT and arrhythmogenic
right ventricular cardiomyopathy (ARVC). Although both conditions can present with sustained VT,
the treatments and prognosis are significantly different and thus it is essential to differentiate the two
conditions.

The resting ECG of a patient with ARVC will often show an epsilon wave after the QRS and T-wave
inversions in V1-V3, as seen in the postcardioversion ECG (Figure 2). Thus, ARVC is the correct
diagnosis and can be confirmed with cardiac magnetic resonance imaging (MRI).

The resting ECG in idiopathic VT originating in the RVOT is most often normal. Electrophysiologic
studies would demonstrate the origin of the VT but would not reliably differentiate benign RVOT VT
or premature ventricular complexes (PVCs) from ARVC.

The patient has a normal QT interval after cardioversion. Although this does not exclude long QT
syndrome, where the ECG may not be abnormal unless provocative maneuvers are used, it is not
the best answer since the ECG has the classic findings of ARVC. In addition, long QT syndrome
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usually presents with polymorphic VT, not monomorphic VT, making this diagnosis unlikely. Thus,
stress testing to assess for long QT is not indicated.

Chagas disease can cause a cardiomyopathy as well as arrhythmias. Virtually all types of atrial and
ventricular arrhythmias occur, and ventricular arrhythmias and atrioventricular (AV) block frequently
occur concurrently. The severity of ventricular arrhythmias tends to correlate with the degree of left
ventricle (LV) dysfunction. However, this patient has no other evidence of heart failure consistent
with a Chagas cardiomyopathy and this condition would not cause the ECG changes seen; thus,
Trypanosoma cruzi (T. cruzi) antibody testing is not the best answer.

Cardiac sarcoidosis (CS) may present with ventricular arrhythmias, sustained or nonsustained
ventricular tachycardia, and ventricular premature beats (VPBs), which are the second most
common clinical presentation of CS, occurring in approximately 30% of cases. Although isolated CS
can occur in 25% of cases, the baseline ECG changes cannot be explained by CS and thus
fluorodeoxyglucose-positron emission tomography (FDG-PET) is not indicated.

12. The correct answer: A

Key Point
The important differential diagnosis in right ventricular outflow tract ventricular tachycardia (VT) is VT
occurring in the situation of arrhythmogenic right ventricular cardiomyopathy (ARVC). One important
difference is that the resting 12-lead echocardiogram (ECG) shows marked T-wave inversion in the
right precordial leads (V1-V3/4) and there may be evidence of an epsilon wave. The 12-lead ECG in
patients with normal heart VT is normal. If ARVC is suspected, computed tomography or magnetic
resonance imaging should be performed to rule out this condition, which is a genetic abnormality.

Question 13
13.An 18-year-old male presented to the clinic referred by his primary care clinician. He denied any
past cardiac issues and has no history of syncope. His electrocardiogram (ECG) showed a pattern of
pseudo right bundle branch block with ST elevation and T-wave inversion in leads V1 and V2. You
interpret this ECG as a type I pattern of Brugada syndrome. His oldest brother died suddenly when
he was 25 years old, and no ECGs of his brother are available.

Which of the following is the most appropriate next step?

A. Implant implantable cardioverter-defibrillator.
B. Provocative testing with sodium channel blockade.
C. Continued clinical follow-up.
D. Advise to avoid strenuous activity.
E. Implant loop recorder.

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The patient has a type 1 Brugada syndrome ECG pattern (Figure 1). An older brother who died
suddenly does not increase his risk for sudden death, and because the patient is asymptomatic, he
does not meet criteria for implantable cardioverter-defibrillator (ICD) implantation. The utility of
electrophysiologic study for treatment decision remains controversial. Provocative testing with
sodium channel blockers is not indicated in a type 1 ECG pattern. There is no need for this patient to
refrain from physical activity. At this point, the patient needs to be followed and advised to avoid
drugs with sodium channel blocking activity (see www.brugadadrugs.org).

(Figure 1)
Reproduced with permission from Mizusawa Y, Wilde AA. Brugada syndrome. Circ Arrhythm
Electrophysiol 2012;5:606-16.

13. The correct answer: C

Key Point
Use of a sodium channel-blocking agent (flecainide or ajmaline) can “unmask” BrS and can assist in
the diagnosis of borderline cases. Recording leads V1 and V2 in second and third intercostal space
can also assist in the diagnosis.

Question 14
14.A 34-year-old male presented on Wednesday morning to the emergency department after a
weekend of heavy drinking. He felt his heart racing when he woke up on Saturday morning. The
sensation has persisted. On assessment, he was in an irregularly irregular rhythm with otherwise
normal vital signs. He has no history of hypertension, diabetes mellitus, stroke, or vascular disease.
He was anticoagulated with intravenous unfractionated heparin. He underwent tranesophageal
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echocardiography followed by direct-current cardioversion. The left atrial appendage was normal in
size and there was no spontaneous echocardiogram contrast seen. The left ventricular ejection
fraction was normal and there was trace mitral regurgitation.

Which of the following is an appropriate anticoagulation strategy upon discharge?

A. Enoxaparin 1 mg/kg for 5 days.
B. Aspirin 81 mg daily for 1 month.
C. No additional therapy.
D. Warfarin with international normalized ratio (INR) goal 2.0-3.0 for 1 month.
E. Apixaban 5 mg BID for 1 month.

This is a patient with acute onset of atrial fibrillation, likely related to alcohol. The duration of atrial
fibrillation is >48 hours, therefore cardioversion with transesophageal echocardiography (TEE)
guidance is recommended (alternatively 3 weeks of anticoagulation prior to cardioversion may be
considered). The patient has nonvalvular atrial fibrillation and normal left ventricular (LV) systolic
function. The CHADS2VASC score is zero. After cardioversion for atrial fibrillation lasting >48 hours,
guidelines recommend oral anticoagulation for at least 4 weeks; thus, apixaban for 1 month is the
best answer. It would not be appropriate to withhold anticoagulation in this patient. Aspirin offers
inferior thromboembolic prophylaxis and is not the correct answer. Low molecular weight heparin for
5 days as a bridge to a therapeutic international normalized ratio (INR) on warfarin could be
combined to provide safe anticoagulation, but either medication alone would be inadequate for
thromboembolic prophylaxis. Long-term anticoagulation use is determined by aggregate stroke risk
using CHADS2 or CHADS2VASC. In this case, with a CHADS2VASC score of 0, long-term
anticoagulation is not indicated.

14.The correct answer: E

Key Point
Because of the risk of cardioembolic stroke associated with cardioversion, anticoagulation is
recommended independent of the CHA2DS2-VASc score in patients in whom AF has lasted >48
hours or if there is uncertainty regarding onset time.

Question 15
15.A 56-year-old woman with a nonischemic cardiomyopathy with ejection fraction 20%, status
postcardiac resynchronization therapy-defibrillator 6 months ago, presented to your office with a 2-

week history of progressive dyspnea on exertion. She denied any medication or dietary
nonadherence.
Her medications include furosemide 40 mg twice daily, carvedilol 6.25 mg twice daily,
sacubitril/valsartan 24/26 mg twice daily, and spironolactone 12.5 mg daily.

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On examination, her heart rate was 88 bpm, blood pressure was 98/60 mm Hg, jugular venous pulse
was 12 cm H20, lungs were clear, and heart was regular with a soft S3 gallop. Her extremities were
warm with trace edema.

An electrocardiogram (ECG) was recorded (Figure 1). The chest X-ray showed mild pulmonary
vascular congestion.

What is the best next step?

(Figure 1)
Reproduced with permission from
https://upload.wikimedia.org/wikipedia/commons/f/ff/Duelchamber.JPG. Accessed 05/14/2018.

A. Complete blood count.

B. Ventilation-perfusion scan.
C. N-terminal pro–B-type natriuretic peptide.
D. Device interrogation.
E. Echocardiogram.

This patient has decompensated heart failure despite optimal guideline-directed medical therapy and
recent cardiac resynchronization therapy-defibrillator (CRT-D) placement. There are a number of
typical causes of heart failure decompensation, including medication and dietary nonadherence,
ischemia, thyroid disease, arrhythmias, and infection. However, this patient's ECG shows a left
bundle branch block pattern, consistent with RV only pacing. Thus, the correct answer is device
interrogation to confirm this finding. On device interrogation, one would specifically assess for LV
pacing and capture.

An echocardiogram is not indicated, as her ejection fraction is already severely reduced and physical
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examination discloses no new anticipated changes. A ventilation-perfusion scan is not indicated, as

her shortness of breath appears due to decompensated heart failure and there is no suggestion of
predisposing factors for pulmonary embolism. N-terminal pro–B-type natriuretic peptide (NT-
proBNP) will undoubtedly be elevated, as she has decompensated heart failure, and will not aid in
determining the cause of her decompensation. She may be anemic, but this is not the best next
step, as nothing in the history or physical points to this as a likely diagnosis.

15. The correct answer: D

Key Point
"CRT response is dependent on >90% biventricular pacing. Causes of nonresponse to CRT through
diminished pacing include atrial fibrillation with rapid conduction, inappropriate device programming,
and frequent ventricular ectopy.
CRT non-response can also be due to loss of LV lead capture or poor LV lead position.

Question 16
16.You were asked to see a 71-year-old male for preoperative evaluation prior to craniotomy. He
presented with confusion 1 month after falling and striking his head. Due to the confusion, he has not
taken any of his medications in 4 days. Physical examination demonstrated a blood pressure of
124/72 mm Hg, clear lung sounds, and a regular rhythm. An electrocardiogram was obtained (Figure
1). A computed tomography scan demonstrated a chronic subdural hematoma.

Which of the following is the anatomic site of the arrhythmia on his electrocardiogram?

(Figure 1)

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A. Cavotricuspid isthmus.
B. Pulmonary vein.
C. Fossa ovalis.
D. Crista terminalis.
E. Bachmann's bundle.

The patient is in rate-controlled atrial flutter. Typical atrial flutter is characterized by a "saw-tooth"
pattern in the inferior (II, III, aVF) leads and a positive flutter wave in lead V1, reflecting the macro-
reentrant circuit, which includes the isthmus between the inferior vena cava and the tricuspid valve
(Figure 2). This distinction is important because cavotricuspid isthmus–dependent flutter can be
ablated with >90% success.

Atypical right-sided atrial flutter may originate from the fossa ovalis or superior vena cava and would
not produce the same pattern of saw-tooth waves in inferior leads and positive flutter waves in
V1 as cavotricuspid isthmus–dependent flutter.

Islands of pacemaker cells in pulmonary veins are responsible for atrial fibrillation.

The crista terminalis can be responsible for atrial arrhythmias by initiating ectopic atrial beats.
Ectopic rhythms originating from the crista terminalis have the same electrocardiogram appearance
as normal sinus P waves due to the location of the crista terminalis near the sinus node.

Bachmann's bundle is the electrical connection between the right and left atria.

(Figure 2)
Legend: AAo = ascending aorta; CS/ThV = coronary sinus and thebesian valve; CT = crista
terminalis; CTI = cavotricuspid isthmus; ER/EV = Eustachian ridge and valve; IVC = inferior vena
cava; OF = oval fossa; RAA = right atrium appendage; RCA = right coronary artery; RV = right
ventricle; SI = septal isthmus; STV = tricuspid valve; SVC = superior vena cava; TT = tendon of
Todaro.
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16. The correct answer: A

Key Point
AFL is a macro–re-entrant circuit most commonly within the RA and is sustained through a region of
slow conduction along a channel or “isthmus” between the IVC and the tricuspid valve, the so called
“cavotricuspid isthmus.” Due to the critical dependence of the arrhythmia on conduction through this
region, the rhythm is classified a “cavotricuspid isthmus-dependent” or CTI-dependent AFL.

Question 17
17. You supervised a treadmill stress test for a 36-year-old male with palpitations and a prior resting
electrocardiogram (ECG) (Figure 1). He has no other medical problems and takes no medications;
he has no known drug allergies. Six minutes into the Bruce protocol, he reported palpitations and
another ECG was obtained (Figure 2). After stopping the treadmill and placing him on the stretcher,
the rhythm continued. He was alert and said the palpitations were just like what he feels when he
exercises at the gym. His pulse was 180 bpm and irregular, and his blood pressure was 104/60 mm
Hg. Vagal maneuvers did not change the rhythm.
Which of the following is the best next step?

(Figure 1)

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(Figure 2)

A. Metoprolol.
B. Lidocaine.
C. Amiodarone.
D. Magnesium.
E. Procainamide.

This patient with Wolff-Parkinson-White syndrome has pre-excited atrial fibrillation. Prompt treatment
with intravenous procainamide, intravenous ibutilide, or synchronized cardioversion is critical to
prevent deterioration to ventricular fibrillation; thus, procainamide is the best answer choice.

Agents that slow conduction through the atrioventricular node (such as beta-blockers, calcium
channel blockers, or amiodarone) can lead to preferential conduction along the accessory pathway
and may precipitate ventricular fibrillation.

Magnesium can be a useful adjunctive therapy for polymorphic ventricular tachycardia associated
with long QT syndrome. There are case series of transient slowing of accessory pathway conduction
after intravenous magnesium administration in sinus rhythm, but magnesium does not have an
established role in managing pre-excited atrial fibrillation.

Lidocaine, a class Ic antiarrhythmic drug, is useful for substrate-mediated (monomorphic) ventricular

tachycardia but does not treat atrial fibrillation. Additionally, lidocaine has been reported to
accelerate accessory pathway conduction.

17. The correct answer: E

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Question 18
18. A 68-year-old man was referred for evaluation and treatment of paroxysmal atrial fibrillation. He
experiences symptoms attributable to atrial fibrillation infrequently, but they are bothersome and
interfere with his quality of life.

His medical history was notable for hypertension but was otherwise unremarkable. His medications
included apixiban 5 mg BID, lisinopril 10 mg daily, and metoprolol succinate 50 mg daily.

A recent echocardiogram demonstrated normal right and left ventricular function. There was no
significant valve disease. The left atrium was mildly dilated. Estimated pulmonary artery systolic
pressure (PASP) was 25 mm Hg. A nuclear stress test performed 1 year ago in the setting of
atypical chest pain demonstrated normal perfusion at rest and stress.

You decided to prescribe flecainide for pill-in-the-pocket treatment of his atrial fibrillation.

What property of flecainide makes it particularly effective in this setting?

A. Use dependence.
B. Shortening of the action potential.
C. Shortening of the refractory period.
D. Increase rate of membrane depolarization.
E. Increase in delayed afterdepolarizations.

Class Ic antiarrhythmic agents slow conduction by blocking open sodium channels. They dissociate
slowly from the sodium channels during diastole, making them effective at rapid heart rates. This
property of the class Ic antiarrhythmics is called "use dependence" and is responsible for their
efficacy, particularly in supraventricular arrhythmias. This is also why these medications can be used
successfully as part of a "pill-in-pocket" approach for supraventricular tachycardia. Use dependence
is seen most commonly with class Ic drugs (flecanide, propafenone), less commonly with class Ia
drugs (quinidine, procainamide), and rarely in class Ib drugs (mexilitine). Thus, use dependence is
the correct choice.

Sotalol is a class III agent that blocks potassium channels, resulting in prolongation of repolarization,
action potential duration, and the refractory period. Class III agents have "reverse use dependence"
and are generally more effective at slower heart rates. Therefore, sotalol is more effective as a daily
medication for maintenance of sinus rhythm. It is unlikely to be effective at faster heart rates as part
of a "pill-in-pocket" approach.

Class Ib antiarrhythmic drugs increase the rate of membrane depolarization, increase delayed
afterdepolarizations, and shorten the refractory period; flecainide does not.

18. The correct answer: A

Key Point
Class I drugs block inward sodium channels and slow conduction velocity in the myocardium. Class I
drugs exhibit a phenomenon called "use dependence" in which the extent of channel block is
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increased at a higher heart rate. These drugs therefore block the sodium channels to a greater
degree at faster heart rates. Proarrhythmic potential of class I drugs can be assessed by performing
treadmill exercise testing to increase the heart rate.

Question 19
19. A 50-year-old male with paroxysmal atrial fibrillation and CHADSVaSC 0 was admitted with two
weeks of symptomatic atrial fibrillation. While in the hospital, he underwent a transesophageal
echocardiogram and cardioversion, and was loaded on amiodarone.

Which of the following is the most appropriate anticoagulation regimen?

A. No anticoagulation.
B. Aspirin plus clopidogrel.
C. Apixaban.
D. Warfarin.
E. Ticagrelor.

Patients with atrial fibrillation lasting more than 48 hours or of uncertain duration require
anticoagulation after cardioversion independent of their CHADSVaSC risk score because of the high
rates of thromboembolism in the 4 weeks following cardioversion. Possible regimens for
anticoagulation in the immediate post-cardioversion period include a direct oral anticoagulant such
as dabigatran, rivaroxaban, apixaban, or edoxaban; or warfarin with a heparin bridge until the
international normalized ratio is >2.0. Clopidogrel plus aspirin was studied in the ACTIVE-W trial and
proven to be inferior to warfarin for reduction of thromboembolic events in patients with atrial
fibrillation. Ticagrelor has never been studied in atrial fibrillation.

19. The correct answer: C

Key Point
Because of the risk of cardioembolic stroke associated with cardioversion, anticoagulation is
recommended independent of the CHA2DS2-VASc score in patients in whom AF has lasted >48
hours or if there is uncertainty regarding onset time.

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Question 20
20. Genetic testing for diagnosing long QT syndrome (LQTS) would be most appropriate for which of
the following individuals?

A 53-year-old male with a history of myocardial infarction, left ventricular

A.
ejection fraction 45% on amiodarone, and QTc 510 msec.
A 32-year-old male with QTc 470 msec, systolic murmur with Valsalva
B.
maneuver, and syncope while playing basketball.
A 51-year-old female with QTc 400 msec whose daughter has long QT
C.
syndrome with positive genetic screen.
A 28-year-old male with QTc 520 msec whose brother has suspected long
D.
QT syndrome and negative genetic screen.
A 45-year-old female with frequent premature ventricular complexes,
E.
right bundle branch block, and QTc 485 msec.

The Heart Rhythm Society/European Heart Rhythm Association (HRS/EHRA) Expert Consensus
Statement on the State of Genetic Testing for the Channelopathies and Cardiomyopathies provides
recommendations for when genetic testing for LQTS may be appropriate. Of note, the sensitivity of
genetic testing for suspected LQTS is approximately 80% and 20-25% of patients with LQTS
confirmed by a known genetic mutation may have a normal-range QTc.

• Class I recommendation for comprehensive or LQTS types 1-3 (LQT1-3)-targeted LQTS

genetic testing (e.g., KCNQ1, KCNH2, and SCN5A) for patients with a strong clinical
suspicion for LQTS and QT prolongation.
• Class I recommendation for comprehensive or LQT1-3–targeted LQTS genetic testing for
asymptomatic adult patients with QTc >500 msec (adults) or >480 msec (prepubescents) in
the absence of other clinical conditions that might prolong the QT interval (e.g., electrolyte
abnormalities, hypertrophy, bundle branch block).
• There is also a Class I recommendation for mutation-specific genetic testing for first-degree
relatives of individuals with LQTS and an identified causative mutation.
• There is a Class IIb recommendation for (what may be considered) comprehensive or LQT1-
3–targeted LQTS genetic testing (e.g., KCNQ1, KCNH2, and SCN5A) for any asymptomatic
patient with otherwise idiopathic QTc values >480 msec (adults) or >460 msec
(prepubescents) on serial 12-lead electrocardiograms (ECGs).

The 51-year-old female with normal QTc (400 msec) whose daughter has LQTS with positive
genetic screen should have genetic screening, as her daughter has LQTS and an identified
causative mutation. The 28-year-old male with QTc 520 msec whose brother has
suspected LQTS would not be expected to have a positive genetic screen despite his long
QTc due to the negative screen in his brother (the proband). The 45-year-old female with
QTc 485 msec and 53-year-old male with QTc 510 msec have alternative explanations for
prolonged QTc (right bundle branch block [RBBB] and drug induced, respectively). Finally,
the 32-year-old male with QTc 470 msec, systolic murmur with Valsalva maneuver, and
syncope while playing basketball is more likely to have hypertrophic cardiomyopathy and
does not meet other criteria for LQTS testing.

20. The correct answer: C

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Key Point
The success of genetic testing in LQTS depends in large part on the robustness of the clinical
diagnosis with mutations identified in 70-80% of cases when the diagnosis is certain (QTc >480
msec), but dropping significantly in borderline cases.

Question 21
21. A 32-year-old female presented to your office for evaluation of syncope. She has had 3 syncopal
episodes over the past 3 months. The first occurred while exercising on a treadmill, the second while
running to catch the bus, and the third while eating dinner. During the most recent episode, she
bruised her cheek and nose.

She has no past medical history. Her medications include sertraline 50 mg daily and an oral
contraceptive pill. Her family history is unknown, as she was adopted.

On examination, her heart rate was 74 bpm and blood pressure 110/70 mm Hg. Her lungs were
clear, heart was regular with no murmurs, and extremities were without edema.

Laboratories showed sodium 140 mmol/L, potassium 4.2 mEq/L, magnesium 2.2 mg/dL, and
calcium 9.8 mg/dL. An electrocardiogram (ECG) was recorded (Figure 1).

What is the best next step?

(Figure 1)
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A. Start nadolol 40 mg daily.

B. Implantable cardioverter-defibrillator implantation.
C. Perform genetic testing.
D. Stop sertraline.
E. Start verapamil sustained release 180 mg daily.

Long QT syndrome (LQTS) is diagnosed in the presence of corrected QT interval (QTc) = 500 msec
or LQTS risk score = 3.5 when secondary causes have been excluded or in the presence of a
pathogenic mutation in one of the LQTS genes. It can also be diagnosed when the QTc is 480-499
msec in a patient presenting with syncope. There are several genetic forms of LQTS, which affect
presentation and response to therapy. Given that syncope is often the result of an arrhythmic event
in patients with LQTS, early recognition and treatment are needed to avoid recurrences, which could
present as cardiac arrest or sudden cardiac death.

This ECG shows a QT of 639 msec with a QTc >480 msec in a patient presenting with syncope; the
diagnosis of LQTS can be made. Beta-blocker therapy, in the absence of contraindications, is
indicated as a first-line therapy in patients with LQTS and suspected arrhythmic syncope. Thus, the
correct answer is nadolol.

There are a number of factors that may cause acquired LQTS, including metabolic disorders of
hypokalemia, hypomagnesemia, and hypocalcemia, and medications such as -azole antifungals,
macrolide antibiotics, antipsychotics, and antiemetics. The website www.crediblemeds.org is a
resource for QT-prolonging drugs. Sertraline does not typically cause LQTS and thus there is no
need to stop this medication.

Implantable cardioverter-defibrillator (ICD) implantation is reasonable in patients with LQTS and

suspected arrhythmic syncope who are on beta-blocker therapy or are intolerant to beta-blocker
therapy. As this patient has not failed beta-blocker therapy, ICD is not indicated.

Verapamil may be prescribed to patients with catecholaminergic polymorphic ventricular tachycardia

(VT) but is not used in LQTS.

Genetic testing would not change management in a patient who has LQTS diagnosed by the
presence of syncope and a QTc >480 msec, and thus is not the best next step.

21.The correct answer: A

Key Point
LQTS can be diagnosed clinically if a patient presents with recurrent syncope and QTc interval >480
msec in repeated ECGs in the absence of secondary causes (e.g., drugs, electrolytes).

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Question 22
22. A 23-year-old male law school student presented for evaluation of several months of palpitations
and lightheadedness. These episodes often occur when he is playing hockey but also can occur
during mock trials. His physical examination was unremarkable. He underwent echocardiography,
which showed normal biventricular size and function and no valvular abnormalities. His
electrocardigram (ECG) was normal. His family history was significant for a maternal uncle who died
at age 24 while skiing.

Which of the following mutations is the most likely cause of his symptoms?
A. Plakophilin.
B. Ryanodine receptor.
C. Myosin heavy chain.
D. Transforming growth factor-beta.
E. Fibrillin.

This patient most likely has catecholaminergic polymorphic ventricular tachycardia (CPVT) given his
family history and symptoms with exercise and emotional stress. Although both calsequestrin and
ryanodine receptor mutations can cause this syndrome, ryanodine receptor mutations are much
more common and account for almost half of cases. Transforming growth factor-beta (TGF-β)
mutations are associated with familial thoracic aortic aneurysm diseases. Fibrillin mutations are
associated with Marfan syndrome. Myosin heavy chain mutations are associated with dilated
cardiomyopathy and hypertrophic cardiomyopathy. Mutations in plakophilin are associated with
arrhythmogenic right ventricular cardiomyopathy.

22. The correct answer: B

Key Point
A number of inheritable syndromes associated with increased risk of sudden death are recognized.

Question 23
23. A 23-year-old female with no past history presented to the emergency department after a
syncopal episode. She was eating dinner with her family when she felt faint and passed out, bruising
her forehead and cheek on the side of the table as she fell to the floor. Paramedics were called and
an electrocardiogram (ECG) was obtained (Figure 1).

Her medical history includes exercise-induced asthma and frequent urinary tract infections. She
uses albuterol as needed, takes a daily oral contraceptive, and recently completed a course of
levofloxacin. She does not smoke cigarettes or drink alcohol but smokes marijuana a few
days/week. Her family history is unremarkable.

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On examination, she was a young, thin female appearing scared but otherwise in no distress. Her
heart rate was 104 bpm and blood pressure was 96/60 mm Hg. A cardiac examination revealed
premature beats and her examination was otherwise unremarkable.

Laboratories showed thyroid stimulating hormone (TSH) 4.2 U/mL, potassium (K) 3.8 mEq/L, and
magnesium (Mg) 1.9 mg/dL.

Which of the following, in conjunction with levofloxacin, is most likely the cause of her presentation?

(Figure 1)
A. Hypokalemia.
B. Marijuana.
C. Hypomagnesemia.
D. Oral contraceptives.
E. Albuterol.

This patient presented with polymorphic ventricular tachycardia (VT) resulting in syncope. Although
drug-induced polymorphic VT is sometimes regarded as an idiosyncratic event, a number of risk
factors have been identified. Patients with multiple risk factors may face the greatest risk.

A comprehensive list of QT-prolonging drugs can be found at www.crediblemeds.org. Concurrent

use of more than one drug that can prolong the QT interval or use of a QT-prolonging drug with one
that slows drug metabolism due to inhibition of hepatic cytochrome P450 enzymes increases the risk
of polymorphic VT. Other risk factors for polymorphic VT include baseline QT prolongation,
bradycardia, and electrolyte disturbances (especially hypokalemia and hypomagnesemia, and less
often hypocalcemia).

This patient is on two medications with a known risk of polymorphic VT, albuterol and levofloxacin.
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This is the correct answer.

Oral contraceptives and marijuana do not prolong the QT interval separately or in combination with
levofloxacin, so these answers are incorrect.

Low potassium and low magnesium can prolong the QT intervals, but her levels would not be
considered low enough to increase the risk and thus these answers are not correct.

23. The correct answer: E

Key Point
Ventricular arrhythmia may be either monomorphic with a single QRS morphology or polymorphic
with changing QRS morphology. The most common cause of polymorphic ventricular tachycardia
(PVT) is acute myocardial ischemia, which may quickly progress to ventricular fibrillation (VF). Less
commonly, PVT is caused by circumstances that prolong the Q-T interval (drugs and ion-channel
disorders). It is important to recognize which common drugs are associated with Q-T prolongation
and discontinue.

Question 24
24. A 26-year-old female with no past medical history came to see you in clinic for follow-up after an
emergency department visit for an episode of syncope. She said that she was exercising when she
began feeling dizzy and nauseated. She tried to walk to the bathroom and then lost consciousness.
Her husband ran to her side and found her awake and oriented, but with a rapid pulse. By the time
emergency medical technicians arrived, her vital signs were normal, and her evaluation in the
emergency department was unremarkable.

On physical examination today, her heart rate was 66 bpm and blood pressure was 108/72 mm Hg.
She appeared well. Jugular venous pressure was normal and lungs were clear. There were no
murmurs. She had no lower extremity edema. Her electrocardiogram (ECG) demonstrated normal
sinus rhythm and right bundle branch block (RBBB) with QRS duration of 128 msec. Electrolytes
were within normal limits. Her echocardiogram showed normal left ventricular (LV) function, mild
right ventricular (RV) enlargement, mild RV dysfunction, and mild tricuspid regurgitation. An exercise
treadmill test in which the patient had multiple runs of the rhythm was performed (Figure 1). There is
no family history of syncope, cardiac arrhythmia, or sudden death.

Which of the following is the most appropriate next step?

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A. Genetic testing.
B. Endomyocardial biopsy.
C. Cardiac catheterization.
D. Cardiac magnetic resonance imaging.

The concern is that this young female has arrhythmogenic right ventricular cardiomyopathy (ARVC)
because she has exercise-induced ventricular tachycardia and RV enlargement on echocardiogram,
as well as RBBB on ECG. In order to meet criteria for ARVC, there should be two major criteria, or
one major and two minor criteria, in the categories of ventricular dysfunction, tissue characterization,
arrhythmias, and ECG de/repolarization criteria. The magnetic resonance imaging (MRI) will allow
both evaluation of ventricular dysfunction and size, as well as the tissue and fibrofatty infiltration
(Figure 2). Endomyocardial biopsy would also be helpful for tissue characterization, but is invasive
and subject to sampling error. MRI provides more data with less risk to the patient. Once the
diagnosis is made, you can refer for genetic testing for confirmation and family planning.

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Reproduced with permission from Tandri H, Castillo E, Ferrari VA, et al. Magnetic resonance
imaging of arrhythmogenic right ventricular dysplasia: sensitivity, specificity, and observer variability
of fat detection versus functional analysis of the right ventricle. J Am Coll Cardiol 2006;48:2277-84.
24. The correct answer: D
Key Point
The important differential diagnosis in right ventricular outflow tract ventricular tachycardia (VT) is VT
occurring in the situation of arrhythmogenic right ventricular cardiomyopathy (ARVC). One important
difference is that the resting 12-lead echocardiogram (ECG) shows marked T-wave inversion in the
right precordial leads (V1-V3/4) and there may be evidence of an epsilon wave. The 12-lead ECG in
patients with normal heart VT is normal. If ARVC is suspected, computed tomography or magnetic
resonance imaging should be performed to rule out this condition, which is a genetic abnormality.

Question 25
25. A 68-year-old male with a history of hypertension presented to the emergency department for
evaluation of palpitations. An electrocardiogram (ECG) was recorded (Figure 1).

He was advised to undergo catheter-based ablation.

Which of the following best describes the ablation that will be performed?

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(Figure 1)

Ablation of the posterior slow pathway most commonly located in the posterior
A.
third of Koch's triangle.
Radiofrequency energy is applied to create a line of ablation from the tricuspid
B.
annulus to the inferior vena cava.
Radiofrequency ablation of the pathway localized by catheter-based mapping of
C.
the atrial and ventricular insertion sites.
The release of a cryoablation balloon delivers confluent lesions that encircle the
D.
ostia of all four pulmonary veins.
Substrate-based extensive ablation of myocardium displaying abnormal
E.
electrogram characteristics.

Catheter ablation using radiofrequency or cryothermal energy is an important therapy in the

management of patients with various types of tachyarrhythmia. Catheter ablation is generally
indicated for the treatment of a recurrent or persistent symptomatic arrhythmia that has been
refractory to medical therapy or for which medical therapy is not tolerated or preferred. For select
arrhythmias known to have a high cure rate with ablation therapy (e.g., atrial flutter, paroxysmal
supraventricular tachycardia, Wolff-Parkinson-White [WPW] syndrome, idiopathic premature
ventricular complexes/ventricular tachycardia), catheter ablation may be indicated as a first-line
treatment.

The ECG shows atrial flutter. Typical (also called isthmus-dependent) atrial flutter utilizes a large
macroreentrant pathway in the right atrium. The cavotricuspid isthmus between the inferior vena
cava and the tricuspid annulus (IVC-TA isthmus) is an obligatory route for typical flutter and, as
such, is the best anatomic target for ablation. Thus, creation of a line of ablation from the TA to the
IVC is the correct answer.

Various methods of catheter ablation have been used for atrial fibrillation, and most focus on
isolating the triggers in the pulmonary veins (PVs) from the vulnerable substrate in the left atrium.
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Circumferential PV isolation involves the creation of confluent ablation lesions that encircle the ostia
of all four PVs. The goal of these lesions is to electrically separate the PVs from the left atrium.
Thus, PV isolation is not the correct answer, as it describes the ablation of atrial fibrillation (AF), not
atrial flutter.

The general approach to the catheter ablation of atrioventricular nodal re-entry tachycardia (AVNRT)
is based upon the concept of dual atrioventricular (AV) nodal pathways. The most common AVNRT
circuit involves anterograde conduction down the slow pathway and retrograde conduction up the
fast pathway. The ablation target is the posterior slow pathway since ablation here carries the lowest
risk of AV block, preserves fast pathway function (and a normal P-R interval postablation), and is
facilitated by reliable anatomic and electrophysiologic landmarks of Koch's triangle. Thus, ablation of
the slow pathway describes AVNRT ablation and is not the correct answer.

Patients with recurrent sustained monomorphic ventricular tachycardia (VT) resulting in implantable
cardioverter-defibrillator (ICD) shocks despite treatment with an antiarrhythmic drug may benefit
from catheter-based radiofrequency ablation. VT in patients with a prior myocardial infarction (MI) is
usually due to re-entry in a circuit created by the heterogeneous electrical properties of residual
myocardium in the region of the scar from the infarct. Usually, extensive ablation of myocardium
displaying abnormal electrogram characteristics is performed, so-called substrate-based ablation.
Thus, substrate ablation describes VT ablation and is not correct.

For patients with an accessory pathway and symptomatic arrhythmias including orthodromic
atrioventricular re-entry tachycardia (AVRT), antidromic AVRT, and pre-excited AF or atrial flutter,
catheter ablation is recommended. The location of most accessory pathways can be estimated using
the pre-excitation pattern on the surface echocardiogram. However, more precise localization of the
accessory pathway during catheter-based mapping prior to catheter ablation utilizes several
parameters to determine the atrial and ventricular insertion sites. Thus, ablation of the accessory
pathway seen in WPW syndrome is not the correct answer.

25. The correct answer: B

Key Point
Atrial flutter is a macro–re-entrant circuit most commonly within the RA and is sustained through a
region of slow conduction along a channel or “isthmus” between the IVC and the tricuspid valve, the
so called “cavotricuspid isthmus.” Due to the critical dependence of the arrhythmia on conduction
through this region, the rhythm is classified a “cavotricuspid isthmus-dependent” or CTI-dependent
atrial flutter.

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Question 26

26. An 84-year-old woman with paroxysmal atrial fibrillation and hypertension presented to the
hospital after a syncopal episode without prodrome while eating dinner. She regained consciousness
after 20 seconds, and other than a contusion on her cheek, she was unharmed. There was no
incontinence or generalized tonic clonic activity.

Her heart rate was 64 bpm and blood pressure was 146/80 mm Hg. She had a systolic ejection
murmur and her examination was otherwise unremarkable. An electrocardiogram (ECG)
demonstrated normal sinus rhythm with a right bundle branch block (RBBB) and left anterior
fascicular block. An echocardiogram showed normal biventricular function and aortic sclerosis.
Myocardial perfusion stress test revealed no evidence of ischemia.

The patient underwent an electrophysiology (EP) study without inducible ventricular tachycardia
(VT). An intracardiac electrogram was recorded (Figure 1).

Which of the following is the best next step?

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A. Single-chamber pacemaker.
B. Cardiac resynchronization therapy pacemaker.
C. Event monitor.
D. Dual-chamber pacemaker.
E. Implantable loop recorder.

The patient developed syncope in the setting of bifascicular block. An EP study is appropriate given
the high likelihood of a malignant cardiac etiology of her symptoms with a negative standard
evaluation. EP study reveals a prolonged H-V interval (normal is 35-55 msec). Current guidelines
give a Class I indication to permanent pacing in the setting of prolonged H-V interval, and a Class IIa
indication to implantation for unexplained syncope in the setting of bifascicular block. Thus,
permanent pacing is indicated.

Further testing with ambulatory ECG monitoring with an event monitor or implantable loop recorder
is not indicated because the etiology was already established on EP study.

In a patient with normal ejection fraction, cardiac resynchronization therapy is not indicated.
However, if she is subsequently noted to have frequent right ventricular pacing (>40%),
consideration of upgrade to cardiac resynchronization therapy (CRT) is reasonable.

An implantable cardioverter-defibrillator is not indicated, as her EP study showed no inducible VT

and her ejection fraction is >35%. As this patient has paroxysmal, not permanent, atrial fibrillation,
she may benefit from AV synchrony, which a dual-chamber pacemaker would provide and thus a
single-chamber pacemaker is not the best choice.

26. The correct answer: D

Key Point
AV block occurs due to a failure of impulse propagation through the cardiac conduction system.
Several types of AV block are recognized; most commonly first-, second-, and third-degree block.
Prognosis of AV block is related to the severity of block. AV block may be due to failure of impulse
propagation in the AV nodal tissues (narrow QRS complex) or within the His-Purkinje system (intra-
or infra-Hisian block). Failure of impulse propagation due to infra-Hisian block may be associated
with a wide QRS complex with bundle branch or fascicular block.

Question 27
27. A 54-year-old female presented to your office with frequent symptoms of palpitations associated
with lightheadedness. She has a history of hypertension and takes lisinopril. She denies any family
history of cardiac disease. She was recently seen in the emergency department with a 12-lead
electrocardiogram (ECG) during her palpitations (Figure 1). She was given 6 mg of adenosine; this
did not cause her rhythm to change, although by report there was evidence of organized atrial
activity during the transient period of atrioventricular (AV) block. Her palpitations resolved
spontaneously while she was in the emergency department and a repeat ECG was performed
(Figure 2).

Which of the following is the most likely etiology of her tachycardia?

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(Figure 1)

(Figure 2)

A. Re-entrant tachycardia involving dual pathway physiology within the atrioventricular node.
B. Rapidly firing foci originating from the pulmonary vein.
C. Enhanced automaticity originating within the fascicular system.
D. Re-entrant tachycardia involving an accessory pathway.
E. Abnormal ectopic atrial foci.

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Her ECG shows a narrow complex, regular tachycardia with an abnormal P-wave axis consistent
with atrial tachycardia. Administration of adenosine is more likely to convert an AV nodal-dependent
re-entrant tachycardia, such as AV nodal re-entrant tachycardia (AVNRT) or AV re-entrant
tachycardia (AVRT), than atrial tachycardia. The ECG in Figure 2 shows sinus rhythm and there is a
difference in P-wave morphology and axis compared with the supraventricular tachycardia (SVT)
tracing. Atrial tachycardia originates from an ectopic atrial focus. It is the least common cause of
SVT in a patient with a normal baseline ECG. Enhanced automaticity within the fasicular system
would cause fascicular ventricular tachycardia and would not demonstrate rapid atrial activity during
the period of AV block after administration of adenosine. Typical AVNRT involves dual-pathway
physiology within the AV node and may be precipitated by a premature atrial depolarization that is
blocked in one of the pathways. Because the re-entrant circuit involves the AV node, it is more likely
to terminate with administration of adenosine and would not show evidence of organized atrial
activity during the period of AV block after adenosine. Similarly, AVRT involves a re-entrant circuit
consisting of an accessory pathway and the AV node, and is also more likely to terminate with
administration of adenosine. The presence of rapidly firing foci in the pulmonary veins is one
mechanism of atrial fibrillation, which is irregular and would not reveal organized atrial activity during
the period of AV block after administration of adenosine.

27. The correct answer: E

Key Point
PSVT may be due to AVNRT, accessory pathway mediated tachycardia (AVRT), or AT. AT is most
commonly due to a single abnormally firing focus in the atrium. Unlike AVNRT and AVRT, AT is not
dependent on conduction through the AV node. Adenosine is an effective acute treatment of both
AVNRT and AVRT, but is less effective against AT, although use of adenosine in this situation may
produce transient AV block and unmask the mechanism of the tachycardia.

Question 28
28. A 30-year-old male with rheumatoid arthritis presented to your office for evaluation of syncope.
His medications include prednisone 7.5 mg daily, methotrexate 15mg weekly, hydroxychloroquine
200 mg daily, and dapsone 50 mg daily.

His blood pressure was 140/80 mm Hg with a pulse of 80 bpm. His examination was remarkable for
mildly swollen metacarpal joints bilaterally and was otherwise normal.

He became lightheaded in your office and an electrocardiogram (ECG) was obtained (Figure 1).

Which of the following is the most likely cause of the arrhythmia seen?

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(Figure 1)
Reproduced with permission from Roediger JE. 2012. Available at:
https://en.wikipedia.org/wiki/Torsades_de_pointes#/media/File:Torsades_de_Pointes_TdP.png.
Accessed 05/31/2018

A. Hydroxochloroquine.
B. Methotrexate.
C. Dapsone.
D. Prednisone.
E. Rheumatoid heart disease.

Acquired long QT syndrome can occur due to a variety of factors including medications and
electrolyte derangements. A comprehensive list can be found at www.crediblemeds.org.

In this patient, hydroxychloroquine is the likely culprit. His QT interval may return to normal once this
medication is stopped. Thus, the first step in assessment of long QT syndrome is identification of
treatable/reversible causes.

Prednisone, methotrexate, and dapsone do not prolong the QT interval and are incorrect choices.

Although rheumatoid arthritis can have an associated cardiomyopathy, there are no other symptoms
or signs of heart failure and this would not explain the ECG findings.

28. The correct answer: A

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Key Point
Ventricular arrhythmia may be either monomorphic with a single QRS morphology or polymorphic
with changing QRS morphology. The most common cause of polymorphic ventricular tachycardia
(PVT) is acute myocardial ischemia, which may quickly progress to ventricular fibrillation (VF). Less
commonly, PVT is caused by circumstances that prolong the Q-T interval (drugs and ion-channel
disorders). It is important to recognize which common drugs are associated with Q-T prolongation
and discontinue.

Question 29
29. An 82-year-old female with a history of hypertension and type II diabetes mellitus presented to
the hospital with dyspnea on exertion and lower extremity swelling and redness. She was placed on
vancomycin for presumed cellulitis. Her inpatient medications included aspirin 81 mg daily, atenolol
50 mg daily, amlodipine 10 mg daily, sliding scale insulin, heparin 5,000 units subcutaneous three
times a day, and pravastatin 40 mg daily. She had progressive shortness of breath. Chest computed
tomography (CT) with intravenous contrast showed no pulmonary embolism but did demonstrate
vascular congestion. She was started on furosemide 80 mg intravenous twice a day. The following
day, she was noted to have oliguria.

Which of the following medications should be held at this point?

A. Atenolol.
B. Amlodipine.
C. Heparin.
D. Pravastatin.
E. Aspirin.

The patient was admitted with lower extremity edema and dyspnea due to heart failure with
preserved ejection fraction (EF). However, on presentation she was started on vancomycin for
possible cellulitis. The combination of vancomycin, iodinated contrast, and acute heart failure
precipitated oliguric renal failure. Atenolol is a water-soluble beta-blocker and has a prolonged half-
life in the setting of renal failure. Therefore, it can accumulate to toxic levels in the setting of renal
failure and may cause significant sinus node dysfunction with severe bradycardia. The other
medications listed may be continued in the setting of acute renal failure.

29. The correct answer: A

Key Point
SND is a common and often age-related cause of bradycardia although extrinsic causes such as
drug therapy also may cause apparent SND.

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Question 30
30. A 63-year-old male came to the emergency department after an episode of syncope. Earlier in
the day, he had sudden onset of rapid palpitations and took a dose of propafenone; less than an
hour later, he stumbled and fell to the floor. He has a longstanding history of paroxysmal atrial
fibrillation with frequent episodes of symptomatic rapid ventricular response. He has good control of
his symptoms with pill-in-the-pocket propafenone. His other medical history includes hypertension,
benign prostatic hypertrophy, and chronic obstructive pulmonary disease. His current medications
are losartan 100 mg, tamsulosin 0.4 mg, and ipratropium inhaled as needed. His pulse was 58 bpm,
blood pressure was 130/82 mm Hg, and respirations were 12 breaths per minute. A physical
examination disclosed a well-appearing middle-aged male with regularly split S1 and S2, no
murmurs, and normal peripheral pulses. His electrocardiogram (ECG) showed sinus bradycardia
with P-R interval 180 msec and QRS duration 98 msec. Serum chemistries were normal and
troponin was negative. He underwent a stress echocardiogram 1 year ago, exercising 8:30 minutes
and reaching 86% age-predicted maximum heart rate with no ischemic ECG changes and normal
left ventricular ejection fraction (LVEF).

In addition to stopping propafenone, which of the following is the best next step in his care?

A. Thirty-day monitor.
B. Electrophysiology study.
C. Implantable loop recorder.
D. Tilt table test.
E. Coronary angiogram

The correct answer is a 30-day event monitor to assess for sinus pauses or other signs of sinus
node dysfunction after discontinuing the propafenone. Both propafenone and flecainide can
exacerbate underlying conduction system disease, unmasking sinus node dysfunction,
atrioventricular block, or infrahisian block, and increases in the P-R and QRS intervals of ≤25% over
baseline values are commonly seen with the use of these drugs. In this patient who has been using
propafenone without adverse effects for years, it is likely that sinus node dysfunction has gradually
progressed to the point that it is now symptomatic when using propafenone, but more information is
required to determine if invasive electrophysiology studies or pacemaker implantation will be
required.

He has no anginal symptoms and a negative troponin, as well as a negative stress test a year ago,
so there is no indication for coronary angiography at this time.

Tilt table testing would not be expected to add diagnostic value, as his symptoms do not point to
orthostatic or autonomic dysfunction.

An implantable loop recorder is not indicated at this time.

30. The correct answer: A

Key Point
Age-related SND also may be associated with AF, the so-called “tachy-brady” syndrome. This may
be associated with prolonged pauses, which can be symptomatic after termination of AF. A common
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scenario would be an elderly patient with persistent AF who develops a prolonged pause one
termination of AF. This can be exacerbated by drugs, especially those that block sodium channels
such as flecainide.

Question 31
31. A 64-year-old male with a history of sinus node dysfunction and complete heart block who
underwent dual-chamber permanent pacemaker 2 years ago came to your office complaining of
intermittent palpitations. During performance of his electrocardiogram by the nurse, he felt the onset
of palpitations, which continued for a minute after the electrocardiogram was completed (Figure 1).

Which of the following interventions is most likely to decrease his symptoms of palpitations?

A. Increasing his lower rate to 80 bpm.

B. Metoprolol 25 mg BID.
C. Ventricular lead revision.
D. Shortening his postventricular atrial refractory period.
E. Decreasing his atrioventricular delay.

The beats are ventricular ectopics and are not related to pacing. There is no evidence of pacemaker
lead malfunction, as there is appropriate sensing and ventricular capture. A trial of an antiarrhythmic,

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such as a beta blocker, should be attempted. If unsuccessful and he remains highly symptomatic,
then a ventricular tachycardia ablation could be considered.

31. The correct answer: B

Key Point
In many cases, PVCs are benign and do not require further treatment unless symptomatic.

Question 32
32. A 38-year-old male was admitted to the hospital following an episode of syncope. He had been
seated, watching late-night television, when he became unresponsive. A family member called 911
but he regained consciousness before the paramedics arrived. There was no postictal confusion and
he was reluctant to come to the hospital, but his wife insisted, saying, "This has happened before."
He does not remember details of the prior syncope. His family history is remarkable for an uncle who
died suddenly at age 41. His vital signs were pulse 98 bpm, respirations 14 breaths per minute, and
blood pressure 116/78 mm Hg. A physical examination revealed flat jugular veins, normal heart
sounds, and warm extremities. An electrocardiogram (ECG) was recorded (Figure 1). A bedside
echocardiogram demonstrated normal right and left ventricular size and function.

Which of the following is the most appropriate next step in the management of his syncope?

(Figure 1)
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A. Amiodarone.
B. Implantable cardioverter-defibrillator.
C. Verapamil.
D. Tilt table test.
E. Electrophysiology study.

This ECG is consistent with the Brugada type 1 pattern, described in the 2002 consensus statement
as "cove-shaped ST elevation in right precordial leads with J wave or ST elevation of at least 2 mm
(mV) at its peak followed by a negative T wave with little or no isoelectric interval in more than one
right precordial lead (V1-V3)." This type 1 pattern is diagnostic of the Brugada syndrome, and the
recurrent syncope and family history of sudden cardiac death are confirmatory clinical evidence that
intervention with an implantable cardioverter-defibrillator (ICD) is warranted. Provocative testing
such as drug challenge with procainamide or flecainide or programmed electrical stimulation during
electrophysiology testing would be indicated if he had type 2 or type 3 Brugada pattern on ECG
(Figure 2), but in cases of type 1 Brugada, these measures carry risks (of inducing ventricular
tachycardia or ventricular fibrillation) without adding benefits.

Tilt table testing can be useful for evaluating orthostatic intolerance and autonomic dysfunction, but
would not be expected to add to the diagnosis in this patient with unheralded syncope while seated.

Anti-arrhythmic therapy with either amiodarone or verapamil has not been shown to be effective in
preventing sudden cardiac death in these patients, although amiodarone may be considered for
adjunctive therapy if recurrent arrhythmias or in patients who are not candidates for ICD therapy.

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(Figure 2)
Precordial leads of a resuscitated patient with Brugada syndrome (BrS) showing all three
echocardiogram (ECG) patterns and dynamic changes over an 8-day period. Arrows indicate J
32. The correct answer: B
Key Point
ICDs are indicated in BrS patients with prior cardiac arrest (Class I) and those with spontaneous
type I pattern and recurrent syncope.

Question 33
33. A 21-year-old male college student was seen in clinic for evaluation of palpitations. These have
occurred sporadically since his teen years but have worsened in the last 2 years. He described
feeling an extra heartbeat followed by racing heartbeats. He has sometimes been able to terminate
these episodes with coughing. His past medical history and physical examination were otherwise
unremarkable.

A resting electrocardiogram (ECG) was obtained (Figure 1).

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Which of the following maneuvers/interventions is most likely to make the abnormal finding depicted
in the ECG less apparent?

(Figure 1)

A. Passive leg raise maneuver.

B. Inhaled amyl nitrate.
C. Exercise.
D. Intravenous metoprolol.
E. Valsalva maneuver.

This patient has a delta wave on ECG, which is due to "fusion" of impulses activating the ventricles
via both the atrioventricular (AV) node and via an accessory pathway. His history is suggestive
of paroxysmal re-entry supraventricular tachycardia (SVT). The combination of pre-excitation on
resting ECG and a history of SVT is consistent with Wolff-Parkinson-White (WPW) syndrome.

Exercise may increase conduction through the AV node, thus decreasing the degree of pre-
excitation and the delta wave will diminish.

Interventions that slow AV nodal conduction (such as metoprolol or vagal maneuvers, including the
Valsalva maneuver) will increase the degree of ventricular pre-excitation and make the delta wave
more apparent.

Amyl nitrate decreases preload, whereas a passive leg raise maneuver will increase preload. Under
normal conditions, changing preload will not change AV nodal conduction and thus should not
influence the appearance of the delta wave.

33. The correct answer: C

Key Point
Genesis of the delta wave is due to “fusion” of impulses activating the ventricles via both the AV
node and activation via the accessory pathway. In other words, a delta wave is only present if the
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accessory pathway can conduct from the atria to the ventricles. Factors that enhance AV nodal
conduction (such as exercise) cause predominantly more of the ventricles to be activated in a
normal fashion such that the delta wave will diminish with exercise, whereas factors that slow AV
nodal conduction such as drugs will increase the degree of pre-excitation. In other words, the degree
of pre-excitation can change and is not fixed.

Question 34
34.An 82-year-old female with atrial fibrillation presented with syncope. She had been watching
television when she felt a racing heart beat followed by lightheadedness. Her past history includes
hypertension and osteoarthritis. Her medications include apixaban 5 mg twice daily, amlodipine 5 mg
daily, metoprolol succinate 25 mg daily, and flecainide 100 mg twice daily.

On examination, her heart rate was 50 bpm and blood pressure was 140/80 mm Hg. Her lungs were
clear to auscultation. A cardiac examination revealed a soft systolic ejection murmur. Her extremities
were warm, without edema.

An electrocardiogram (ECG) showed sinus bradycardia at 50 bpm with nonspecific ST-T changes.
An echocardiogram showed normal ventricular function, proximal septal hypertrophy with left
ventricular outflow tract velocity of 1.1 m/sec with Valsalva, peak aortic velocity 2.5 m/sec, and left
atrial enlargement.

A 30-day ambulatory ECG monitor demonstrated heart rate 40-110 bpm with paroxysmal atrial
fibrillation and sinus pauses of up to 4 seconds upon conversion to sinus rhythm.

What is the most likely cause of her presentation?

A. Sinus node dysfunction.

B. Aortic stenosis.
C. Chronotropic incompetence.
D. Dynamic outflow tract obstruction.
E. Autonomic dysfunction.

This elderly female with atrial fibrillation has significant sinus pauses consistent with a diagnosis of
tachy-brady syndrome. This is likely caused by flecainide, a Class IC agent, which blocks sodium
channels, resulting in bradycardia. Flecainide prolongs depolarization and slows conduction in the
atrioventricular (AV) node, and profound sinus bradycardia can be induced in patients with pre-
existing sinus node disease.

Her presentation is not consistent with aortic stenosis, as she does not have a significant elevated
velocity across the aortic valve. Nor does she have a significant left ventricular outflow tract gradient
to cause dynamic outflow tract obstruction.

Her history is not consistent with autonomic dysfunction in that her episode did not occur with
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changes in position but while seated and preceded by palpitations, which is more consistent with
tachy-brady syndrome.

Chronotropic incompetence would not be expected to cause syncope at rest and, in addition, her
monitor demonstrated an appropriate resting heart rate.

34. The correct answer: A

Key Point
Age-related SND also may be associated with AF, the so-called “tachy-brady” syndrome. This may
be associated with prolonged pauses, which can be symptomatic after termination of AF. A common
scenario would be an elderly patient with persistent AF who develops a prolonged pause one
termination of AF. This can be exacerbated by drugs, especially those that block sodium channels
such as flecainide.

Question 35
35. A 60-year-old woman was admitted to the hospital for palpitations and lightheadedness, and was
referred to you for consultation. For the past 3 months, she has had spells about once a week in
which she suddenly feels very lightheaded, followed by a sensation of breathlessness and anxiety.
Twice in the past month she has fainted completely, one episode which was witnessed by her son.
Her past history includes obesity and fibromyalgia. Her medications are citalopram 20 mg daily and
pregabalin 50 mg 3 times daily. On physical examination, her blood pressure was 130/80 mm Hg,
heart rate was 70 bpm, and weight was 180 pounds (body mass index 32). Cardiovascular
examination revealed an irregular rhythm without murmur. Her abdomen was obese, but the
remainder of the examination was unremarkable. An electrocardiogram showed sinus rhythm at 80
bpm, with a borderline PR interval of 200 msec. An echocardiogram was performed the day prior
with normal findings. Laboratory studies were normal except for potassium of 3.5 mEq/dl. On
telemetry, strips were recorded during the physical examination (Figure 1).

Which of the following is the next step in management?

A. Pacemaker implant.
B. Continued observation.
C. Correct potassium.
D. Discontinue citalopram.
E. Discontinue pregabalin.

These strips show second-degree atrioventricular (AV) block with periods of 2:1 AV block. The
tracing likely indicates Mobitz type 2 block, which suggests that the site of block is below the AV
node (at the level of the His bundle), and therefore is more likely to progress to complete heart block.
She therefore qualifies for a pacemaker. The third strip may show Mobitz type 1 block with PR
prolongation prior to the blocked beat. Nevertheless, a pacemaker would still be indicated for all
types of symptomatic second-degree AV block. Premature ectopic (narrow) beats also are seen
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(these do not affect the sinus rate, which is about 75 bpm). Her episodes of syncope are likely
related to her conduction disease. Discontinuing medications or supplementing potassium will not
treat her subnodal conduction disease.

35. The correct answer: A

Key Point
Implantation of a permanent pacemaker is indicated for patients with irreversible symptomatic
bradycardia due to SND or AV block.

Question 36
36. A 66-year-old man presented to the emergency department with palpitations and shortness of
breath. He had a history of persistent atrial fibrillation and underwent radiofrequency ablation last
year. He was previously on sotalol, which was stopped 6 months following his ablation because he
was in sinus rhythm. He is currently taking metoprolol succinate 100 mg daily, lisinopril 20 mg daily,
and apixaban 5 mg twice daily.

An electrocardiogram was obtained (Figure 1).

What is the most likely mechanism of his arrhythmia?

(Figure 1)

A. Delayed afterdepolarizations.
B. Early afterdepolarizations.
C. Non–isthmus-dependent macro re-entry tachycardia.
D. Rapid focal ectopic activity.
E. Re-entry circuit within the triangle of Koch.
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Non–isthmus-dependent atrial flutter or atypical flutter describes macro re-entry atrial tachycardias
that are not dependent on conduction through the cavotricuspid isthmus. Non–isthmus-dependent
atrial flutters often occur in patients with atrial scarring from prior heart surgery or ablation, but may
also be idiopathic or occur in any form of heart disease. In this patient with a prior radiofrequency
ablation for atrial fibrillation, his arrhythmia is highly suspicious for an atypical atrial flutter originating
in the left atrium at the site of scar tissue. A surface EKG cannot reliably differentiate between typical
and atypical flutter in these patients. If the patient fails to respond to rate-controlling agents, then an
electrophysiology study with atrial mapping is indicated.

Rapid focal ectopic activity is the mechanism of atrial fibrillation.

Re-entry circuit within the triangle of Koch is the mechanism of atrioventricular nodal re-entry
tachycardia.

Early afterdepolarizations are the mechanism for torsades des pointes and polymorphic ventricular
tachycardia (VT).

Delayed afterdepolorizations can occur with acute myocardial infarction or digoxin toxicity, and may
manifest as bidirectional VT or catecholaminergic polymorphic VT.

36. The correct answer: C

Key Point
Non–isthmus-dependent atrial flutter involves macro–re-entrant circuits elsewhere in the RA or LA
and may occur in a variety of clinical settings, including congenital heart disease, after cardiac
surgery, and after catheter ablation of AF.

Question 37
37. Review the electrocardiogram (ECG) in Figure 1.

What is the correct diagnosis?

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(Figure 1)
Reproduced with permission from https://www.healio.com/cardiology/learn-the-heart/ecg-review/ecg-
topic-reviews-and-criteria/. Accessed 05/29/2018.

A. Accelerated idioventricular rhythm.

B. Mobitz type I second-degree heart block.
C. Third-degree heart block.
D. Mobitz type II second-degree heart block.
E. First-degree heart block.

This ECG demonstrates sinus bradycardia with a narrow QRS (first two beats) with a wider
ventricular rhythm subsequently overtaking the sinus rate, consistent with accelerated idioventricular
rhythm (AIVR) at a rate of ~75 bpm. AV dissociation can occur with an accelerated idioventricular
rhythm (AIVR) or complete heart block. The two, however, can be distinguished by the atrial and
ventricular rates.

In complete heart block, the sinus rate is faster than the ventricular rate. In AIVR, the ventricular rate
is faster than the sinus rate. Because the ventricular rate is faster than the atrial rate, this is not
complete heart block but AIVR. As there is no relation between the P waves and QRS complexes,
this is not consistent with first- or second-degree heart block.

37. The correct answer: A

Key Point
AV block should be distinguished from AV dissociation, where the ventricular rate is faster that the
atrial rate. In AV block, the atrial rate is faster than the ventricular rate.

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Question 38
38. A 30-year-old female has a sudden onset of palpitations and lightheadedness while playing with
her children in the park. After an hour, with no relief, she presented to the emergency department.
Her vital signs were blood pressure 120/70 mm Hg and heart rate 180 bpm. She was then given
adenosine 6 and 12 mg but without effect. Her electrocardiogram (ECG) was obtained (Figure 1).
She was offered cardioversion but refused.

Which of the following medications is most likely to chemically cardiovert this rhythm?

A. Ibutilide.
B. Verapamil.
C. Metoprolol.
D. Lidocaine.
E. Adenosine (high dose).

In this young patient without structural heart disease, one must first assess hemodynamic stability.
Because she has no chest pain, dyspnea, or hypotension, emergent therapy is not necessary. This
ECG shows a tachycardia with just a mildly wide QRS complex. There is a right bundle branch block
(RBBB)-like morphology. In a young person who is otherwise healthy, this ECG is most consistent
with idiopathic left ventricle (fascicular) ventricular tachycardia, which is a re-entrant tachycardia
involving most commonly the left posterior fascicle, giving an RBBB-like appearance and a superior
axis. This rhythm is highly sensitive to verapamil. It is a low-risk tachycardia and catheter ablation of
the fascicle involved is usually curative.

38. The correct answer: B

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Key Point
Distinction of ventricular tachycardia in patients with or without structural heart disease is
importantsince therapeutic approaches are very different. In patients with structural heart disease,
an implantable cardioverter-defibrillator is generally recommended (secondary prevention); in
patients without structural heart disease, drug therapy or catheter ablation may be effective in
reducing or eliminating the arrhythmia.

Question 39
39. A 25-year-old female presented to the emergency department after a fainting spell. She
collapsed without warning and appeared to stop breathing for about 30 seconds, after which she
spontaneously regained consciousness. A similar event occurred 2 months prior after she raced up
three flights of stairs. She has no significant past medical history and her only medication is
azithromycin, which was started 3 days prior for acute bronchitis. On examination, she felt fatigued.
In the emergency department, she lost consciousness and a rhythm strip was recorded (Figure 1).

Which of the following best explains the mechanism for the initiation of this heart rhythm?

(Figure 1)

A. Re-entry due to loss of the epicardial action-potential dome in phase II.

B. Re-entry due to enhanced potassium efflux during phase III of the action potential.
C. Re-entry due to myocardial scar and unidirectional block.
D. Triggered activity due to early afterdepolarizations.

This patient has a prolonged QT interval with polymorphic ventricular tachycardia (torsades de
pointes) degenerating into ventricular fibrillation. The prolonged QT interval triggers early
afterdepolarizations. Azithromycin is known to increase the QT interval and may predispose a

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patient with long QT syndrome to this event.

A young female with no prior history is unlikely to have scar-related ventricular tachycardia. Thus,
this is not the correct answer.

Acute myocardial ischemia causes re-entry due to loss of the epicardial action-potential dome in
phase II. This young and otherwise healthy patient is unlikely to have myocardial ischemia and thus
this is not the correct answer.

Enhanced potassium efflux during phase III of the action potential does not occur with azithromycin
and is therefore not the correct answer.

39. The correct answer: D

Key Point
EADs occur in the setting of a prolonged action potential (prolonged QT interval on the ECG) and
can result from drug exposure (e.g., class Ia and class III antiarrhythmic drugs, many
noncardiovascular drugs), gene mutations that cause LQTS, and clinical conditions such as marked
hypokalemia or acute ischemia. EADs can initiate monomorphic or polymorphic VT/VF.

Question 40
40. A 35-year-old male came to your clinic for his symptoms of palpitations. For the past 5 years, he
has felt an irregular beat with frequent ”skips.” He said that in the past 6 months his running capacity
has declined as well, and it takes longer for him to complete his usual 5-mile daily run. He denied
any syncope or chest pain. He has no past medical history. His family history is notable for an acute
myocardial infarction in his father at age 50 years. He takes no regular medications. On physical
examination, his blood pressure was 108/60 mm Hg with a pulse of 70 bpm. He weighed 170
pounds (body mass index was 25.8). The jugular venous pulse was seen at 6 cm and carotid
upstrokes were normal and without bruit. Lung sounds were clear and cardiac auscultation showed a
single S1, physiologically split S2, and a grade 1/6 short systolic murmur. An ectopic beat about every
three beats was heard on auscultation. An electrocardiogram (ECG) was obtained (Figure 1).

In addition to a transthoracic echocardiogram, what is the best next step?

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A. Implantable loop recorder

B. Holter monitor
C. Coronary Computed Tomography
D. EP Study

Frequent premature ventricular contractions (PVCs) may result in significant exercise intolerance or
a PVC-related cardiomyopathy. Establishing the burden of PVCs is critical in determine the next
step, including the need for PVC ablation, which can be considered if the PVC burden is over 25%.
Implantable loop recorders are only indicated in patients for whom noninvasive ECG monitoring is
inconclusive. Coronary computed tomography (CT) angiogram would not be the first step in
assessment of ischemia in a low-risk patient. An electrophysiology study (EP) is not indicated
without other findings such as syncope or abnormalities on ambulatory monitoring. Signal-averaged
ECG was previously used for risk stratification of sudden cardiac death in patients with coronary
artery disease, reduced ejection fraction, or unexplained syncope, but is no longer routinely used in
clinical practice.

40. The correct answer: B

Key Point
In such patients, it is important to assess the burden of PVC (the best way to do this is with a 24-
hour Holter monitor) and to rule out any degree of ventricular dysfunction. A 12-lead ECG also is
useful if it shows the same PVC in most of the leads because this can help to determine the site of
the VT origin. A cardiac MRI should be considered in certain situations to help exclude other
underlying structural disease such as ARVD or incidental scarring from remote inflammation.

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Question 41
41. A 52-year-old male with a history of paroxysmal atrial fibrillation presented to the emergency
department after an episode of syncope while he was cooking dinner in his kitchen. He reported he
felt lightheaded for a second before collapsing; his wife witnessed the episode, and he woke up and
felt fine 10 seconds later. In the emergency department, his heart rate was 80 bpm, his blood
pressure was 140/76 mm Hg, and he appeared well. His home medications include flecainide 100
mg twice daily and metoprolol 50 mg twice daily. His electrocardiogram (ECG) in the emergency
department (Figure 1) was compared with his prior ECG from last year (Figure 2).

Which of the following most likely explains his current ECG findings and syncope?

(Figure 1)

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(Figure 2)
A. Excessive beta-blockers.
B. Ischemia.
C. Increased vagal tone.
D. Brugada syndrome.
E. Torsades de pointes.

This patient's ECG demonstrates type 1 Brugada pattern. Sodium channel blockers (flecainide,
propafenone) can exacerbate the transient ECG abnormalities that occur in patients with Brugada
syndrome who commonly have normal ECGs. Serial ECG testing may demonstrate the intermittent
changes, and use of class 1c agents during electrophysiologic testing can be used to unmask
Brugada pattern. This patient's ECG and history are most concerning for Brugada-induced
polymorphic ventricular tachycardia/ventricular fibrillation. Excessive beta-blockers and vagal tone
may result in syncope, particularly in the setting of a conversion pause from atrial fibrillation, but
these would not explain the ECG changes. There are no ECG findings of ischemia, and the QT
interval is normal. Torsades de pointes would be a concern if the patient were on a class III
antiarrhythmic drug (sotalol, dofetilide) and his QT were prolonged.

41. The correct answer: D

Key Point
BrS is an arrhythmogenic condition in patients with a structurally normal heart and a characteristic
ECG appearance of incomplete/complete RBBB pattern. Familial transmission is autosomal
dominant, but often there is incomplete penetrance.

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Question 42
42. A 65-year-old man with a history of coronary artery disease with a remote history of
percutaneous coronary intervention (PCI) for angina symptoms, hypertension, hyperlipidemia, and
type 2 diabetes mellitus presented to your office for evaluation. He reported that he was in his usual
state of health until 2 days prior when he experienced a first syncopal episode while washing dishes.
He does not recall any symptoms prior to or after the event. He denies any chest pain, dyspnea, or
palpitations. He reported being compliant with his medications, which include aspirin, atorvastatin,
lisinopril, and metformin. On examination, his heart rate was 72 bpm and regular, and his blood
pressure was 132/78 mm Hg. His physical examination including neurologic assessment was
unremarkable. An electrocardiogram (ECG) demonstrates sinus rhythm and poor R wave
progression.

In addition to ambulatory ECG monitoring, which of the following is the best next step in this patient's
management?

A. Carotid ultrasound.
B. Electroencephalogram.
C. Echocardiogram.
D. Autonomic testing.
E. Tilt table testing.

Evaluation of left ventricular (LV) structure and function is the most appropriate next step. In patients
with unexplained syncope, arrhythmic causes are more common in patients with LV dysfunction. The
patient described a history concerning for cardiogenic syncope due to a malignant ventricular
arrhythmia, typically characterized as sudden onset in nature and associated with a rapid recovery.
Several factors favor a cardiac cause for syncope in this patient, including age >60 years, male sex,
and known ischemic heart disease. The absence of a prodrome makes reflex syncope less likely
and therefore tilt table testing less appropriate as an initial evaluation. Routine carotid ultrasound
and electroencephalogram (EEG) are not indicated in the evaluation of syncope in the absence of
focal neurologic findings (Class III). This patient's presentation is not consistent with neurogenic
orthostatic hypotension and therefore autonomic testing is not indicated.

42. The correct answer: C

Key Point
Cardiogenic syncope is characterized clinically with sudden loss of consciousness and rapid
recovery. Most cardiogenic syncope is related to bradycardia or a ventricular arrhythmia.

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Question 43
43. A 30-year-old male with hypertrophic cardiomyopathy status post–dual-chamber implantable
cardioverter-defibrillator (ICD) for secondary prevention of sudden cardiac death presented to your
office for routine follow-up. He was on metoprolol succinate 50 mg daily. ICD interrogation disclosed
3 episodes of atrial flutter in the past 3 months, the longest lasting 2 hours. He has been
asymptomatic during these episodes.

What is the best next step?

A. Aspirin 325 mg daily.
B. Left atrial appendage occlusion device.
C. No change in medications.
D. Radiofrequency ablation.
E. Rivaroxaban 20 mg daily.

Patients with atrial flutter have the same risk of thromboembolism as patients with atrial fibrillation
(AF); therefore, recommendations for anticoagulation mirror those for patients with AF guided with
the CHADS2VASC score (2). In this patient with hypertrophic cardiomyopathy, the risk of stroke is
unrelated to the CHADS2VaSC score and is high enough that anticoagulation is recommended in all
patients with AF or atrial flutter and hypertrophic cardiomyopathy (Class I).

Catheter-based ablation for atrial arrhythmias is not indicated in an asymptomatic patient. Aspirin
offers inferior thrombotic prophylaxis and thus is not the preferred choice. Left atrial occlusion is only
indicated in patients who do not tolerate anticoagulation.

43. The correct answer: E

Key Point
Risk of stroke in patients with AFL is identical to risk in patients with AF and should be estimated
based on the CHA2DS2-VASc score, as is the case with AF.

Question 44
44. A 32-year-old man presented to your office for evaluation of syncope. One month ago, he
passed out while standing during church services on a hot summer day. He reports feeling
nauseated and diaphoretic at the time.
He has no past history and takes no medications. He has no significant family history of cardiac
disease or sudden death.

On examination, his heart rate was 84 bpm and blood pressure was 122/70 mm Hg. His lungs were
clear, heart regular with no murmurs, and extremities were without edema.
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An electrocardiogram (ECG) was recorded (Figure 1).

What do you advise the patient?

(Figure 1)

A. Electrophysiology study to assess for ventricular arrhythmias.

B. Cardiac magnetic resonance imaging.
C. Physical counterpressure maneuvers.
D. Genetic testing.
E. Implantable cardioverter-defibrillator implantation.

This patient's ECG is consistent with a Brugada pattern. Brugada syndrome is a genetic disease
characterized by an increased risk of sudden cardiac death (SCD) and ST elevation with type 1
morphology =2 mm in at least 1 lead among the right precordial leads V1 and V2.

Syncope is a risk factor for cardiac arrhythmic events in patients with Brugada syndrome.
Implantable cardioverter-defibrillator (ICD) implantation is reasonable in these patients; however, the
benefit seems to be limited to patients with suspected arrhythmic syncope. Patients with syncope
consistent with a reflex-mediated mechanism should not undergo the implantation of an ICD.

This patient's history is consistent with classic vasovagal syncope. Given the lack of benefit of ICD
therapy in patients with reflex syncope and the known rate of inappropriate shocks and ICD
complications in patients who receive an ICD, ICD implantation is not recommended when the
syncope mechanism is believed to be reflex mediated. Thus, ICD is not the correct answer.

Management of vasovagal syncope focuses on preventive and abortive measures. Patients with a
syncope prodrome should be instructed to assume a supine position to prevent a faint and minimize
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possible injury. In patients with a sufficiently long prodrome, physical counter-maneuvers (e.g., leg
crossing, limb and/or abdominal contraction, squatting) are a core management strategy. In a
randomized, parallel, open-label trial, leg crossing with conventional therapy (e.g., fluid, salt intake,
counseling, and avoidance) was superior to conventional therapy in preventing syncope recurrence.

The value of an electrophysiology study (EP) in assessing the mechanism of syncope or prognosis
in patients with Brugada is unknown. Therefore, EP may be considered only in patients with syncope
suspected to be due to an arrhythmia and is not recommended in patients with reflex syncope.

Genetic testing is not used to make the diagnosis of Brugada syndrome, nor does genetic testing
affect management. Thus, this is not the correct answer.

While cardiac magnetic resonance imaging (MRI) is helpful in some cardiac conditions associated
with arrhythmias, such as sarcoidosis or arrhythmogenic right ventricular cardiomyopathy, it does not
play a role in the diagnosis or management of Brugada syndrome.

44. The correct answer: C

Key Point
Sudden death can be the first manifestation of BrS, although most patients have a history of
recurrent syncope, particularly at rest.

Question 45
45. A 60-year-old female has a history of paroxysmal atrial fibrillation (AF) that has been
symptomatic enough to warrant treatment. She was started on sotalol 80 mg BID, which was titrated
up to 120 mg BID because of ongoing recurrences. She was started on hydrochlorothiazide 25 mg
QD 1 week ago for hypertension. She was brought by ambulance to the emergency department for
multiple episodes of lightheadedness and dizziness. Her blood pressure was 120/76 mm Hg and her
serum potassium was 3.2 mEq/L. Her electrocardiogram was obtained (Figure 1).

Which of the following should be the next step in her management?

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(Figure 1)

A. Intravenous potassium and magnesium.

B. Electrical cardioversion.
C. Intravenous metoprolol.
D. Temporary transvenous pacing.
E. Intravenous amiodarone.

Sotalol is a class III antiarrhythmic agent used primarily for the treatment of AF. It blocks the inward
rectifying potassium channel, resulting in a prolongation of the QT interval.

Figure 1 shows nonsustained episodes of torsades de pointes (TdP), accounting for the patient’s
complaints of palpitations and dizziness. Her QTc is 570 msec (QT 400 msec/square root of the RR
interval of 600 msec). The risk of TdP with sotalol is <2%, but is higher in the setting of bradycardia,
female sex, pre-existing QT prolongation, history of heart failure, history of ventricular
tachycardia/ventricular fibrillation, or hypokalemia.

The treatment of sustained TdP in a hemodynamically unstable patient is prompt electrical

defibrillation. In a stable patient, intravenous (IV) magnesium is effective in both the treatment and
prevention of TdP. Temporary transvenous overdrive pacing to treat TdP usually is reserved for
patients not responding to IV magnesium.

Class III antiarrhythmic drugs such as sotalol have reverse-use dependence, such that the QT
lengthens as the heart rate (HR) slows, and the QT shortens as the HR increases. This helps
explain why temporary pacing and IV isoproterenol help treat TdP by increasing the HR and
decreasing the QT interval, whereas slowing the HR with beta-blockers would be contraindicated. IV
amiodarone, which also prolongs the QT interval, would be contraindicated. Therefore, the most
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appropriate first steps in treating this patient would be treating her hypokalemia, infusing IV
magnesium and potassium, and discontinuing her sotalol.

45. The correct answer: A

Key Point
Proarrhythmic effect of class III drugs is due to excessive prolongation of the QT interval, leading to
polymorphic VT (torsades de pointes). Proarrhythmic effect of class III drugs is maximal at slower
heart rates.

Question 46
46. A 75-year-old male presented to your office with 2 episodes of near syncope over the past 2
months. The symptoms occurred while lying in bed prior to falling asleep and again while he was
driving to work. His past medical history is significant for hypertension. His medications include
aspirin and amlodipine. He denied exertional chest pain, shortness of breath, or palpitations. On
examination, his blood pressure was 145/80 mm Hg and heart rate was 49 bpm. His jugular venous
pressure was normal, there was no S3 or S4, his lungs were clear, and there was no peripheral
edema. The remainder of his examination was normal. His electrocardiogram (ECG) showed sinus
bradycardia with first-degree atrioventricular (AV) block and no ST or T-wave abnormalities.

Which of the following is the best next diagnostic study for this patient?
A. Cardiac catheterization.
B. Tilt table test.
C. Vasodilator single-photon emission computed tomography.
D. Thirty-day event monitor.
E. Transthoracic echocardiogram.

This older patient is having intermittent near syncope with resting bradycardia, raising the suspicion
of sinus node dysfunction. The best test to determine whether his symptoms are due to bradycardia
or sinus pauses would be an event monitor. Due to the infrequent nature of his episodes, a more
extended monitoring period would be needed than would be afforded by a Holter monitor. This
patient has no signs or symptoms of heart failure, so a transthoracic echocardiogram is less likely to
reveal the etiology of his symptoms. The patient does not have any symptoms suggestive of
ischemia, and thus neither a vasodilator single-photon emission computed tomography (SPECT) nor
cardiac catheterization are indicated. Finally, a tilt table test would not be the best next test, as the
history is not consistent with neutrally mediated presyncope.

46. The correct answer: D

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Key Point
SND comprises a number of different manifestations including symptomatic bradycardia, sinus
pauses due to sinus arrest or sinoatrial exit block, and chronotropic incompetence.

Question 47
47.An 18-year-old female with no significant past medical history had been evaluated at a college
sports physical. Her physicial examination was normal. Although she had swum before, at the
second meet of the year, she experienced an episode of sudden cardiac death while swimming.

Which of the following mechanisms explains the likely pathophysiology of the sudden death?
A. Gain of function in the calcium channel.
B. Loss of function in the potassium channel.
C. Loss of function in the sodium channel.
D. Gain of function in the sodium channel.
E. Gain of function in the potassium channel.

A young adult with sudden cardiac death should raise the suspicion of an underlying primary
electrical disorder or hypertrophic cardiomyopathy. There are certain settings that are regarded as
classic for certain syndromes. Of these, long QT syndrome type 1, with a mutation in the IKs
potassium channel leading to a loss of function and thus a delay in membrane repolarization, has a
classic predilection for events with swimming.

47. The correct answer: B

Key Point
Avoidance of strenuous competitive sports is important (Class I).

Question 48
48. A 50-year-old male with a history of paroxysmal atrial fibrillation and coronary artery disease was
initiated on dofetilide. His medications include aspirin 81 mg daily, lisinopril 10 mg daily, simvastatin
40 mg daily, and metoprolol 50 mg twice daily. His heart rate was 55 bpm, blood pressure was
123/75 mm Hg, and the remainder of the examination was unremarkable. His electrocardiogram
(ECG) showed sinus bradycardia at 54 bpm with a nonspecific intraventricular conduction delay,
QRS duration of 105 msec, and QT interval of 430 msec. The laboratory panel showed normal renal
function and a serum potassium level of 5.6 mEq/L.

Which of the following effects of dofetilide may increase the risk for torsades de pointes in this
patient?
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A. Decreased potassium efflux exacerbated by hyperkalemia.

B. Increased potassium efflux exacerbated by hyperkalemia.
C. Reverse-use dependence that is exacerbated by bradycardia.
D. Use dependence that is exacerbated by bradycardia.
E. Decreased drug metabolism in the presence of simvastatin.

Dofetilide exhibits reverse-use dependence, with an increased effect at slow heart rates.
Bradycardia in this patient may exacerbate the QT-prolonging effects of dofetilide. Dofetilide does
not have a drug interaction with simvastatin. Dofetilide has many drug interactions, especially those
that prolong the QTc, cause hypokalemia, or cause hypomagnesemia. Verapamil also alters
dofetilide metabolism and increases toxicity.

48. The correct answer: C

Key Point
Class III drugs block outward potassium channel activity (IKs) and prolong refractoriness. These
agents exhibit "reverse use-dependence" in which a greater degree of channel block is seen at
slower heart rates.

Question 49
49. A 50-year-old female with rheumatoid arthritis presented to the emergency department with
syncope. Her current medications are prednisone 7.5 mg daily, methotrexate 15 mg weekly,
hydroxychloroquine 200 mg daily, dapsone 50 mg daily, and nystatin swish and spit daily. On
examination, her heart rate was 84 bpm, blood pressure was 130/80 mm Hg without orthostatic
change, and respiration was 14 breaths per minute. She appeared anxious. Other than swelling of
her hand joints, her examination was normal. An electrocardiogram (ECG) was obtained (Figure 1).

Which medication is most likely responsible for her presentation?

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(Figure 1)

A. Prednisone.
B. Nystatin.
C. Methotrexate.
D. Dapsone.

This patient's ECG demonstrates prolonged QTc. Acquired long QT syndrome may be caused by
a number of factors including electrolyte disturbances and medications. A comprehensive list of
drugs that can prolong the QT interval may be found at www.crediblemeds.org.
Hydroxychloroquine contributes to QT interval prolongation. The other medications she is taking
do not significantly affect the QT interval.

49. The correct answer: E

Key Point
Ventricular arrhythmia may be either monomorphic with a single QRS morphology or polymorphic
with changing QRS morphology. The most common cause of polymorphic ventricular tachycardia
(PVT) is acute myocardial ischemia, which may quickly progress to ventricular fibrillation (VF).
Less commonly, PVT is caused by circumstances that prolong the Q-T interval (drugs and
ion-channel disorders). It is important to recognize which common drugs are associated
with Q-T prolongation and discontinue.

Question 50

50. A 52-year-old male came to the emergency department for mild palpitations that started the prior
evening. Palpitations persisted when he awoke the following morning, and therefore he drove himself
to the hospital. He has had 3 prior episodes over the past 2 months, each lasting only 5-10 minutes.
He has had a prior myocardial infarction with a single angioplasty and stent to the left circumflex artery
and an overall left ventricular ejection fraction of 45%. He takes aspirin 81 mg, clopidogrel 75 mg,
ramipril 5 mg daily, bisoprolol 5 mg, and atorvastatin 40 mg daily. He was somewhat anxious
appearing, although not in distress. His blood pressure in the emergency department was
90/65 mm Hg and heart rate was 135 bpm. The lungs were clear with no peripheral edema.
The presenting electrocardiogram (ECG) was obtained (Figure 1).

Which of the following is the best next step in management?

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A. Administer intravenous ibutilide.

B. Administer intravenous adenosine.
C. Perform carotid sinus massage.
D. Perform synchronized cardioversion.
E. Administer intravenous metoprolol.

The ECG shows wide complex tachycardia with atrioventricular dissociation diagnostic of ventricular
tachycardia (VT). The patient is hemodynamically stable, although somewhat hypotensive and
anxious. The prior infarct and VT morphology is suggestive of a scar-based VT. Cardioversion is the
best option for this patient (although amiodarone or procainamide may be considered as well).
Ibutilide is appropriate for atrial fibrillation or atrial flutter, whereas carotid sinus massage or
adenosine would be appropriate for supraventricular tachycardia. Metoprolol also would be
ineffective and potentially harmful given the patient's hypotensive state.

50. The correct answer: D

Key Point
Ventricular arrhythmia may be either monomorphic with a single QRS morphology or polymorphic
with changing QRS morphology. The most common cause of polymorphic ventricular tachycardia
(PVT) is acute myocardial ischemia, which may quickly progress to ventricular fibrillation (VF). Less
commonly, PVT is caused by circumstances that prolong the Q-T interval (drugs and ion-channel
disorders). It is important to recognize which common drugs are associated with Q-T prolongation
and discontinue.

Question 51
51. A 45-year-old male saw you in consultation in the office regarding frequent palpitations. He
described pounding heart beats, 1 or 2 per hour throughout the day, which increase during times of
stress. An echocardiogram was normal. An exercise stress test had to be stopped due to increasing
ventricular ectopy, including a five-beat run of ventricular tachycardia that terminated as soon as

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exercise stopped; there were no other electrocardiographic (ECG) changes during the stress test. A
12-lead ECG was obtained (Figure 1).

Which of the following is the most likely origin of the premature ventricular contractions (PVCs)?

A. Left ventricular outflow tract.

B. Body of the left ventricle.
C. Right ventricular outflow tract origin.
D. Right ventricle apical morphology.

This patient's palpitations are due to idiopathic ventricular outflow tract PVCs. These are commonly
associated with nonsustained and sustained ventricular arrhythmias during stress or during 24-hour
ECG (Holter) monitoring. Possible origins of this arrhythmia are in the right ventricular outflow tract
(RVOT; more common) or left ventricular outflow tract (LVOT; less common). The PVCs in the
ECG example have a left bundle, inferior-axis morphology. The precordial R-wave transition (totally
positive QRS complexes in lead V3) is earlier than is typically seen in RVOT ventricular tachycardia.
The LVOT (sinuses of Valsalva specifically) is posterior to the RVOT, which results in more positive
voltage and earlier transition in the precordial leads. When idiopathic outflow tract PVCs are more
frequent than 10% of total heart beats, they may be associated with development of
cardiomyopathy. Beta-blockers or nondihydropyridine calcium channel blockers are often effective,
with ablation reserved for patients who are refractory to medical therapy or with cardiomyopathy due
to the high-density arrhythmia.

51. The correct answer: A

Key Point
RVOT VT is important to recognize, as this is a focal VT amenable to curative catheter ablation. This
form of VT has a very characteristic ECG signature (LBBB with inferior axis and late R-wave
progression, beyond V3). Catheter ablation is highly successful in this condition. The VT is NOT
ischemia driven, and therefore, ischemic workup is not required or necessary unless there are other
symptoms such as exertional chest pain with risk factors that make CAD likely.
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Question 52
52. A 52-year-old male presented to his cardiologist's office for routine follow-up. He has a history of
ventricular tachycardia and long QT syndrome (LQTS) with a mutation found in the KCNQ1 gene.
He had an implantable cardioverter-defibrillator (ICD) placed 2 years prior. He had been seen by his
primary care clinician the previous day for an upper respiratory infection and was started on
levofloxacin. His other medications include metoprolol succinate, atorvastatin, and aspirin. His
physical examination was unremarkable. His electrocardiogram (ECG) showed normal sinus rhythm
with a QTc of 435 msec.

Which of the following is the best next step in the management of this patient?
A. Increase metoprolol.
B. Stop atorvastatin.
C. Initiate amiodarone.
D. Discontinue levofloxacin.
E. Continue current management.

This patient is genotype positive for LQTS and has a history of ventricular tachycardia. Although his
QTc is currently in the normal range, QT-prolonging medications should still be avoided.
Levofloxacin is known to prolong QT, so discontinuing this medication and using a non-QT
prolonging antibiotic is most appropriate. Amiodarone is not recommended in the treatment of LQTS.
Atorvastatin does not prolong the QT and thus is not the correct answer. Metoprolol succinate is a
first-line treatment for LQTS. While increasing metoprolol may reduce the risk of ventricular
arrhythmias, the correct response here is to discontinue the offending medication.

52. The correct answer: D

Key Point
All genotype-positive/phenotype-negative subjects should avoid QT-prolonging drugs and correct
any electrolyte abnormalities immediately.

Question 53
53. A 63-year-old man came to see you in your clinic. He has a past medical history of coronary
artery disease for which he underwent percutaneous coronary intervention to the mid left anterior
descending and mid right coronary artery 3 years ago. A recent echocardiogram showed a dilated
left ventricle with a left ventricle ejection fraction of 26%. He tries to walk 3 times a week but feels
shortness of breath when trying to walk up a flight of stairs. He is on optimal medical therapy,
including carvedilol and lisinopril. His electrocardiogram showed normal sinus rhythm and left bundle
branch block with a QRS width of 160 msec.

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At this point, which of the following is the next best step in his management?
A. Implant a single-chamber implantable cardioverter-defibrillator.
B. Continue current medical therapy.
C. Implant a dual-chamber implantable cardioverter-defibrillator.
D. Implant a biventricular implantable cardioverter-defibrillator.

The correct answer is to implant a biventricular implantable cardioverter-defibrillator (ICD) to

decrease the risk of death or heart failure hospitalization based on the results from MADIT-CRT
(Multicenter Automatic Defibrillator Implantation Trial With Cardiac Resynchronization Therapy) and
RAFT (Resynchronization/Defibrillation for Ambulatory Heart Failure Trial), which demonstrated that
a biventricular ICD in a patient with New York Heart Association class II reduced the composite of
death and heart failure events. In the RAFT trial, the secondary endpoint of mortality also was
achieved with a biventricular ICD compared with ICD alone. Continuing current medical therapy is
incorrect, as the patient meets criteria at this time for a device implant. In the 2012 update to the
2008 American College of Cardiology Foundation/American Heart Association/Heart Rhythm Society
device guidelines, it is now a Class I indication to implant a biventricular ICD in a patient with a left
ventricle ejection fraction of 35%, in sinus rhythm, in New York Heart Association class II, III, or
ambulatory IV, with left bundle branch block and QRS duration >150 msec.

53. The correct answer: D

Key Point
About two thirds of patients respond to CRT with symptomatic improvement. Pre-implant causes of
CRT non-response include non-LBBB native conduction, or a relatively narrow native QRS.

Question54
54. The patient is a 67-year-old male with a history of coronary artery disease for which he
underwent coronary artery bypass graft surgery 5 years ago. A recent echocardiogram showed a
mildly enlarged left ventricle with a left ventricular ejection fraction of 25%. He is able to exercise 4
times a week for 1 hour and also hikes for a couple of hours every weekend. He is on optimal
medical therapy with carvedilol, lisinopril, aspirin, and atorvastatin. His electrocardiogram showed a
normal sinus rhythm with a right bundle branch block pattern and QRS duration of 138 msec.

Which of the following is the most appropriate recommendation for this patient?
A. Continue current medical therapy.
B. Implant an implantable cardioverter-defibrillator.
C. Implant a biventricular implantable cardioverter-defibrillator.
D. Implant a biventricular pacemaker.
E. Initiate sotalol therapy.

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The patient meets criteria for a primary prevention implantable cardioverter-defibrillator (ICD) based
on MADIT-II (Multicenter Automatic Defibrillator Implantation Trial II) clinical trial results. Continuing
current medical therapy and initiating sotalol therapy are incorrect, as the patient meets criteria for
device implant and antiarrhythmic therapy is not demonstrated to improve survival. Implanting a
biventricular ICD and implanting a biventricular pacemaker are also incorrect, as the guidelines state
it is a Class III indication for biventricular pacing cardiac resynchronization therapy in patients with
New York Heart Association class I or II and a non-left bundle branch block pattern with QRS
duration of <150 ms.

54. The correct answer: B

Question 55
55. A 32-year-old male presented to the clinic for evaluation of "heart flutters" and lightheadedness.
These symptoms have become more frequent over the last 6 months and are mainly noticeable at
rest or as he is cooling down from exercise. He was able to run 2 miles 3 days a week without
limitations. He would prefer to avoid chronic medical therapy. He has no other medical conditions.
His family history includes myocardial infarction in his father at age 63. His physical examination was
normal. An electrocardiogram (ECG) demonstrated multiple premature ventricular complexes
(PVCs) with a left bundle branch block (LBBB) morphology and inferior axis. A transthoracic
echocardiogram is unremarkable.

A 24-hour Holter monitor revealed 9,240 PVCs as well as multiple runs of nonsustained ventricular
tachycardia (VT).
Which of the following would be the best next step?

A. Exercise stress test.

B. Metoprolol.
C. Cardiac catheterization.
D. Catheter ablation.
E. Propafenone.

This patient's presentation and ECG are consistent with right ventricular outflow tract (RVOT) VT,
which may be seen in individuals with structurally normal hearts. In patients with symptomatic RVOT
VT, beta-blockers or calcium channel blockers may be useful. Catheter ablation has been shown to
be more effective for suppression of RVOT PVCs than antiarrhythmic drugs or beta-blockers, and
may be useful in patients with a preference for avoiding chronic daily therapy. This patient has no
symptoms of ischemic heart disease and his symptoms are not prompted by exertion, so neither a
cardiac catheterization nor exercise stress test are indicated.

55. The correct answer: D

Key Point
RVOT VT is important to recognize, as this is a focal VT amenable to curative catheter ablation. This
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form of VT has a very characteristic ECG signature (LBBB with inferior axis and late R-wave
progression, beyond V3). Catheter ablation is highly successful in this condition. The VT is NOT
ischemia driven, and therefore, ischemic workup is not required or necessary unless there are other
symptoms such as exertional chest pain with risk factors that make CAD likely.

Question 56
56. A 75-year-old male presented to the emergency department with palpitations. He has a history of
hypertension and symptomatic bradycardia for which he underwent dual-chamber pacemaker
implantation 2 years prior. His pacemaker has been programmed to the DDD mode 50-120 bpm. An
electrocardiogram (ECG) was obtained (Figure 1).

An intervention was performed, with resolution of the patient's symptoms. An ECG was obtained
after the intervention (Figure 2).

Based on the ECG shown in Figure 2, which of the following interventions was performed?

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A. Administering adenosine.
B. Increasing the atrial sensitivity.
C. Programming to VVI mode.
D. Increasing the ventricular pacing output.
E. Programming hysteresis off.

The first tracing (Figure 1) demonstrates ventricular pacing at 110 bpm, consistent with tracking of
either a supraventricular tachycardia or pacemaker-mediated tachycardia. In the second tracing
(Figure 2), the rate has been decreased to 50 bpm with an underlying atrial tachycardia at 110 bpm.
There is no relation between the P waves and paced QRS morphology, consistent with
reprogramming the device to VVI mode, the correct answer.

Adenosine causes transient atrioventricular (AV) block, which would not affect pacemaker function,
and therefore would not resolve the rhythm.

Increasing the ventricular pacer output is not indicated because the pacemaker has adequate
ventricular capture.

Increasing the atrial sensitivity will not resolve the rhythm because there is no evidence of failure to
track atrial rhythms.

Hysteresis promotes intrinsic activity lower than the normal rate by allowing slow but appropriate
intrinsic rhythms. Changing the hysteresis parameters will not help resolve the tachycardia.

56. The correct answer: C

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Key Point
Age-related SND also may be associated with AF, the so-called “tachy-brady” syndrome. This may
be associated with prolonged pauses, which can be symptomatic after termination of AF. A common
scenario would be an elderly patient with persistent AF who develops a prolonged pause one
termination of AF. This can be exacerbated by drugs, especially those that block sodium channels
such as flecainide.

Question 57
57. A 29-year-old male with Brugada syndrome underwent ambulatory electrocardiogram (ECG)
monitor for complaints of palpitations. He was found to have several episodes of sustained
ventricular tachycardia (VT). Two episodes occurred the day prior; one was associated with
syncope. He was admitted for implantable cardioverter-defibrillator (ICD) implantation. On postop
day #1, he had 3 ICD shocks. His ICD was interrogated and shocks deemed appropriate for
sustained VT. He was counseled about use of antipyretic medications in the setting of fever,
avoidance of drugs that may induce or aggravate ST-segment elevation, and avoidance of excess
alcohol.

The addition of which of the following is most appropriate in his care?

A. Procainamide.
B. Flecainide.
C. Quinidine.
D. Amiodarone.
E. Propranolol.

Quinidine, a Class Ia antiarrhythmic drug with Ito and IKr blocker effects, prevents induction of
ventricular fibrillation (VF) and suppress spontaneous ventricular arrhythmias in patients with
Brugada syndrome. Quinidine is currently being used in (1) patients with ICD and multiple shocks;
(2) cases in which ICD implantation is contraindicated; or (3) for the treatment of supraventricular
arrhythmias. In this patient, the use of quinidine would be indicated to suppress ventricular
arrhythmias and avoid future ICD shocks (Figure 1). Procainamide and flecainide (sodium channel
blocking agents) can be used to make the diagnosis of Brugada syndrome. Brugada syndrome can
be diagnosed by type I ST-segment elevation occuring spontaneously or after the administration of
these agents. Amiodarone has also been reported to unmask ST-segment elevation and has been
reported to be proarrhythmic in patients with Brugada syndrome. In patients with Brugada syndrome,
beta-blockers can increase ST-segment elevation and are therefore contraindicated.

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(Figure 1)

57. The correct answer: C

Key Point
Quinidine may be effective to prevent recurrent arrhythmias in BrS patients with an ICD.

Question 58
58. A 47-year-old female with a history of tobacco use, hypertension, and obesity presented to the
emergency department with sudden onset of palpitations associated with dyspnea and neck
discomfort. On examination, she appeared mildly uncomfortable and vital signs included heart rate
of 170 bpm and blood pressure of 138/66 mm Hg. A 12-lead electrocardiogram (ECG) was obtained,
revealing a regular narrow complex tachycardia with inferolateral ST-T changes. Vagal maneuvers
were performed without effect, so the patient subsequently received 6 mg intravenous (IV)
adenosine with conversion to sinus rhythm and improvement in her symptoms. A repeat ECG was
normal.
Which of the following is the most likely diagnosis?

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A. Junctional tachycardia.
B. Atrioventricular nodal re-entry tachycardia.
C. Atrial tachycardia.
D. Atrial flutter.
E. Atrioventricular re-entry tachycardia.

This patient presented with supraventricular tachycardia (SVT). In patients with a normal baseline
12-lead ECG (no pre-excitation or delta wave) such as observed in this patient, the most common
mechanism of a regular narrow complex tachycardia is atrioventricular nodal re-entry tachycardia
(AVNRT). The neck discomfort she reported may be due to cannon A waves, related to pulsatile
reverse flow from right atrial contraction against a closed tricuspid valve. The next most frequent
narrow complex SVT is atrioventricular re-entry tachycardia (AVRT), followed by atrial tachycardia,
which accounts for <10% of cases. Junctional tachycardia is even less common in adults. Finally,
the absence of flutter waves on the ECG makes a diagnosis of atrial flutter unlikely.

58. The correct answer: B

Key Point
In patients with a normal 12-lead ECG (no pre-excitation or delta wave), the most common
mechanism of narrow complex tachycardia is AVNRT followed by AVRT. AT is the least common
form of SVT, accounting for <10% of cases.

Question 59
59.An 80-year-old female with hypertension and diabetes mellitus presented to your clinic with
exertional dyspnea and fatigue. Her current medications included lisinopril 10 mg daily and glyburide
5 mg daily. Her family history was significant for coronary artery disease and hypertension. On
examination, her blood pressure was 145/80 mm Hg with heart rate of 50 bpm. A cardiovascular
examination revealed II/VI early peaking ejection systolic murmur and the reminder of the
examination was unremarkable. She underwent a nuclear stress imaging study on a modified Bruce
protocol; she exercised for 6 minutes and stopped secondary to fatigue. Her maximum blood
pressure and heart rate were 180/90 mm Hg and 96 bpm, respectively. There were no ischemic
electrocardiogram changes and her perfusion images showed no evidence of stress-induced
ischemia.

What is the most likely cause of her symptoms?

A. Deconditioning.
B. Chronotropic incompetence.
C. Aortic stenosis.
D. Hypertension.
E. Microvascular dysfunction.

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Sinus node dysfunction (SND) refers to a broad array of abnormalities in sinus node and atrial
impulse formation and propagation. These include persistent sinus bradycardia and chronotropic
incompetence without identifiable causes, paroxysmal or persistent sinus arrest with replacement by
subsidiary escape rhythms in the atrium, atrioventricular (AV) junction, or ventricular myocardium.
This patient's symptoms are related to SND with chronotropic incompetence as evidenced by her
heart rate response during exercise, with a peak heart rate <100 bpm and <70% age-predicted
maximum heart rate.
Although she may also be deconditioned, the blunted heart rate increase in response to exercise
suggests that deconditioning is not her primary problem. Deconditioned patients should have an
exaggerated heart rate response to low levels of exercise.

Although she has a murmur, it was early peaking and her blood pressure response to exercise was
normal, so significant aortic stenosis is unlikely.
Her blood pressure response was normal, not exaggerated, and so would not explain her symptoms.
The normal perfusion study does not suggest ischemia as a factor in her symptoms.

59. The correct answer: B

Key Point
SND comprises a number of different manifestations including symptomatic bradycardia, sinus
pauses due to sinus arrest or sinoatrial exit block, and chronotropic incompetence.

Question 60
60. An 18-year-old woman was referred for evaluation for a family history of long QT syndrome
(LQTS). She felt well and denied any history of fainting, although she noted that her heart pounds
with exercise. She has no past medical history and does not smoke or drink alcohol. Her uncle was
resuscitated from a cardiac arrest and subsequently diagnosed with LQTS based on his postcardiac
arrest electrocardiogram (ECG). On physical examination, her blood pressure was 110/60 mm Hg
with a heart rate of 70 bpm, and the remainder of her examination was normal. She brought in a
copy of an ECG done by her family clinician 2 weeks prior, which showed sinus rhythm at 75 bpm
with a QTc of 460 msec. An ECG in your office today had similar findings, with a QTc of 450 msec.

Which of the following is the next step in management?

A. Stress test.
B. Implantable cardioverter-defibrillator implant.
C. Repeat electrocardiogram standing.
D. Electrophysiologic study.
E. Genetic testing of the Uncle.

This patient has a family history of probable LQTS in her uncle. Her ECG shows borderline QT
prolongation. Genetic testing, starting with her uncle, should be performed. Initiation of beta-blockers
would be reasonable if a diagnosis of LQTS can be made in the 18-year-old patient. There is no role
for electrophysiologic testing and implantable cardioverter-defibrillator (ICD) implant carries a Class
IIb indication only in the presence of strong risk factors for sudden cardiac death, which this patient
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has not demonstrated. Stress testing as a provocative test for QT prolongation may be considered in
uncertain cases. However, the clinical use of this test requires more extensive validation and would
not be the best choice.

60. The correct answer: E

Key Point
The success of genetic testing in LQTS depends in large part on the robustness of the clinical
diagnosis with mutations identified in 70-80% of cases when the diagnosis is certain (QTc >480
msec), but dropping significantly in borderline cases.

Question 61
61. A 30-year-old male presented to your office for evaluation of syncope. He has fainted twice in the
past year, both during exercise. He has no other medical problems and takes no medications. His
family history includes a paternal uncle who died at age 34 in a drowning accident. On examination,
his heart rate was 70 bpm, respirations were 12 breaths per minute, and blood pressure was 108/72
mm Hg. His heart and lung sounds were normal. An electrocardiogram (ECG) was obtained (Figure
1).

Which of the following is the best next step in his care?

(Figure 1)

A. Cardiac magnetic resonance imaging.

B. Reassurance.
C. Genetic testing.
D. Provocative testing with intravenous isoproterenol.
E. Electrocardiogram with high precordial leads.

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Brugada syndrome is an inherited arrhythmia syndrome characterized by ST elevation in right-sided

precordial leads. Type 1 ST-segment changes, "coved," are diagnostic of the syndrome whereas
type 2 "saddle back" ST changes are equivocal, as seen in this ECG (Figure 2). To make a definitive
diagnosis, type 1 ST-segment elevations must be observed following superior placement of
precordial leads (V1 and V2) in the second intercostal space or after administration of sodium
channel blocking drugs.

This patient has a history of syncope, but the ECG is not diagnostic (i.e., type 2 Brugada pattern)
and so the next step is to perform an ECG with superior placement of leads V1 and V2.

Isoproterenol challenge would be useful for diagnosing ventricular arrhythmias in the setting of
arrhythmogenic right ventricular cardiomyopathy or as an adjunct to tilt table testing for syncope.

Reassurance would be inappropriate for this patient with equivocal ECG findings and a concerning
family history.
Genetic testing and cardiac magnetic resonance imaging (MRI) are not useful in the diagnosis of
Brugada syndrome, which is suggested by the ECG.

(Figure 2)
Precordial leads of a resuscitated patient with Brugada syndrome (BrS) showing all three
echocardiogram (ECG) patterns and dynamic changes over an 8-day period. Arrows indicate J
waves.

61. The correct answer: E

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Key Point
Use of a sodium channel-blocking agent (flecainide or ajmaline) can “unmask” BrS and can assist in
the diagnosis of borderline cases. Recording leads V1 and V2 in second and third intercostal space
can also assist in the diagnosis.

Question 62
62. A 20-year-old man presented to the emergency department with palpitations for the past 2 hours.
He has no past medical history. He takes no medications. On arrival, his blood pressure was 108/66
mm Hg with a heart rate of 190 bpm. An electrocardiogram was obtained (Figure 1).

What is the best next step in the management of this patient?

(Figure 1)

A. Ibutilide 1 mg intravenously.
B. Synchronized cardioversion.
C. Digoxin 0.5 mg intravenously.
D. Metoprolol 5mg intravenously.
E. Adenosine 6 mg intravenously.

The patient's resting rhythm reveals a Wolff-Parkinson-White (WPW) pattern and was found on
presentation to be in atrial fibrillation (AF) with rapid ventricular rate, but hemodynamically stable.
Small observational studies support the use of ibutilide or intravenous procainamide for the
treatment of pre-excited AF in patients who are not hemodynamically compromised. Both
medications can decrease ventricular rate by slowing conduction over the accessory pathway and
have the additional benefit of possibly terminating AF.

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Prompt direct-current cardioversion is recommended for patients with AF, WPW syndrome, and
rapid ventricular response who are hemodynamically compromised, which is not the situation. While
cardioversion could be considered, it would not be the first option, in an awake and
hemodynamically stable patient.

Administration of intravenous beta-blocker, adenosine, or digoxin (oral or intravenous) in patients

with WPW syndrome who have pre-excited AF is potentially harmful because these drugs accelerate
the ventricular rate.

62. The correct answer: A

Question 3
63. A 56-year-old female was referred for evaluation of intermittent dizziness with occasional near-
syncope. Episodes occur 1-2 times a month, do not have any specific triggers, and last <1 minute.
She has not had a syncopal event, but is often presyncopal. She had no other cardiac symptoms
and was otherwise healthy. Her family history is notable for hypertension and atrial fibrillation in her
mother, and her grandmother had a pacemaker.

Which of the following is the best next diagnostic test for this patient?

A. Patient-activated event monitor.

B. Tilt table test.
C. A 24-hour continuous electrocardiogram (Holter).
D. Implanted loop recorder.
E. Electrophysiologic study.
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A patient-activated event monitor can record electrocardiogram (ECG) tracings immediately prior to
and during symptoms, allowing identification of possible arrhythmias. A 24-hour continuous ECG
(e.g., Holter) is not correct: Episodes are likely to be too infrequent to be captured on a 24-hour
Holter. An implanted loop recorder may be considered when noninvasive diagnostic testing is
unrevealing, if symptom frequency is too low to capture on event monitor, or if the patient's symptom
is syncope. Although electrophysiologic study (EPS) allows direct assessment of the cardiac
conduction system, it does not allow correlation of symptoms with an arrhythmia. Tilt table testing
can evaluate for orthostatic symptoms or postural orthostatic tachycardia syndrome, but is not
appropriate for identifying other arrhythmias.

63. The correct answer: A

Key Point
When evaluating patients with lesser degrees of bradycardia, symptom-rhythm correlation is
important. This can be achieved with ambulatory monitoring (Holter or similar). More prolonged
monitoring with an implantable subcutaneous monitor may be needed in some cases.

Question 64
64. A 32-year-old woman presents to the emergency department with palpitations and progressive
shortness of breath for the past two days.

She has no medical history. Her medications include ibuprofen as needed for headaches and oral
contraceptive pills.

On examination, her temperature is 99.4°F, heart rate is 134 bpm and irregular, blood pressure was
90/60 mm Hg, respiratory rate is 20 breaths/min, and oxygen saturation 90% on room air. Her
jugular venous pressure (JVP) is 14 cm H2O. Her lungs are clear, heart is s irregular with a soft
systolic murmur at the left sternal border, abdomen is soft, and extremities are without edema. An
electrocardiogram (ECG) is recorded (Figure 1). A chest X-ray (CXR) shows no infiltrate, effusion, or
edema.

Which test is most likely to elucidate the cause of her symptoms?

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(Figure 1)

A. Ventilation-perfusion scan.
B. Thyroid-stimulating hormone.
C. Echocardiogram.
D. Blood cultures.
E. Troponin.

Many potentially “reversible” causes of atrial fibrillation (AF) have been reported, including binge
drinking, cardiothoracic and noncardiac surgery, myocardial infarction (MI), pericarditis, myocarditis,
hyperthyroidism, electrocution, pneumonia, and pulmonary embolism. It is important to assess for
treatable causes because without targeting the underlying cause, control of AF is likely to be
ineffective and the underlying cause may result in significant morbidity and mortality.

This patient is on oral contraceptives, increasing her risk of thromboembolism. She has shortness of
breath with relative tachypnea, hypoxemia, all consistent with possible pulmonary embolism. Her
ECG shows atrial fibrillation. The fact that she has no evidence of lower extremity deep vein
thrombosis (DVT) on examination does not argue against pulmonary embolism, as the venous
thrombosis could have embolized to her lungs, leaving a benign lower extremity examination. Thus,
a ventilation-perfusion scan to assess for pulmonary embolism is the best answer.

Whereas she has a mildly elevated JVP, she has no evidence of pulmonary vascular congestion and
thus does not appear to have heart failure with reduced ejection fracture. The elevated JVP is likely
from right heart strain from the pulmonary embolism. Although the echocardiogram might show signs
of this, it is not the test most likely to directly yield the correct diagnosis.

It is unlikely that a young female without cardiac risk factors presenting with dyspnea and hypoxia is
having an acute coronary syndrome, and thus troponin is not the best answer.
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Although hyperthyroidism could cause AF, and could be responsible for the patient's anxious
appearance, it would not explain her other findings and is thus not the best answer.

Her low-grade temperature is likely due to thromboembolic disease, not infection, and thus blood
cultures are not indicated.

64. The correct answer: A

Key Point
Wherever possible, reversible causes for AF should be sought and corrected. These include:
hyperthyroid state, pulmonary embolism, and pericarditis.

Question 65
65. An 81-year-old female was admitted to the hospital with syncope. Two weeks ago, she was
started on paroxetine for depression. Since then, she has felt increasing fatigue when walking or
doing household chores, and she felt presyncopal several times. This morning, she "blacked out"
and fell to the floor on her way to the kitchen. Her home health aide found her on the floor and called
911; when they arrived, her pulse was 41 bpm and she was "groggy." Her past medical history
includes depression, glaucoma, hypertension, hypothyroidism, dyslipidemia, and intermittent
claudication. Her current medications are amlodipine 5 mg, aspirin 81 mg, cilostazol 100 mg,
levothyroxine 75 mcg, lisinopril 20 mg, paroxetine 20 mg, simvastatin 40 mg, and timolol eye drops.
Her vital signs on admission were blood pressure 138/88 mm Hg without orthostatic change, pulse
48 bpm, and respiration 12 breaths per minute. She appeared her stated age with moist mucous
membranes and normal skin turgor. Her cardiac examination was normal except for bradycardia. An
electrocardiogram was recorded (Figure 1).

Interaction between paroxetine and which of the following is most likely responsible for her syncope?

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(Figure 1)

A. Amlodipine.
B. Levothyroxine.
C. Cilostazol.
D. Simvastatin.
E. Timolol.

The correct answer is timolol. Timolol eye drops can produce systemic effects including
bradyarrhythmias that mimic sinus node dysfunction. Both paroxetine and timolol are metabolized by
cytochrome P450 2D6 and when coadministered can increase the concentration of the other drug.
Drugs causing bradycardia should be discontinued before making a diagnosis of sinus node
dysfunction, which requires pacing for treatment. Ophthalmic beta-blockers can often be replaced
with prostaglandins or other ophthalmic preparations for glaucoma with resolution of
bradyarrhythmias.

Cilostazol is a PDE-3 inhibitor that produces systemic vasodilation. This can lead to reflex
tachycardia and would not explain bradyarrhythmias.
Amlodipine, levothyroxine, and simvastatin do not have clinically significant interactions with
paroxetine and would not be expected to produce bradycardia.

65. The correct answer: E

Key Point
SND is a common and often age-related cause of bradycardia although extrinsic causes such as
drug therapy also may cause apparent SND.

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Question 66
66. A 30-year-old female presented for evaluation of episodes of recurrent syncope. Over the past
20 years, she has fainted 10 times, but in the past 6 months she has had 3 episodes. Typical spells
occur when she sings in the church choir and are preceded by about 30 seconds of nausea and
sweating. She has not injured herself, but is embarrassed, as the last time she fainted the
paramedics were called. She has no other past medical history and takes no medications. On
physical examination, her blood pressure (BP) was 110/60 mm Hg and heart rate was 90 bpm. Her
jugular venous pulse was 5 cm. There was a single S1 and physiologically split S2 without murmur.
The remainder of her examination was unremarkable. An electrocardiogram in the office showed
normal sinus rhythm at a rate of 93 bpm. The following day, she underwent a tilt table study showing
a supine BP of 120/70 mm Hg and a heart rate of 80 bpm. With passive tilt, the BP over the first 5
minutes was 110-120/55-65 mm Hg and heart rates were 80-100 bpm. At 8 minutes into the tilt, she
complained of nausea just like she feels before her usual faints, and then 30 seconds after that she
lost consciousness. Her BP reading obtained about 20 seconds before fainting was 65/40 mm Hg
and heart rate was 40 bpm. At the moment when she fainted, her heart rate was 30 bpm. She was
immediately laid supine, with prompt return of her BP and heart rate to baseline levels.

Which of the following is the best next step in her management?

A. Initiate midodrine.
B. Implant a dual-chamber pacemaker.
C. Avoid triggers for syncope.
D. Initiate metoprolol.

This patient has vasovagal syncope with a well-defined trigger: standing in the choir at church. Her
tilt table study suggests a mixed vasodepressor and cardioinhibitory response, and it reproduced her
symptoms. While a clinical syncopal spell may show a different physiologic response, the first step in
management should be conservative, stressing hydration, salt, and avoidance of triggers. While
other diagnostic tests such as echocardiogram and stress testing are commonly used, the diagnosis
in this patient is clear, without the need for further diagnostic testing. Likewise, electrophysiologic
study can be useful for patients with syncope suspected to be due to a ventricular arrhythmia, but
does not play a role for the workup of vasovagal syncope. If conservative measures fail, then an
implantable loop recorder can be considered to determine if clinical episodes show a marked cardio-
inhibitory response (despite the tilt table findings), as there can be a role for pacing for patients with
prolonged periods of asystole.

66. The correct answer: C

Key Point
Therapy for recurrent vasovagal syncope should center on education and recognition of prodromal
symptoms. Counterpressure maneuvers can be helpful; salt and fluid liberalization may be helpful in
selected patients.

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Question 67
67. A 56-year-old male presented to the emergency department with a 12-hour history of
palpitations. He has had similar spells over the last few years, some of which have required
cardioversion. On physical examination, he was mildly anxious. His heart rate was 145 bpm, blood
pressure was 135/88 mm Hg, respiratory rate was 18 breaths/min, and oxygen saturation 99% while
breathing ambient air. A cardiovascular examination revealed tachycardia but no murmurs. The
remainder of his examination was unremarkable. His electrocardiogram (ECG) was recorded (Figure
1). He was given metoprolol 5 mg intravenously and 25 mg PO. Twenty minutes later, he felt much
better. A repeat ECG was normal and did not reveal any structural abnormalities.

What is the best therapy in the management of this patient?

(Figure 1)

A. Amiodarone.
B. Digoxin.
C. Flecainide PRN.
D. Catheter ablation.
E. Metoprolol.

This patient's ECG demonstrates typical atrial flutter, for which the best treatment is catheter
ablation, which has a 95% success rate. Achieving ventricular rate control in atrial flutter is
characteristically difficult with atrioventricular nodal blocking agents. Given the high success rate
with catheter ablation, either chronic (amiodarone) or episodic (PRN flecainide) use would not be
ideal.

67. The correct answer: D

Key Point
Catheter ablation is the preferred treatment option for recurrent symptomatic PSVT – success rates
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exceed 95% in good centers. Drug therapy (prophylaxis or as the occasion arises with drugs that
block AV node or antiarrhythmic drugs) is rarely effective or helpful.

Question 68
68. A 75-year-old man presented to the emergency department with symptoms of palpitations
associated with dizziness. He has a history of coronary artery disease and underwent a 3-vessel
coronary artery bypass graft (CABG) 5 years ago. His most recent echocardiogram 3 months ago
showed an ejection fraction of 20% with a dilated left ventricle and hypokinesis of the mid anterior
and apical segments. On examination, he was in mild distress but alert and oriented. His blood
pressure was 110/55 mm Hg. An electrocardiogram (ECG) was obtained (Figure 1).

Which of the following is the underlying mechanism of his arrhythmia?

(Figure 1)

A. Delayed afterdepolarizations.
B. Early afterdepolarizations.
C. Re-entrant circuit.
D. Abnormal automaticity.
E. Aberrancy in setting of supraventricular tachycardia.

The ECG shows a wide complex tachycardia with positive concordance across the precordial leads
and a monophasic R wave in V1 consistent with ventricular tachycardia (VT). The most common
mechanism of ventricular tachycardia in patients with coronary artery disease is scar-mediated re-
entrant VT. Delayed afterdepolarizations are a type of triggered activity typical of idiopathic outflow
tract VT. Early afterdepolarizations are seen in torsades de pointes in setting of long QT syndrome.

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Abnormal automaticity can be seen in fascicular ventricular tachycardias. The 12-lead ECG is not
consistent with supraventricular tachycardia (SVT) with aberrancy.

68. The correct answer: C

Key Point
Monomorphic ventricular tachycardia (MVT) is most commonly due to a re-entry mechanism around
an infarcted scar tissue. Less commonly, MVT is due to a single focus in patients without structural
heart disease.

Question 69
69. A 54-year-old female presented to your office for a second opinion regarding management of
atrial fibrillation. She was diagnosed with atrial fibrillation 5 months ago. She was advised by her
primary cardiologist to consider catheter ablation due to episodes of uncomfortable palpitations and
exercise intolerance that occurred during episodes of atrial fibrillation on an ambulatory
electrocardiogram (ECG) monitor.

Her other medical problems include hypertension, diabetes mellitus, dyslipidemia, and obstructive
sleep apnea. Her medications include hydrochlorothiazide (HCTZ) 12.5 mg daily, metoprolol
succinate 50 mg once daily, metformin 500 mg twice daily, rivaroxaban 20 mg daily, atorvastatin 40
mg daily, and zolpidem 5 mg at bedtime. She reported that she wears a continuous positive airway
pressure (CPAP) mask nightly. She drinks 1 alcoholic beverage nightly and admitted to smoking 1/2
pack cigarettes daily, although she is trying to quit.

On examination, she was 5'2" and 160 lbs (body mass index [BMI] 29.3 kg/m2). Her blood pressure
was 128/70 mm Hg, heart rate 76 bpm. Her lungs were clear, heart regular without murmurs, and
extremities were without edema. ECG showed normal ventricular and valvular function with no
pulmonary hypertension. A treadmill stress test showed no ischemia at adequate heart rate. She
would like to avoid catheter ablation and asked what other interventions might help.

What intervention do you recommend?

A. Switch hydrochlorothiazide to valsartan 80 mg daily.
B. Change continuous positive airway pressure to bilevel positive airway pressure.
C. Weight loss of 15-20 lbs.
D. Smoking cessation.
E. Switch metformin to glyburide 5 mg daily.

Weight management is associated with a reduction in atrial fibrillation (AF) symptom burden. In
the LEGACY (Long-Term Effect of Goal-Directed Weight Management in an Atrial Fibrillation
Cohort) trial, 335 patients with BMI >29 kg/m2 were offered weight management assistance and
followed for 5 years. Weight loss of ≥10% of body weight resulted in a sixfold greater
probability of arrhythmia-free survival at 5 years. Thus, 15-20 lbs weight loss is the correct
answer.
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Treatment of obstructive sleep apnea (OSA) with CPAP appears to favorably impact AF
management.3,4 However, there is no evidence that switching CPAP to bilevel positive airway
pressure (BiPAP) for the treatment of OSA would help and this is not the correct answer.

Although smoking cessation has numerous benefits, the relationship between smoking cigarettes
and AF recurrence is not established and this is not the correct answer.

Whether different antihypertensive medications affect the development of AF independent of

blood pressure reduction is not clear. In the GISSI-AF (Gruppo Italiano per lo Studio Della
Sopravvivenza Nell'Infarto Miocardico–Atrial Fibrillation) trial, valsartan was not superior to
placebo in secondary prevention of AF at 1 year. In the ANTIPAF (Angiotensin II-Antagonist in
Paroxysmal Atrial Fibrillation) trial, olmesartan did not decrease AF burden compared to
placebo in patients without structural heart disease. Thus, switching HCTZ to an angiotensin-
receptor blocker (ARB) when the blood pressure is already optimally controlled is not the best
answer.

The presence of diabetes mellitus is a risk factor for the development of AF. An observational
study from Taiwan assessed the effects of metformin on the development of AF. In 645,710
patients over a 13-year follow-up, fewer patients taking metformin developed AF, suggesting
that metformin had a protective effect on the development of AF in patients with diabetes
mellitus. Thus, switching metformin to a different agent is not the right answer.
69. The correct answer: C
Key Point
Chronic contributors to AF, including hypertension, obstructive sleep apnea, and obesity, should be
addressed in all patients. Not infrequently, rate control and correction of these comorbidities can
resolve symptoms.

Question 70
70.A 77-year-old female with hypertension was brought to the emergency department by her
husband after a syncopal episode at home. Prior to the event, she had been standing in the kitchen
preparing dinner and feeling well; she denied any warning symptoms. The event was unwitnessed.
She only recalled awakening on the floor. There was no history of syncope. Her medications
included aspirin 81 mg and amlodipine 5 mg. An electrocardiogram was recorded (Figure 1). The
patient remained asymptomatic and initial laboratories were unremarkable.

Which of the following is the best next step in this patient's management?

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(Figure 1)
Modified from Roediger JE. Available at: https://en.wikipedia.org/wiki/

A. Start fludrocortisone.
B. Discontinue amlodipine.
C. Permanent pacemaker implantation.
D. Thirty-day event monitor.
E. Coronary angiography.

This patient experienced unheralded syncope and has second-degree atrioventricular (AV) block,
Mobitz II. In patients with symptomatic irreversible bradycardia due to sinus node dysfunction or AV
block, placement of a permanent pacemaker is indicated. An event monitor may be beneficial to
diagnose the cause of syncope in a patient with vague symptoms and no identifiable conduction
system disease. Discontinuing amlodipine would not be beneficial, as it has no significant action on
the sinus or AV nodes, and the conduction abnormality in this case is infranodal. Fludrocortisone
might be useful to treatment orthostatic hypotension but is not indicated for symptoms due to
conduction system disease. Finally, coronary angiography would not be indicated in this patient
without angina or other evidence of myocardial ischemia.

70. The correct answer: C

Key Point
Patients with symptomatic irreversible bradycardia due to SND or AV block require permanent
pacemaker implantation.

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Question 71
71.A 28-year-old male was referred by his primary care clinician for evaluation of a murmur and an
abnormal echocardiogram. He exercises regularly and denied any exertional symptoms. He is an
avid cyclist, takes no medications, and has no relevant medical history. His family history is
unremarkable.

On examination, his heart rate was 62 bpm with blood pressure of 135/80 mm Hg. Cardiac
auscultation revealed a soft, II/VI systolic ejection murmur loudest at the right upper sternal border
that augments with rising from a squatting position.

An electrocardiogram (ECG) revealed normal sinus rhythm and left ventricular hypertrophy with
repolarization changes. An echocardiogram demonstrated normal biventricular function with normal
left ventricle (LV) size. There was a severely thickened interventricular septum measuring 2.5 cm.
There was systolic anterior motion of the mitral valve with a peak left ventricular outflow tract (LVOT)
velocity of 3.8 m/sec; this increased to 5.0 m/sec with Valsalva.

Which of the following is the best next step?

A. Treadmill stress test.
B. Implantable loop recorder.
C. Repeat echocardiogram in 1 year.
D. Electrophysiology study.
E. Dobutamine stress test.

Risk factors for sudden death in hypertrophic cardiomyopathy include: sudden death in a first-degree
relative, maximal septal thickness >30 mm, unexplained syncope, low blood pressure during
treadmill stress testing, and nonsustained ventricular tachycardia (NSVT) on Holter. In this patient
with hypertrophic cardiomyopathy (HCM), more data about his risk of sudden death, including the
blood pressure response to exercise, are needed; thus, this is the correct answer. The degree of
LVOT obstruction is dynamic and affected by loading conditions and autonomic tone. Although some
studies have suggested an association between LVOT obstruction and sudden cardiac death, the
gradient alone is not a basis for decisions about implantable cardioverter-defibrillator (ICD) therapy
(Figure 1).

Repeating an echocardiogram in 1 year is the not the best answer because additional risk
stratification is required at this time.
Electrophysiology study is not used for risk stratification in HCM.
Dobutamine stress testing offers no additional information in patients with HCM. Additionally,
dobutamine would be expected to worsen the LVOT gradient, which could be dangerous.

Although an ambulatory rhythm monitor (i.e., Holter or event monitor) may be useful to assess for
NSVT as part of his risk stratification, there is no indication for an implantable recorder in this patient.

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(Figure 1)
*SCD risk modifiers include established risk factors and emerging risk modifiers

71. The correct answer: A

Key Point
In patients with other cardiomyopathies, such as hypertrophic cardiomyopathy, arrhythmogenic RV
cardiomyopathy, and sarcoidosis, and inherited channelopathies, such as long QT syndrome,
catecholaminergic polymorphic VT, and Brugada syndrome, ICD implantation is a Class I indication
for those patients who have survived a cardiac arrest due to VT/VF. An ICD may be considered to
be beneficial (Class IIa indication) in subgroups of patients with high risk factor profiles, for example,
hypertrophic cardiomyopathy with unexplained syncope, LV wall thickness =30 mm, or sudden
cardiac death in a first-degree relative.

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Question 72
72. A 52-year-old male was admitted with palpitations. His electrocardiogram (ECG) was obtained
(Figure 1). He has a history of hypertension and is active at baseline without symptoms of chest pain
or dyspnea. His examination was notable for tachycardia, jugular venous pressure of 5 cm H20, no
appreciable cardiac murmurs, clear lungs, and no lower extremity edema.

In addition to an echocardiogram, which of the following tests is indicated?

(Figure 1)

A. Metanephrines.
B. D-dimer.
C. Lyme antibody.
D. C-reactive protein.
E. Thyroid stimulating hormone.

In patients with atrial fibrillation, reversible causes should be investigated, including hyperthyroidism,
pericarditis, pulmonary embolism, and electrolyte abnormalities. An echocardiogram is an
appropriate test to determine if there is structural heart disease. Lyme disease can be associated
with atrioventricular (AV) block but is not considered a typical cause of atrial fibrillation. D-dimer for
pulmonary embolism and c-reactive protein testing for pericarditis are not indicated in patients
without symptoms of these diseases.

72. The correct answer: E

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Key Point
Wherever possible, reversible causes for AF should be sought and corrected. These include:
hyperthyroid state, pulmonary embolism, and pericarditis.

Question 73
73. A 25-year-old male with no significant past medical history presented to his primary care clinician
for a pre-employment physical examination. He has been active and has no complaints. His family
history was unavailable, as he was adopted. On physical examination, his blood pressure was
110/60 mm Hg with a heart rate of 60 bpm, and his examination was within normal limits. An
electrocardiogram (ECG) was obtained (Figure 1).

Which of the following activities would you recommend as possibly safe?

(Figure 1)

A. Hockey.
B. Raquetball.
C. Swimming.
D. Diving.
E. Golf.

Athletes with a definite diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) should
not participate in most competitive sports, with the possible exception of low-intensity class 1A
sports (Class III, Level of Evidence C).

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Class 1A sports are low in static and dynamic loads. Examples include billiards, bowling, cricket,
curling, golf, and riflery. All other options include higher dynamic and/or static loads (Figure 2).

(Figure 2)
Reproduced with permission from Levine BD, Baggish AL, Kovacs RJ, et al. Eligibility and
disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task
Force 1: classification of sports: dynamic, static, and impact: a scientific statement from the
American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015;66:2350-5.
73. The correct answer: E
Key Point
If arrhythmogenic right ventricular cardiomyopathy is diagnosed in an index case, family screening
should be considered. Implantable cardioverter-defibrillator therapy may be warranted. Patients
should avoid vigorous exercise, as this can clearly accelerate disease progression.

Question 74
74. A 72-year-old female presented with complaints of weakness and fatigue. She has a past history
significant for atrial fibrillation managed with rate control strategy, peripheral vascular disease, and
hypertension. She recently was discharged from the hospital following a recurrent episode of atrial
fibrillation requiring cardioversion. She was unclear what her home medications are, but knows she
was taking several new medications for her heart.
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Upon physical examination, she was confused and lethargic. Her blood pressure was 91/54 mm Hg
and pulse was 103 bpm. Physical examination was remarkable for a tachycardic rhythm and no
murmurs. She complained of blurred vision. An electrocardiogram (ECG) was obtained in the
emergency department (Figure 1).

The recent addition of which of the following medications to this patient's medication regime is most
likely responsible for her clinical findings and ECG changes?

A. Amlodipine.
B. Nadolol.
C. Dronedarone.
D. Labetalol.
E. Apixaban.

Dronedarone is a potent inhibitor of P-glycoprotein. When administered concomitantly with digoxin, it

may lead to substantial increases in digoxin steady-state levels. Coadministration of digoxin and
dronedarone requires a dose reduction in digoxin (as is also the case with amiodarone
coadministration). The ECG demonstrates bidirectional ventricular tachycardia, a classical finding in
digoxin toxicity. The patient is digoxin toxic and should be treated with digoxin immune fab (ovine).
The other agents are not inhibitors of P-glycoprotein and would not be expected to result in the
clinical condition described.

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74. The correct answer: C

Key Point
Elimination of antiarrhythmic drugs is via kidneys and hepatic P450 system. Important interactions
include amiodarone and warfarin (increased INR), and P-glycoprotein a renal transporter for drugs
such as digoxin.

Question 75
75. A 54-year-old female with a history of hypertension and surgical atrial septal defect repair
approximately 10 years prior presented to your office for urgent evaluation. She had been
experiencing palpitations that began suddenly the evening prior and that are associated with fatigue
and decreased exercise tolerance. Her only medication is lisinopril 20 mg daily. Her blood pressure
was 124/76 mm Hg, heart rate 100 bpm and regular. The remainder of her physical examination was
unremarkable. An electrocardiogram (ECG) was recorded (Figure 1).

Which of the following is the best next step in this patient's management?

(Figure 1)
Reproduced with permission from Ferreira R, Primo J, Adão L, et al. Late atypical atrial flutter after
ablation of atrial fibrillation. Rev Port Cardiol 2016;35:doi: 10.1016/j.repc.2015.10.005.

A. Catheter ablation.
B. Transesophageal echocardiogram.
C. Intravenous flecainide.
D. Start diltiazem.
E. Direct current cardioversion.
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Although definitive diagnosis frequently requires electrophysiology study and intracardiac mapping,
the history of prior surgical atrial septal defect repair and the concordant ECG flutter wave polarities
suggest that this patient has atypical atrial flutter. Atypical atrial flutter is a macroreentrant atrial
arrhythmia that does not utilize the cavo-tricuspid circuit present in typical atrial flutter, and therefore
is classified as “non–isthmus-dependent” atrial flutter. As the patient has been in symptomatic atrial
flutter for <48 hours, proceeding with direct-current (DC) cardioversion without performing
transesophageal echocardiogram (TEE) is reasonable. Compared to DC cardioversion, intravenous
flecainide and other antiarrhthmic medications exhibit poor cardioversion efficacy. Cather ablation
can be considered, but in general is more difficult in atypical atrial flutter, and the current guidelines
recommend reserving ablation for recurrent atypical atrial flutter after failure of at least 1
antiarrhythmic agent. Pursuing an acute rate control strategy by starting diltiazem is unlikely to be
very effective in this patient, as atrial flutter is notoriously difficult to rate control. Finally, as patients
with atrial flutter are thought to have the same thromboembolism risk as patients with atrial
fibrillation, they therefore should be anticoagulated similarly.

75. The correct answer: E

Key Point
Non–isthmus-dependent atrial flutter involves macro–re-entrant circuits elsewhere in the RA or LA
and may occur in a variety of clinical settings, including congenital heart disease, after cardiac
surgery, and after catheter ablation of AF.

Question 76
76. A 67-year-old female presented to your office with fatigue and dizziness. She had hypertension
and coronary artery disease with a prior right coronary artery stent. Her medications include aspirin
81 mg daily, atorvastatin 20 mg daily, and lisinopril 10 mg daily. On physical examination, her heart
rate was 60 bpm and blood pressure was 126/80 mm Hg with no orthostatic changes. There was no
nystagmus and the Epley maneuver did not elicit vertigo. She had a split S2. The remainder of her
examination was unremarkable. Her electrocardiogram (ECG) was recorded (Figure 1).

Which of the following is the best next step?

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(Figure 1)

A. Thirty-day event monitor.

B. Coronary angiography.
C. Permanent pacemaker.
D. Electrophysiological study.
E. Implantable loop recorder.

This patient's ECG demonstrates right bundle branch block (RBBB). Her symptoms of dizziness may
be due to intermittent advanced heart block, and ambulatory ECG monitoring may be helpful for
correlating her symptoms with ECG findings. An event recorder would be the best next step.

It would be premature to consider an implantable loop recorder prior to a noninvasive evaluation.

As the patient does not have evidence of symptomatic bradycardia or high-degree atrioventricular
(AV) block yet, there is no indication for a permanent pacemaker.

Electrophysiology study is not indicated without further evidence of high-degree AV block warranting
further invasive investigation of the conduction system.

The patient does not have any subjective or objective findings of ischemia to warrant coronary
angiography at this time.

76. The correct answer: A

Key Point
When evaluating patients with lesser degrees of bradycardia, symptom-rhythm correlation is
important. This can be achieved with ambulatory monitoring (Holter or similar). More prolonged
monitoring with an implantable subcutaneous monitor may be needed in some cases.

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Question 77
77. A 16-year-old female high school soccer player presented to your office for evaluation after an
abnormality was detected on her electrocardiogram (ECG), which was done as part of her high
school's athletic screening program (Figure 1).

What is the most appropriate next step?

(Figure 1)

A. Isoprotenolol challenge.
B. Procainamide challenge.
C. Exercise stress test.
D. Genetic testing.
E. Reassurance.

The ECG (Figure 1) demonstrates evidence of pre-excitation, likely as a result of an accessory

pathway. In this case, the patient has Wolff-Parksinon-White (WPW) pattern, as she is asymptomatic
but has an ECG pattern consistent with WPW. The prevalence of a WPW pattern on ECG is
estimated at 0.13-0.25% in the general population. WPW syndrome (pre-excitation on ECG
associated with symptomatic arrhythmia) is 10-100 times less common. Noninvasive exercise testing
for risk stratification is the next appropriate step (Class I recommendation). During exercise stress,
complete and abrupt disappearance of the delta wave is suggestive of a low-risk pathway (Figure 2).
Persistent pre-excitation, including gradual shortening of the delta wave, suggests a higher-risk
pathway capable of conducting at higher heart rates, placing the patient at risk for arrhythmias,
including ventricular fibrillation. An alternative approach is to proceed directly to electrophysiologic
study including procainamide challenge (Class IIa), but a noninvasive approach is preferable as the
first step. Isoproterenol may be useful for provocation of ventricular arrhythmias such as are seen in
arrhythmogenic right ventricular cardiomyopathy or as an adjunct to tilt table testing for syncope, but
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is not useful in WPW patients. Genetic testing does not have an established role in a WPW pattern.
It is not appropriate to reassure the patient without further risk stratification.

(Figure 2)
During exercise treadmill testing, this patient abruptly lost pre-excitation at a heart rate of 117 bpm.
Beginning abruptly with the beat under the arrow, the PR interval normalizes , and the QRS changes
from pre-excited to narrow.

77. The correct answer: C

Key Point
Genesis of the delta wave is due to “fusion” of impulses activating the ventricles via both the AV
node and activation via the accessory pathway. In other words, a delta wave is only present if the
accessory pathway can conduct from the atria to the ventricles. Factors that enhance AV nodal
conduction (such as exercise) cause predominantly more of the ventricles to be activated in a
normal fashion such that the delta wave will diminish with exercise, whereas factors that slow AV
nodal conduction such as drugs will increase the degree of pre-excitation. In other words, the degree
of pre-excitation can change and is not fixed.

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Question 78
78.A 22-year-old male with arrhythmogenic right ventricular cardiomyopathy presented to the clinic to
establish care. His diagnosis was made based on the findings of symptomatic nonsustained
ventricular tachycardia, an electrocardiogram with T-wave inversions in leads V1-V3, and a cardiac
magnetic resonance imaging with an elevated right ventricular end-diastolic volume and regional
hypokinesis. He subsequently underwent implantation of an implantable cardioverter-defibrillator.
During discussion of management of his condition, he asked for advice regarding lifestyle
interventions.

Which of the following lifestyle modifications do you suggest?

A. Limit alcohol.
B. Avoid air travel.
C. Limit competitive sports participation.
D. Limit caffeine.
E. Restrict fluid intake.

Arrhythmogenic cardiomyopathy is a progressive, heritable disease of cardiac muscle resulting in a

high risk of ventricular arrhythmias and sudden death, even when cardiac structural changes are
minimal. Based on the increased risk of cardiac arrest and sudden death in athletes with
arrhythmogenic cardiomyopathy, patients are advised to avoid competitive sports and endurance
training. Physical activity may accelerate structural progression of arrhythmogenic cardiomyopathy;
in a study of heterozyogous plakoglobin- deficient mice, endurance training accelerated the
development of arrhythmias and right ventricular dysfunction. Although caffeine, alcohol,
dehydration, and mild hypoxia may trigger arrhythmia in individuals, there are no known specific
links to arrhythmogenic cardiomyopathy.

78. The correct answer: C

Key Point
If arrhythmogenic right ventricular cardiomyopathy is diagnosed in an index case, family screening
should be considered. Implantable cardioverter-defibrillator therapy may be warranted. Patients
should avoid vigorous exercise, as this can clearly accelerate disease progression.

Question 79
79. A 65-year-old woman presented for follow-up in the heart failure clinic. She was able to do her
usual activities, but was limited when trying to walk upstairs. She denied any chest pain. Her past
medical history includes systolic heart failure (left ventricular ejection fraction 30%) for the past 3
years, coronary artery disease with an anterior wall myocardial infarction 5 years ago, type 2
diabetes mellitus, hyperlipidemia, and hypertension. Her medications include lisinopril 20 mg daily,
carvedilol 25 mg BID, atorvastatin 40 mg daily, aspirin 81 mg daily, and metformin 850 mg BID. On
examination, her blood pressure was 100/70 mm Hg and pulse was 70 bpm. Her examination was
otherwise unremarkable and euvolemic. Her electrocardiogram showed sinus rhythm at 70 bpm,
with a left bundle branch block (LBBB) and QRS duration of 155 msec.
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In addition to referring her to cardiac rehabilitation, which of the following is the best next step in this
patient's care?

A. Refer for cardiac transplantation.

B. Add torsemide.
C. Implant cardioverter defibrillator.
D. Implant biventricular defibrillator.

The guidelines for biventricular pacing specify the type of QRS complex, QRS duration, and New
York Heart Association (NYHA) functional class. Class I indication for defibrillator includes an EF of
35% or less with NYHA class II or III symptoms.

This patient also has a class I indication for biventricular pacing with class II, III, or ambulatory IV
heart failure, LBBB, and QRS duration of 150 ms or greater, which is why a defibrillator alone would
not be the best choice.

She is euvolemic and does not need diuresis.

Her NYHA class II symptoms and good functional capacity do not merit advanced therapy (left
ventricular assist device or heart transplantation) at this time.

79. The correct answer: D

Question 80
80. A 75-year-old female with hypertension and atrial fibrillation presented to your office for
consultation. She felt well with no symptomatic palpitations or dyspnea. Her medications include
carvedilol 6.25 mg twice daily, lisinopril 10 mg daily, and rivaroxaban. On examination, her blood
pressure was 105/70 mm Hg, her heart rate was 98 bpm, and she was afebrile. Cardiac examination
revealed irregularly irregular rhythm and the remainder of her examination was unremarkable. Her
electrocardiogram showed atrial fibrillation at approximately 100 bpm and left bundle branch block. A
nuclear myocardial perfusion scan performed 6 months prior showed a normal left ventricular
ejection fraction (LVEF) without perfusion defects.

Which of the following is the most appropriate next step in the management of this patient?
A. Add dofetilide.
B. No change in therapy.
C. Add diltiazem.
D. Add digoxin.
E. Atrial fibrillation ablation.

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This patient has asymptomatic, rate-controlled atrial fibrillation. In the absence of symptoms and with
normal LVEF, a strategy of rate control and anticoagulation is reasonable.
The RACE-II Study compared rate control with a target resting heart rate <80 bpm to a rate control
strategy with a target resting heart rate <110 bpm in patients with permanent atrial fibrillation and
found similar rates of major adverse events with both strategies. Therefore, adding additional rate-
controlling drugs such as diltiazem or digoxin may be associated with increased side effects without
clear benefits in a low-risk, asymptomatic patient.

The AFFIRM Study and RACE-I Study both demonstrated that rate and rhythm control strategies
have similar major adverse event rates. Similarly, ablation of atrial fibrillation would not be expected
to have meaningful benefits for this asymptomatic patient with normal LVEF.

80. The correct answer: B

Key Point
The main goal in treating AF is to address symptoms thought to be related to AF. Rate or rhythm
control seems to be equivalent in terms of mortality. Selection of a therapeutic strategy takes into
account factors that include age, symptoms, duration of AF, evidence of tachycardia-mediated
cardiomyopathy, or difficulty in achieving adequate rate control.

Question 81
81.A 42-year-old woman with fibromyalgia presented to your office with weakness, fatigue,
lightheadedness, and palpitations. Her wearable heart rate monitor reported sinus rhythm with heart
rates ranging from 90 to 120 bpm at rest that she said correlated with her symptoms. She had
previously been evaluated with normal thyroid function tests, blood counts, and electrolytes. Her
electrocardiogram is shown (Figure 1). She had previously tried metoprolol, atenolol, and nebivolol,
which all caused debilitating fatigue.

Which of the following is the best treatment?

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(Figure 1)

A. Ivabradine 5 mg BID.
B. Propafenone 225 mg BID.
C. Digoxin 0.125 mg daily.
D. Radiofrequency ablation.
E. Cardiac rehabilitation.

While ivabradine is not FDA-approved for treatment of inappropriate sinus tachycardia, the 2015
heart rhythm society expert consensus statement on the diagnosis and treatment of postural
tachycardia syndrome, inappropriate sinus tachycardia, and vasovagal syncope (Sheldon RS et al
Heart Rhythm 2015) gives ivabradine a Class IIa recommendation for this indication: "Ivabradine can
be useful for treating patients with IST." Ivabradine holds considerable promise for the treatment of
IST. The drug blocks the If current and has a dramatic and generally well-tolerated effect on heart
rate. At doses of 5– 7.5 mg twice daily, the drug slows the heart rate by 25–40 bpm. The strongest
evidence to date comes from a randomized crossover study in which 21 patients with IST were
randomized to placebo or ivabradine 5 mg twice daily for a total of 12 weeks. ß-Adrenergic blockers
are not usually effective and can cause adverse effects. Digoxin and propafenone are not
recommended for treatment of IST. Other treatments have been suggested, including
fludrocortisone, volume expansion, pressure stockings, phenobarbital, clonidine, psychiatric
evaluation, exercise training, and erythropoietin, but have not proven useful and are not specific
recommended in the guidelines. Modification or ablation of the sinus node can be performed IST.
However, there is a high rate of symptom recurrence, and the complication rates are significant,
including the need for permanent pacing, phrenic nerve paralysis, and transient superior vena cava
syndrome. Given the young age of the patients and the highly invasive nature of ablations, we do not
recommend that they be part of routine care.

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81. The correct answer: A

Key Point
If (for “funny current”) is located in the SA node and drives sinus rate.

Question 82
82. A 64-year-old male presented to the clinic for evaluation of an abnormal electrocardiogram
(ECG) (Figure 1). His past medical history was significant for hypertension, for which he is treated
with lisinopril. He denied cardiac symptomatology. He is active, works full-time as a car salesman,
and plays golf regularly. His examination was unremarkable. His laboratories, including thyroid
stimulating hormone (TSH), were normal.

Which of the following is the best next step?

A. Carotid sinus massage.

B. Echocardiogram.
C. Vasodilator perfusion imaging stress test.
D. Dual-chamber pacemaker.
E. Cardiac magnetic resonance imaging.

The ECG is consistent with sinus rhythm with 2:1 atrioventricular (AV) block. The differential is
Mobitz type 1 (Wenckebach) second-degree AV block versus Mobitz type 2 second-degree AV
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block. His ECG is more suggestive of Mobitz type 1 second-degree block based on the long PR
interval and narrow QRS interval, but the diagnosis still needs to be established because higher-
grade AV block would merit further investigation and potential pacemaker placement.

Noninvasive vagal and sympathetic maneuvers can help to distinguish one from the other because
Mobitz type 1 block is usually AV nodal, and Mobitz type 2 block is usually in the His-Purkinje
system. In a patient with AV nodal block, carotid sinus massage will slow the sinus rate and worsen
AV conduction, which will worsen the AV block. The slowing of sinus rate and depression of AV
conduction will have a protective effect on the distal conduction system, so conduction in the His-
Purkinje system may improve. Thus, carotid sinus massage is the correct answer.

Alternatively, exercise (sympathetic stimulation) will improve AV nodal conduction and worsen His-
Purkinje conduction. Thus, an exercise stress test may be helpful but a vasodilator perfusion
imaging stress test would offer no further information and is contraindicated based on his conduction
disease.
Although the detection of structural heart disease may help guide patient therapy, it is not the best
next diagnostic maneuver to assess the degree and level of AV block.
If the site of block is within the AV node in an asymptomatic patient, a pacemaker is not indicated.
Cardiac magnetic resonance imaging (MRI) may help establish the etiology if infraHisian block is
identified, but further information is needed before proceeding with an MRI.

82. The correct answer: A

Key Point
AV block occurs due to a failure of impulse propagation through the cardiac conduction system.
Several types of AV block are recognized; most commonly first-, second-, and third-degree block.
Prognosis of AV block is related to the severity of block. AV block may be due to failure of impulse
propagation in the AV nodal tissues (narrow QRS complex) or within the His-Purkinje system (intra-
or infra-Hisian block). Failure of impulse propagation due to infra-Hisian block may be associated
with a wide QRS complex with bundle branch or fascicular block.

Question 83
83.A 73-year-old man with a dual-chamber pacemaker placed 5 years ago for sinus node
dysfunction presented to your clinic with increasing dependent edema and paroxysmal nocturnal
dyspnea. His past medical history includes hypertension, diabetes mellitus, and obesity. His current
medications are aspirin 81 mg, atorvastatin 80 mg, lisinopril 10 mg, metoprolol succinate 100 mg,
and glyburide 5 mg. His height was 5'6", weight 200 lbs, pulse 50 bpm, blood pressure 110/80 mm
Hg, and respiration 12 breaths per minute. The jugular veins were not visible, the lungs were clear,
the heart was regular with paradoxical splitting of S2, and the ankles were swollen with chronic
venous stasis changes.

An echocardiogram showed dilated left ventricle with severe global left ventricular systolic
dysfunction, estimated left ventricular ejection fraction 25%. There was moderate functional mitral
regurgitation

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What is the best next step?

A. Endomyocardial biopsy.
B. Pacemaker interrogation.
C. Polysomnography.
D. Cardiac magnetic resonance imaging.
E. Serum and urine immunofixation electrophoresis.

The correct answer is pacemaker interrogation. Although the device was placed for sinus node
dysfunction, it would be important to assess his percentage of RV pacing. He may have developed
heart block since implantation or have been programmed inappropriately resulting in a high
percentage of RV pacing. Chronic right ventricular pacing may result in the development of heart
failure, occurring in up to 12% of patients who are paced >20% of the time, and this should be
considered since it is reversible. If you are not able to decrease the percentage of RV pacing by
programming changes, upgrade to a biventricular pacing system is indicated in such patients.
Although other causes of cardiomyopathy such as amyloid are possible, testing for amyloid would
not be the first step. Obstructive sleep apnea can worsen heart failure but would be not be likely to
explain his severe left ventricular dysfunction. Cardiac magnetic resonance imaging can be useful
for diagnosing infiltrative heart disease but must be performed with caution in patients with pacing
systems. Furthermore, conditions such as cardiac sarcoidosis or hemochromatosis would be unlikely
to present in a patient at this age. Endomyocardial biopsy is generally low-yield and not indicated in
this situation.

83. The correct answer: B

Key Point
Prolonged RV apical pacing may in some cases cause a deleterious effect on ventricular function:
DAVID trial. Ventricular pacing alone may also increase the likelihood of AF.

Question 84
84. A 76-year-old male with a history of atrial fibrillation, hypertension, diabetes mellitus, stroke 2
months prior, and a dual-chamber pacemaker for tachy-brady syndrome was scheduled to undergo
pacemaker generator change. His medications include Lisinopril 20 mg daily, metformin 850 mg
twice daily, metoprolol succinate 50 mg daily, and warfarin 5 mg daily.

Which of the following is the most appropriate periprocedure anticoagulation management strategy?
A. Hold warfarin 5 days prior.
B. Replace warfarin with dabigatran (150 mg twice daily).
C. Continue warfarin.
D. Bridge with enoxaparin.
E. Replace warfarin with aspirin (325 mg daily).

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Management strategies for periprocedure anticoagulation are based on the risk-benefit assessment
including the indication for anticoagulation and the risk of bleeding from the procedure. In this case,
the patient has a CHA2DS2-VASc score of 6 with a recent prior stroke and therefore continuing
warfarin is the most appropriate strategy.

Withholding all anticoagulation is not appropiate is this high-risk patient.

Substituting with aspirin offers inferior thromboembolic protection and is thus not recommended.

Enoxaparin carries a higher postprocedure bleeding risk as demonstrated in the BRUISE

CONTROL (Bridge or Continue Coumadin for Device Surgery Randomized Controlled
Trial) and would not be indicated.

There is no evidence that periprocedure dabigatran offers less bleeding risk than continuing current
therapy with warfarin and therefore is not the correct choice.

84. The correct answer: C

Key Point
The CHA2DS2-VASc and HAS-BLED score decisions are used to balance the need for long-term
anticoagulant therapy against the risk of bleeding, respectively. These scoring systems are best
utilized in patients with nonvalvular AF, and do not apply to other special populations, such as those
with hypertrophic cardiomyopathy.

Question 85
85. A 43-year-old male with a history of tobacco use and hypertension presented to the emergency
department with chest discomfort that radiated to the back. His chest pain began an hour prior to his
arrival while eating dinner with his family. He denied any shortness of breath, palpitations, or
nausea. His family history is notable for his father dying suddenly at age 50. On physical
examination, he was diaphoretic. His heart rate was 90 bpm and blood pressure was 150/95 mm Hg.
There were no murmurs and his lungs were clear. His abdomen was mildly distended with normal
bowel sounds. His initial electrocardiogram showed normal sinus rhythm with no ST or T-wave
abnormalities. His first troponin was negative. He was placed on a monitor. Ten minutes later, he
became unresponsive. His rhythm strip was obtained (Figure 1).

Which of the following is the most likely etiology of the rhythm shown?

(Figure 1)
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A. Long QT syndrome.
B. Brugada syndrome.
C. Acute myocarditis.
D. Myocardial ischemia.
E. Arrhythmogenic right ventricular cardiomyopathy.

The rhythm strip shows ventricular fibrillation (VF). The most common cause of VF and polymorphic
ventricular tachycardia is acute myocardial ischemia. The patient presented with chest pain due to
an acute coronary syndrome. His ongoing chest pain with a normal ECG can be seen with an acute
circumflex artery occlusion. He has multiple risk factors for coronary disease including tobacco use,
hypertension, and family history of sudden cardiac death (presumably due to a myocardial
infarction). Acute myocarditis, long QT syndrome, arrhythmogenic right ventricular cardiomyopathy
(ARVC), and Brugada syndrome can cause VF but are less compatible with his clinical presentation.

85. The correct answer: D

Key Point
Ventricular arrhythmia may be either monomorphic with a single QRS morphology or polymorphic
with changing QRS morphology. The most common cause of polymorphic ventricular tachycardia
(PVT) is acute myocardial ischemia, which may quickly progress to ventricular fibrillation (VF). Less
commonly, PVT is caused by circumstances that prolong the Q-T interval (drugs and ion-channel
disorders). It is important to recognize which common drugs are associated with Q-T prolongation
and discontinue.

Question 86
86.A 33-year-old male with no significant past medical history was admitted to the hospital with three
days of fevers and chills. His current medication regimen includes multivitamins. His family history
was significant for hypertension. On admission, he was febrile with a temperature of 39.0°C and
blood pressure of 100/60 mm Hg. His physical examination revealed diminished breath sounds in
left lower lung fields and was other wise unremarkable. A chest radiograph confirmed the diagnosis
of community-acquired pneumonia. A previous outpatient electrocardiogram (ECG) was available
(Figure 1); his admission ECG was also recorded (Figure 2).

What is the most appropriate next step in his management?

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(Figure 1)

(Figure 2)

A. Aggressively treat fever.

B. Genetic testing.
C. Implantable cardioverter defibrillator.
D. Provocative testing.
E. Stress echocardiography.
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Brugada syndrome (BrS) is an example of a channelopathy, a disease caused by alteration in the

transmembrane ion currents that form the action potential. Patients with BrS are prone to developing
ventricular tachyarrythmias that may lead to syncope, cardiac arrest, or sudden cardiac death
(SCD). Diagnosis of BrS requires the characteristic ECG pattern along with clinical history such
as unexplained syncope or family history of premature unexplained sudden
death. Febrile illnesses, alcohol or cocaine use, and certain medications may unmask a Brugada
pattern on ECG. The most important intervention at this point is to treat the fever.

Several pathogenic genes have been identified as associated with the disease, but SCN5A is the
most prevalent, present in nearly 30% of cases.1 Mutation-specific genetic testing for this patient is
not recommended, as he has a diagnostic type I Brugada pattern on his ECG but does not have a
history of unexplained syncope or family history of premature unexplained sudden death, and thus
does not meet criteria for BrS. In patients who are asymptomatic or with nondiagnostic ECGs, it
would be reasonable to proceed with intravenous administration of Na+ channel blocking drugs like
ajmaline or flecainide to elicit a type I Brugada pattern. An implantable cardioverter-defibrillator
(ICD) is not indicated in asymptomatic BrS patients with a drug-induced type I ECG pattern or on the
basis of family history of SCD alone.2 Stress testing would not add diagnostic information in this
setting.

86. The correct answer: A

Key Point
Asymptomatic BrS patients without a spontaneous type I pattern (or type 2 or 3 pattern) are at low
risk, and an ICD is NOT indicated, even if an Na channel-blocking drug evokes a type I pattern.

Question 87
87. A 22-year-old man with surgically repaired tetralogy of Fallot presents for evaluation of
palpitations for the past few months with heart rates of 120-130 bpm lasting a few hours at a time.
He notes that his exercise capacity has worsened as well.

He wore a cardiac event monitor prior to this visit, which demonstrated a 2-hour episode of atrial
flutter with variable atrioventricular (AV) conduction, correlating with his symptoms.

His cardiac examination today reveals a regular rate and rhythm, no gallop, and a grade 2/6 systolic
ejection murmur. Electrocardiogram demonstrates sinus rhythm with a first-degree AV block and
right bundle branch block with QRS 140 msec.

Which of the following is the most appropriate next step?

A. Transthoracic echocardiogram.
B. Sotalol 120 mg twice daily.
C. Radiofrequency catheter ablation.
D. Metoprolol 25 mg twice daily.
E. Diagnostic cardiac catheterization.

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While treatment with medications or ablation may eventually be appropriate in this patient, the most
important first step is to reassess his structural and hemodynamic status. The development of atrial
tachycardia, atrial flutter, or atrial fibrillation in adult congenital heart disease patients is often
associated with progressive hemodynamic deterioration of the underlying disease. Thus,
echocardiogram is warranted. Surgical treatment of the hemodynamic problems does not eliminate
atrial arrhythmias, and ablation of atrial arrhythmias alone could allow significant hemodynamic
issues to progress and potentially deteriorate. Successful treatment involves assessing both the
arrhythmia and the contributing hemodynamic changes and addressing both when indicated and
feasible. Cardiac catheterization may be appropriate to perform more detailed hemodynamic
assessment, but in light of his unchanged physical examination, echocardiography will provide
important information without the need for an invasive procedure.

Early experience in adults with unoperated atrial septal defects and atrial arrhythmias demonstrated
the importance of an integrated approach for arrhythmia and hemodynamic problems; similar
principles apply to patients with tetralogy of Fallot, Ebstein’s anomaly, and single-ventricle
physiology; these patients are at highest risk of arrhythmia development, with concurrent
hemodynamic abnormalities. Beta-blockers may decrease catecholamine-related triggers and
provide AV nodal blockade during recurrent atrial arrhythmias. One study of adults with transposition
of the great arteries and prior atrial switch repairs with implanted defibrillators demonstrated
supraventricular tachycardia preceding ventricular tachycardia in 50% of patients; use of beta-
blocker medications in this population was associated with decreased incidence of appropriate
defibrillator shocks.

Observational studies on the use of sotalol in ACHD patients report freedom from recurrent atrial
tachycardia in 41-46% of patients during short-term follow-up. Use of either medication in the setting
of significant sinus node dysfunction may exacerbate bradycardia and requires careful monitoring.
Initiation of sotalol in this population is recommended during inpatient monitoring for proarrhythmia
for 48-72 hours. Multiple observational and multicenter studies have demonstrated acute success
rates of 65-100% for treatment of supraventricular tachycardia associated with ACHD. Acute
success rates vary by tachycardia mechanism and type of congenital heart disease and repair.

87. The correct answer: A

Key Point
Non–isthmus-dependent atrial flutter involves macro–re-entrant circuits elsewhere in the RA or LA
and may occur in a variety of clinical settings, including congenital heart disease, after cardiac
surgery, and after catheter ablation of AF.

Question 88
88. A 60-year-old woman came to the emergency department with palpitations. She described
irregular and racing heart beats as well as exertional shortness of breath starting the prior day . She
has also had 1-2 weeks of a tremulous and jittery feeling, sweating, and difficulty sleeping. Her past
medical history includes hypertension, asthma, and depression with anxiety.
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Her current medications are lisinopril 10 mg, hydrochlorothiazide 25 mg, and extended-release
venlafaxine 150 mg. Her vital signs were pulse 115 bpm, respiration 15 breaths per minute, and
blood pressure 125/62 mm Hg. Her physical examination revealed an anxious-appearing female.
The lung fields showed mild expiratory wheezes and her heart rate was rapid and irregular without
murmurs or rubs.

The electrocardiogram is shown (Figure 1). A bedside ultrasound showed hyperdynamic left
ventricular systolic function and a trivial pericardial effusion. Laboratories included potassium 4.1
mEq/L, creatinine 0.9 mg/dL, thyroid-stimulating hormone 0.1 mIU/L (normal 0.5-5.0 mIU/L), and
free thyroxine 19.8 ng/dL (normal 0.7-1.9 ng/dL).

Which of the following would be the most appropriate for restoring sinus rhythm in this patient?

(Figure

A. Amiodarone.
B. Colchicine.
C. Disopyramide.
D. Propylthiouracil.
E. Propranolol.

The correct answer is treatment of hyperthyroidism with either propylthiouracil or methimazole. Atrial
fibrillation is a common complication of hyperthyroidism, occurring in 5-15% of cases. Antiarrhythmic
drugs, including disopyramide and amiodarone, and electrical cardioversion are unlikely to be
successful in hyperthyroid states and normalization of the thyroid function is usually associated with
spontaneous return of normal sinus rhythm. Additionally, amiodarone must be used cautiously in
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patients with present or prior thyroid disease because of its iodine component. Beta-blockers are
first-line agents for rate control in atrial fibrillation associated with hyperthyroidism, but would not be
the best choice in this patient with active wheezing. Colchicine is effective for treating pericarditis,
which can also trigger atrial fibrillation, but this patient does not have the triad of pleuritic or
positional chest pain, ST elevations, and pericardial friction rub to support a diagnosis of pericarditis.
The 2014 American College of Cardiology/American Heart Association/Heart Rhythm Society
(ACC/AHA/HRS) Guideline for the Management of Patients With Atrial Fibrillation adds that "many
potentially 'reversible' causes of AF have been reported, including binge drinking, cardiothoracic and
noncardiac surgery, myocardial infarction (MI), pericarditis, myocarditis, hyperthyroidism,
electrocution, pneumonia, and pulmonary embolism. It is important to recognize that there are few
data to support the notion that patients with AF that occurs in the setting of one of these potentially
'reversible' conditions are, in fact, cured of AF after effective treatment or elimination of the condition.
Since long-term follow-up data are not available in these clinical scenarios and AF may recur, these
patients should receive careful follow-up."

88. The correct answer: D

Key Point
Wherever possible, reversible causes for AF should be sought and corrected. These include:
hyperthyroid state, pulmonary embolism, and pericarditis.

Question 89
89. A 50-year-old male underwent routine appendectomy. Shortly following extubation in the
recovery room, he was briefly unresponsive and a tracing (Figure 1) was retrieved from the monitor.
He presently was sleepy but responsive, acknowledging abdominal pain. A review of preoperative
evaluation showed a normal stress electrocardiogram 6 months before and no prior history of heart
disease. He received a dose of fentanyl 30 minutes ago. The last serum potassium was 3.6 mEq/dl.

Which of the following should be done prior to hospital discharge?

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A. Catheter ablation.
B. Implantable cardioverter-defibrillator placement.
C. Chronic amiodarone therapy.
D. Coronary angiography.
E. No additional evaluation is required.

The tracing shows sinus rhythm for five beats, followed by a premature ventricular contraction that
initiates polymorphic ventricular tachycardia (VT) degenerating to ventricular fibrillation (VF) (Figure
1). Marching the QRS complexes forward into the arrhythmia does not suggest artifact and the
patient was briefly unresponsive, consistent with an episode of hypotension, with spontaneous
termination of the arrhythmia, which can occur with polymorphic VT and even VF. Polymorphic VT
due to QT prolongation is a consideration, but the QT interval is not prolonged. Furthermore, the
episode is not preceded by a pause or transient slowing of heart rate, as is usually seen with
acquired long QT syndrome. The most likely cause is myocardial ischemia, which may be transient
and needs to be evaluated. Thus, coronary angiography is the correct answer and should be
immediately considered, taking into consideration risks imposed by the recent surgery and any
additional evidence suggesting an acute coronary syndrome. VF due to an acute ischemic syndrome
usually does not warrant placement of an implantable cardioverter-defibrillator. Catheter ablation is
rarely performed for polymorphic VT, and then only for recurrent episodes with premature ventricular
contraction triggers that are not due to acute ischemia. Although acute intravenous amiodarone
therapy could be considered, chronic oral therapy should not be initiated at this time.

89. The correct answer: D

Key Point
Ventricular arrhythmia may be either monomorphic with a single QRS morphology or polymorphic
with changing QRS morphology. The most common cause of polymorphic ventricular tachycardia
(PVT) is acute myocardial ischemia, which may quickly progress to ventricular fibrillation (VF). Less
commonly, PVT is caused by circumstances that prolong the Q-T interval (drugs and ion-channel
disorders). It is important to recognize which common drugs are associated with Q-T prolongation
and discontinue.

Question 90
90. A 26-year-old female with no history presented to your office for evaluation of palpitations. For
the past year, she has had episodes of a racing heart lasting ≤30 minutes, most often at night,
without clear triggers. The episodes seem to start and stop abruptly and are associated with
lightheadedness. She does not drink caffeinated beverages or alcohol. She denied use of illicit
substances, vitamins, or supplements.

On examination, she was a healthy young female in no distress. Her heart rate was 68 bpm and
blood pressure 110/60 mm Hg. Her examination was unremarkable.
She had an episode in your office, which you captured on electrocardiogram (ECG) (Figure 1).

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What do you recommend to this patient?

(Figure 1)

A. Catheter ablation.
B. Digoxin 0.125 mg daily.
C. Cardioversion.
D. Education on vagal maneuvers.
E. Metoprolol succinate 25 mg daily.

The ECG (Figure 1) shows atrial tachycardia. Atrial tachycardia originates from a discrete atrial
origin discharging at a rate that is generally regular at 100-250 bpm. Catheter ablation is preferred
over medical therapy, as it is more effective (Figure 2). Thus, ablation is the correct answer.

Metoprolol would not be preferred over catheter ablation. Digoxin is generally ineffective for atrial
tachyarrhythmias and has not been studied in focal atrial tachycardia.

Vagal maneuvers are not indicated, as this is not vasovagal syncope.

Cardioversion is not indicated, as the arrhythmia is paroxysmal and spontaneously converts to sinus
rhythm with brief episodes.

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(Figure 2)
Reproduced with permission from Page RL, Joglar JA, Caldwell MA, et al. 2015 ACC/AHA/HRS
guideline for the management of adult patients with supraventricular tachycardia: a report of the
American College of Cardiology/American Heart Association Task Force on Clinical Practice
Guidelines and the Heart Rhythm Society. J Am Coll Cardiol 2016;67:e27-e115.

90. The correct answer: A

Key Point
Catheter ablation is the preferred treatment option for recurrent symptomatic PSVT – success rates
exceed 95% in good centers. Drug therapy (prophylaxis or as the occasion arises with drugs that
block AV node or antiarrhythmic drugs) is rarely effective or helpful.

Question 91
91. A 76-year-old woman has permanent atrial fibrillation. She has a history of hypertension,
diabetes, dyslipidemia, and osteoarthritis. Her medications include apixaban 5 mg twice daily,
metoprolol succinate 25 mg twice daily, metformin 500 mg twice daily, and atorvastatin 40 mg daily.

She would like to stop anticoagulation because she bruises easily.

What is this patient's estimated annual stroke risk without anticoagulation?

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A. 21%.
B. 7%.
C. 1%.
D. 15%.
E. 3%.

There is a class I indication for using the CHA2DS2-VASc score to quantify annual stroke risk in
patients with non-valvular atrial fibrillation. This patient has a CHA2DS2-VASc score of 5 (Figure 1),
which corresponds to an annual stroke risk of 6.7%. Thus, 7% is the best answer.

(Figure 1)
*These adjusted stroke rates are based on data for hospitalized patients with AF and were published
in 2001. Because stroke rates are decreasing, actual stroke rates in contemporary nonhospitalized
cohorts might vary from these estimates.

†Adjusted stroke rate scores are based on data from Lip and colleagues. Actual rates of stroke in
contemporary cohorts might vary from these estimates.

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91. The correct answer: B

Key Point
The CHA2DS2-VASc and HAS-BLED score decisions are used to balance the need for long-term
anticoagulant therapy against the risk of bleeding, respectively. These scoring systems are best
utilized in patients with nonvalvular AF, and do not apply to other special populations, such as those
with hypertrophic cardiomyopathy.

Question 92
92.A 40-year-old female was admitted to the hospital with palpitations, diaphoresis, and
lightheadedness. In the emergency department, she was hypotensive and tachycardic; an
electrocardiogram (ECG) was obtained (Figure 1). She was cardioverted to sinus rhythm with relief
of symptoms. Her evaluation included a normal basic metabolic panel, blood counts, and troponin.
Her echocardiogram was normal with normal chamber sizes and no valvular lesions. Her cardiac
magnetic resonance imaging (MRI) did not show any areas of delayed gadolinium enhancement or
structural abnormalities of the heart chambers.

Which of the following is the best next step in her care?

(Figure 1)

A. Propranolol.
B. Cardiac sympathetic denervation.
C. Implantable cardioverter-defibrillator.
D. Mexilitine.
E. Ablation of accessory pathway.

Sustained monomorphic ventricular tachycardia (VT) in the setting of a structurally normal heart
does not necessarily benefit from implantable cardioverter-defibrillator therapy, and preventive
therapy of the arrhythmia is most appropriate. To this end, beta-blockers, calcium channel blockers,
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and antiarrhythmic medications as well as catheter ablation are considered first-line therapies
(Figure 2). This patient has right ventricular outflow tract (RVOT) VT, which can be treated with beta-
blockers, calcium channel blockers, Class Ic antiarrhythmic drugs, or catheter ablation. Cardiac
sympathetic denervation could be effective for catecholaminergic polymorphic ventricular
tachycardia or long QT syndrome, but not for this patient with RVOT VT. Mexilitine, a Class Ib
antiarrhythmic drug, is used for re-entrant or scar-mediated ventricular arrhythmias and would not be
expected to be effective in preventing RVOT VT. Catheter ablation of accessory pathway tracts is
used for Wolff-Parkinson-White syndrome, which is not the correct diagnosis here.

(Figure 2)
ICD = implantable cardioverter-defibrillator; SHD = structural heart disease; VT = ventricular
tachycardia.
92. The correct answer: A
Key Point
Distinction of ventricular tachycardia in patients with or without structural heart disease is
importantsince therapeutic approaches are very different. In patients with structural heart disease,
an implantable cardioverter-defibrillator is generally recommended (secondary prevention); in
patients without structural heart disease, drug therapy or catheter ablation may be effective in
reducing or eliminating the arrhythmia.

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Question 93
93. A 21-year-old male college hockey player was referred for evluation of intermittent palpitations
and a recent episode of near syncope during practice. He reported infrequent episodes of
palpitations dating back ≥10 years. However, for the past couple of years, he has noted that his
symptoms have been more frequent and occur at least once a month. He describes a "rapid and
strong beating in my chest," which is occasionally associated with lightheadedness and presyncope,
although he has never lost consciousness. His symptoms last only "a couple of minutes" and
generally resolve with Valsalva. He has not been able to participate in athletics since his most recent
episode of near syncope and is anxious to return as soon as is safely possible.

He has no prior relevant medical history and takes no medications.

An electrocardiogram (ECG) was obtained (Figure 1).

What is the most appropriate next step?

(Figure 1)

A. Prescribe verapamil.
B. Order a 48-hour Holter monitor.
C. No further intervention or therapy.
D. Referral for catheter ablation.
E. Referral for treadmill stress test.

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Based on the patient's ECG, which demonstrates pre-excitation, and his symptoms, he is considered
to have Wolff-Parkinson-White (WPW) syndrome. Catheter ablation is recommended as first-line
therapy for therapy for symptomatic patients. Multiple large studies have demonstrated the safety
and efficacy of this approach. The success rate for catheter-ablation is approximately 90-95% but
varies based on the location of the pathway and the presence of multiple pathways.

Noninvasive (exercise stress test, Holter monitor, procainamide infusion) or invasive

(electrophysiology study) risk stratification techniques can be useful in asymptomatic patients with
WPW pattern on ECG. However, the patient described in this case is symptomatic and has WPW
syndrome. Therefore, further risk stratification is not indicated in this case.

If he were not interested in proceeding with ablation, medical therapies could be considered. In the
absence of structural heart disease, one could consider flecanide or propafenone (Class IIa).
Amiodarone, beta-blockers, diltiazem, verapamil, sotalol, and dofetilide are also options, although
these medications receive a class IIb recommendation in the 2015 American College of
Cardiology/American Heart Association/Heart Rhythm Society (ACC/AHA/HRS) Guideline for the
Management of Adult Patients With Supraventricular Tachycardia (Figure 2).

(Figure 2)
AVRT = atrioventricular re-entry tachycardia; ECG = electrocardiogram; pt = patient; SHD =
structural heart disease.
93. The correct answer: D
Key Point
Catheter ablation is the preferred treatment option for recurrent symptomatic PSVT – success rates
exceed 95% in good centers. Drug therapy (prophylaxis or as the occasion arises with drugs that
block AV node or antiarrhythmic drugs) is rarely effective or helpful.

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Question 94
94. A 64-year-old female presented to the cardiology clinic for evaluation of exertional dyspnea and
fatigue. She denied any syncope but has had a few episodes of sudden lightheadedness. Her
medical history includes hypertension and diabetes mellitus, both of which are well controlled. Her
medications include aspirin, amlodipine, hydrochlorothiazide, metformin, and atorvastatin. On
examination, her heart rate (HR) was 66 bpm, blood pressure was 127/82 mm Hg, and body mass
index (BMI) was 34 kg/m2. She was referred for cardiopulmonary exercise stress testing. She was
able to complete 5 minutes of a Bruce protocol with a baseline HR of 64 bpm and maximum HR of
98 bpm. She reached her anaerobic threshold. The test was terminated due to patient fatigue. She
denied any anginal symptoms and there were no ischemic electrocardiogram changes.

Which of the following is most likely responsible for her symptoms?

A. Deconditioning.
B. Sinus node dysfunction.
C. Baroreceptor response.
D. Obesity.
E. Medication side effect.

This patient's cardiopulmonary exercise testing (CPET) was notable for a low maximum achieved
HR (63% maximum predicted HR) in the setting of meeting anaerobic threshold, consistent with
chronotropic incompetence, which is one manifestation of sinus node dysfunction. The most
commonly accepted definition of chronotropic incompetence includes a failure to reach 80%
maximum predicted HR with exercise. Sinus node dysfunction may also present with sinus pauses,
which may be responsible for the reported history of presyncope. Obesity, deconditioning, and
baroreceptor response would not be expected to cause inadequate HR response to exercise.
Whereas beta-blockers and nondihydropyridine calcium channel blockers may blunt HR response,
amlodipine should not have this effect.

94. The correct answer: B

Key Point
SND comprises a number of different manifestations including symptomatic bradycardia, sinus
pauses due to sinus arrest or sinoatrial exit block, and chronotropic incompetence.

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Valvular Heart Disease

Question 1
A 62-year-old male with history of bileaflet mechanical aortic valve replacement (AVR)
and ascending aorta replacement 4 years ago is seen in the clinic for preoperative
evaluation prior to planned open nephrectomy. He has a history of well-controlled
hypertension. He does not exercise regularly, but reports being able to climb two flights
of stairs without difficulty. His daily medications are aspirin 81 mg, amlodipine 5 mg, and
warfarin 6 mg. His examination reveals a blood pressure of 128/73 mm Hg and a heart
rate of 74 bpm. His neck veins are not distended. His heart sounds are regular with a
crisp mechanical S2 and soft systolic murmur at the right upper sternal border. His
lungs are clear, and the remainder of his examination is normal. His surgeon requests
that warfarin be discontinued 4 days prior to surgery.
In addition to stopping warfarin, which of the following would you recommend for the
perioperative period in this patient?
A. An exercise nuclear stress test.
B. An infusion of unfractionated heparin.
C. Low-molecular weight heparin.
D. A tranthoracic echocardiogram.
E. Continue aspirin 81 mg.

Per the 2017 updated to valvular heart disease guideline:

Temporary interruption of vitamin K antagonist (VKA) anticoagulation, without bridging
agents while the interantional normalized ratio (INR) is subtherapeutic, is recommended
in patients with a bileaflet mechanical AVR and no other risk factors for thrombosis who
are undergoing invasive or surgical procedures.
“Bridging” therapy with either intravenous unfractionated heparin or low-molecular-
weight heparin has evolved empirically to reduce thromboembolic events during
temporary interruption of oral anticoagulation in higher-risk patients, such as those with
a mechanical mitral valve replacement or AVR and additional risk factors for
thromboembolism (e.g., atrial fibrillation, previous thromboembolism, hypercoagulable
condition, older-generation mechanical valves [ball-cage or tilting disc], left ventricular
systolic dysfunction, or >1 mechanical valve).

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When interruption of oral VKA therapy is deemed necessary, the agent is usually
stopped 3-4 days before the procedure (so the INR falls to 4 METs by history and so a
preoperative stress test is not needed. Absent a suspected change in valve function,
there is no need to repeat the echocardiogram at this time.
Answer E
Key Point
Periodic clinical and echocardiographic evaluation of patients with a prosthetic valve is
essential for early detection of prosthetic valve dysfunction. Careful medical
management of patients with prosthetic valves includes careful control of antithrombotic
therapy and prescription of infective endocarditis prophylaxis.

Question 2
-A 22-year-old female is referred to you for advice regarding anticoagulation
management if she gets pregnant. She had mitral valve (MV) endocarditis 3 years ago
treated with a mechanical MV replacement. She has been on warfarin since then. She
has clinically done well and is asymptomatic. She wants to have children. Her
examination reveals the mechanical MV sounds and is otherwise normal.
Her medications include birth control pills, aspirin 81 mg, and warfarin 4 mg each
evening. She has been reliably following her international normalized ratios and the
values range from 2.5-3.0 monthly.
Based on current guidelines, which of the following medical options would you
recommend to her in regard to the use of anticoagulation during pregnancy?
A. Weight-based low molecular weight heparin for the first trimester, warfarin for the
second and third trimester, and then unfractionated heparin just prior to delivery.
B. Warfarin for the entire pregnancy until the time of delivery and then a change to
unfractionated heparin.
C. Counsel against pregnancy due to the risks involved.
D. Weight-based unfractionated heparin during the entire pregnancy.
E. Weight-based low molecular weight heparin for the entire pregnancy and then a
change to unfractionated heparin just prior to delivery.
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The correct answer is that she may continue to use her low-dose warfarin for the entire
pregnancy and change to unfractionated heparin (UHF; with an activated partial
thromboplastin time of more than twofold control) just prior to vaginal delivery. This is
because the embryopathy from warfarin appears to be dose dependent. It is now a
Class IIa (Level of Evidence B) recommendation that those patients on ≤5 mg/day of
warfarin may safely remain on warfarin the entire pregnancy, changing to UFH just prior
to delivery.
The other options may be considered if the daily dose of warfarin is >5 mg/day. In that
situation, the warfarin should be discontinued for the first trimester and either UFH (with
measured activated partial thromboplastin time of more than twofold control) or low
molecular wieght heparin (LMWH; with measured anti-Xa of 0.8-1.2 U/ml 4-6 hours
postdose) may be used. Weight-based dosing of UFH and LMWH does not provide
adequate anticoagulant effect in many cases because of the increased volume of
distribution that occurs during pregnancy. Warfarin then can be reinstituted for the
second and third trimester and stopped just prior to delivery and replaced with UFH as
mentioned earlier.
Prosthetic heart valves are not a contraindication to pregnancy.
Answer B
Key Point
Selection of valve procedure (repair vs. replacement, surgical vs. transcatheter) and
prosthetic valve type (mechanical vs. bioprosthetic) has to be individualized according
to the patient’s characteristics, including age, surgical risk, and personal preferences.

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Question 3
3-A 30-year-old male is referred for evaluation of a murmur. He reports no symptoms
and has no significant medical history. On physical examination his height is 69 inches
and weight is 185 lbs. His blood pressure is 135/70 mm Hg with a regular heart rate of
78 bpm. His lungs are clear. Prominent carotid pulsations are present. The jugular
venous pulse is at the level of the sternal notch. The apical impulse is slightly enlarged
and laterally displaced to the anterior axillary line. The S1 and S2 are normal, and an S3
is present. There is an early systolic click that does not change with inspiration. Both a
soft (grade 2/6) crescendo-decrescendo systolic murmur and a soft (grade 2/4)
decrescendo diastolic murmur are present along the left sternal border.

Which of the following is the most likely valvular abnormality in this patient?
A. Bicuspid aortic valve with regurgitation.
B. Patent ductus arteriosus.
C. Pulmonic valve stenosis with regurgitation.
D. Rheumatic mitral stenosis and regurgitation.
E. Degenerative aortic valve stenosis with regurgitation.

Bicuspid aortic valve can be associated with sudden cessation of valve opening, leading
to an ejection systolic click, and often is associated with significant aortic valve
regurgitation. Findings of chronic aortic regurgitation with left ventricular volume
overload include an enlarged and laterally displaced apical pulse, wide aortic pulse
pressure, and an S3.
Pulmonic valve stenosis is unlikely because the murmur does not change with
respiration and the jugular venous pulse is normal. Additionally the intensity of the
pulmonic ejection click decreases with inspiration.
Degenerative aortic stenosis is not associated with a systolic click and is less likely
given his young age.
Rheumatic mitral stenosis may be associated with a diastolic click, but not one that
occurs during systole.
Patent ductus arteriosus produces a continuous machine-like murmur and is not
associated with a click or wide pulse pressure. Answer A

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Question 4
4-A 54-year-old male presents for routine evaluation for known chronic aortic valve
regurgitation due to bicuspid aortic valve. He remains asymptomatic. His vital signs
include a heart rate of 84 bpm and blood pressure of 140/50 mm Hg. His physical
examination reveals a grade 2/6 systolic ejection murmur along the left sternal border
and a grade 3/4 diastolic flow murmur. His echocardiogram reveals severe aortic
regurgitation.

Which of the following echocardiographic parameters would be an indication for surgical

aortic valve replacement at this time?
A. A left ventricular end-diastolic dimension of 6.0 cm.
B. A vena contracta width of 0.7 cm.
C. A left ventricular end-systolic dimension of 5.3 cm.
D. A left ventricular ejection fraction of 55%.
E. An aortic regurgitant fraction of 65%.

Based on the 2014 American College of Cardiology/American Heart Association

valvular heart disease guideline, the patient has evidence for severe aortic valve
regurgitation. Aortic valve replacement (AVR) is indicated for the asymptomatic patient
with severe aortic regurgitation when there is evidence of left ventricular (LV) systolic
dysfunction (LV ejection fraction <50%) or other cardiac surgery is planned (Class I
indications; see Figure 1). AVR is also indicated if there is LVEF ≥50% but the left
ventricular end-systolic dimension (LVESD) is >50 mm (Class IIa) or left ventricular end-
diastolic dimension (LVEDD) is >65 mm and the patient is of low surgical risk (Class
IIb). The other echocardiographic parameters (vena contracta width, regurgitant
fraction) confirm severity of aortic regurgitation, but are not critical factors in the decision
for surgery. Answer C

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Key Point
Left ventricular dysfunction and dilatation can be reversible if valve replacement is
performed in a timely fashion. Therefore, serial follow-up with clinical examinations and
echocardiography is recommended to identify patients who require intervention before
symptoms develop.

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Question 5
5-A 33-year-old male is admitted with a 1-week history of fever and shortness of breath.
He has a history of intravenous drug use.
On examination, he has a temperature of 102.5 degrees Fahrenheit, a heart rate of 110
bpm, and a blood pressure of 102/60 mm Hg. He appears mildly dyspneic and
diaphoretic. His jugular venous pressure is 10 cm H20. His lungs have bibasilar rales.
His cardiac exam demonstrates a hyperdynamic precordium with a soft systolic murmur
and S3 gallop. His extremities are warm without edema.
His echocardiogram on admission shows a left ventricular ejection fraction of 75%, a 1
cm vegetation on the tricuspid valve with moderate to severe tricuspid regurgitation, and
thickening of the mitral valve with severe mitral regurgitation. His chest X-ray shows
pulmonary edema.
He is admitted to telemetry. Serial blood cultures are sent and intravenous vancomycin
is started.
What is the next best step in the management of this patient?

A. Intra-aortic balloon counterpulsation.

B. Urgent valve replacement.
C. Repeat transesophageal echocardiogram in 6 weeks.
D. Initiation of sacubitril/valsartan.
E. Addition of rifampin.

Early surgery (during initial hospitalization before completion of a full therapeutic course
of antibiotics) is indicated in patients with infective endocarditis (IE) who present with
valve dysfunction resulting in symptoms of heart failure (HF). Reinfection after
prosthetic valve surgery (which occurs in 5-10% of patients, with a significant
percentage of these being injectable drug users) is low relative to the risk of no surgery
in patients with hemodynamic and microbial indications for surgery. Repair rather than
replacement of a valve is always best; however, such repairs are possible in only a
minority of cases, such as when a leaflet perforation occurs without extensive leaflet
destruction or annular involvement. Prompt surgical consultation should be obtained in
all cases of IE to assist with assessment of the need for surgical treatment and to help
judge the timing of surgery.

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There is no role for sacubitril/valsartan in HF related to acute valvular disease.

Rifampin may be used in cases of IE for long-term suppressive therapy, but antibiotics
are not a substitute for valve surgery in a patient with HF from IE.
The patient is not in cardiogenic shock and thus intra-aortic balloon pump support is not
indicated; furthermore, even if indicated, it would be bridge to definite valve surgery.
Repeating a transesophagael echocardiogram in 6 weeks is not the favored approach
due to the high mortality of left-sided endocarditis with hemodynamically significant
valvular dysfunction.
Answer B
Key Point
Surgical management of infective endocarditis has the following objectives: completely
excise all infected and necrotic tissue, remove and/or replace all infected prosthetic
material, and reconstruct cardiac structures to restore proper physiology.

Question 6
6-A 29-year-old female is referred by her internist for evaluation of a newly identified
murmur. The patient reports no cardiovascular symptoms and has no significant
medical history except for a known penicillin allergy. Her only prescribed medicine is an
oral contraceptive. She denies recreational drug use.
On physical examination, there is a systolic click followed by a grade 2/6 late systolic
murmur.
Her transthoracic echocardiogram demonstrates myxomatous mitral valve disease
(MVD) with moderate regurgitation and mild left atrial enlargement. The left ventricular
end-systolic dimension is 32 mm and ejection fraction is 65%.

Which of the following is required prior to dental procedures in this patient?

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A. No antibiotic prophylaxis.
B. Clindamycin 600 mg.
C. Cephalexin 2 g.
D. Amoxicillin 2 g.
E. Metronidazole 500 mg.

The most current guidelines do not recommend antibiotic prophylaxis for endocarditis
for myxomatous MVD. Endocarditis prophylaxis is only recommended for conditions
associated with the highest risk of adverse outcome from endocarditis. These conditions
include prosthetic cardiac valve or prosthetic valve repair material, prior history of
infective endocarditis, cardiac transplant recipients with valvulopathy, completely
repaired congenital heart disease (CHD) with percutaneous or surgical repair occurring
within the previous 6 months, repaired CHD with residual shunts or defects that impair
endothelialization of prosthetic material, and unrepaired cyanotic CHD.
For patients with these conditions, antibiotic prophylaxis is recommended for dental
procedures that involve manipulation of the gingival tissues, the periapical region of the
teeth, or perforation of oral mucosa. Antibiotic prophylaxis also may be considered for
procedures that involve incision of the respiratory mucosa (e.g., tonsillectomy or
adenoidectomy) and for genitourinary, skin, or gastrointestinal procedures that involve
infected areas. Patients undergoing diagnostic procedures in the absence of these
conditions do not require antibiotic prophylaxis.
Options for antibiotic prophylaxis for endocarditis prior to dental procedures are shown
in Tables 1 and 2. Answer A

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Question 7
7-A 44-year-old male comes to your clinic to establish general cardiology care after a
mitral valve replacement with a mechanical prosthesis. His current medications are
warfarin and metoprolol. His blood pressure is 120/80 mm Hg and heart rate is 60 bpm.
His physical exam reveals a soft systolic murmur and crisp metallic valve sounds.

He is inquiring if the direct oral anticoagulation agents advertised on the internet and
television are a reasonable choice for him as he does not wish to be on warfarin.

Which of the following do you recommend?

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A. Continue warfarin and add aspirin.

B. Switch warfarin to dabigatran.
C. Continue warfarin and add dipyridamole.
D. Continue warfarin and add clopidogrel.

Effective oral antithrombotic therapy in patients with mechanical heart valves requires
continuous vitamin K antagonist anticoagulation (VKA) with an international normalized
ratio (INR) in the target range. It is preferable to specify a single INR target for each
patient and to recognize that the acceptable range includes 0.5 INR units on each side
of this target. A specific target is preferable because it reduces the likelihood of patients
having INR values consistently near the upper or lower boundary of the range. In
addition, fluctuations in INR are associated with an increased incidence of complications
in patients with prosthetic heart valves, so patients and caregivers should strive to attain
the specific INR value.

The effects of VKA anticoagulation vary with the specific drug, absorption, various
foods, alcohol, other medications, and changes in liver function. Most of the published
studies of VKA therapy used warfarin, although other coumarin agents are used on a
worldwide basis. In clinical practice, a program of patient education and close
surveillance by an experienced healthcare professional, with periodic INR
determinations, is necessary. Patient monitoring through dedicated anticoagulation
clinics results in lower complication rates than those seen with standard care and is cost
effective because of lower rates of bleeding and hemorrhagic complications. Periodic
direct patient contact and telephone encounters with the anticoagulation clinic
pharmacists or nurses are equally effective in reducing complication rates. Self-
monitoring with home INR measurement devices is another option for educated and
motivated patients.

Aspirin is recommended for all patients with prosthetic heart valves, including those with
mechanical prosthetic valves receiving VKA therapy. Even with the use of VKA, the risk
of thromboemboli is 1-2% per year.

The addition of aspirin 100 mg daily to oral VKA-anticoagulation decreases the

incidence of major embolism or death (1.9% vs. 8.5% per year; p < 0.001), with the
stroke rate decreasing to 1.3% per year versus 4.2% per year (p < 0.027), and overall
mortality to 2.8% per year versus 7.4% per year (p < 0.01). The addition of low-dose
aspirin (75 mg to 100 mg per day) to VKA therapy (INR 2.0 to 3.5) also decreases
mortality due to other cardiovascular diseases. The combination of low-dose aspirin and
a VKA is associated with a slightly increased risk of minor bleeding such as epistaxis,
bruising, and hematuria, but the risk of major bleeding does not differ significantly
between those who received aspirin (8.5%) versus those who did not (6.6%; p = 0.43).
The risk of gastrointestinal irritation and hemorrhage with aspirin is dose dependent
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over the range of 100 mg to 1,000 mg per day, but the antiplatelet effects are
independent of dose over this range. The addition of aspirin (75 mg to 100 mg per day)
to a VKA should be strongly considered unless there is a contraindication to the use of
aspirin (i.e., bleeding or aspirin intolerance). This combination is particularly appropriate
in patients who have had an embolus while on VKA therapy with a therapeutic INR,
those with known vascular disease, and those who are known to be particularly
hypercoagulable.

The one randomized controlled study of dabigatran compared with warfarin in patients
with mechanical valves was stopped early due to higher rates of bleeding and
thrombosis in the dabigatran arm. No other direct oral anticoagulants have been studied
in this patient population. There is no role for clopidogrel or dipyridamole in the
management of mechanical heart valves. Question

Question 8
8-You are meeting a 54-year-old male who has recently relocated and seeks to
establish cardiovascular care. He underwent aortic valve replacement with a low-profile
bileaflet tilting disk valve and simultaneous aortic root replacement at the age of 51 due
to bicuspid aortic valve disease with an ascending aortic aneurysm. His primary concern
for this visit is to seek an alternative to warfarin, because he was admitted with
gastrointestinal bleeding from gastritis 2 months ago. He has no other medical
problems. His current medications are aspirin 81 mg and warfarin dosed to maintain an
international normalized ratio (INR) of 2.0-3.0. His cardiac exam reveals crisp S1 and
S2 sounds.

Which of the following do you recommend for this patient?

A. Continue warfarin and aspirin.
B. Adjust warfarin for an INR of 1.5-2.5.
C. Stop aspirin.
D. Replace aspirin with clopidogrel.
E. Replace warfarin with dabigatran.

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Effective oral antithrombotic therapy in patients with mechanical heart valves requires
continuous vitamin K antagonist (VKA) anticoagulation with an INR in the target range.
It is preferable to specify a single INR target for each patient and to recognize that the
acceptable range includes 0.5 INR units on each side of the target. A specific target is
preferable because it reduces the likelihood of patients having INR values consistently
near the upper or lower boundary of the range. The effects of VKA anticoagulation vary
with the specific drug, absorption, various foods, alcohol, other medications, and
changes in liver function. Most of the published studies of VKA therapy used warfarin,
although other coumarin agents are used on a worldwide basis.

In clinical practice, a program of patient education and close surveillance by an

experienced healthcare professional, with periodic INR determinations, is necessary.
Patient monitoring through dedicated anticoagulation clinics results in lower
complication rates than those seen with standard care and is cost effective because of
lower rates of bleeding and hemorrhagic complications. Periodic direct patient contact
and telephone encounters with the anticoagulation clinic pharmacists or nurses are
equally effective in reducing complication rates. Self-monitoring with home INR
measurement devices is another option for educated and motivated patients.

A randomized controlled trial of dabigatran compared with warfarin for patients with
mechanical prosthetic valves was stopped early due to higher rates of both bleeding
and thrombosis in patients receiving dabigatran. No other direct oral anticoagulants
have been studied in this patient population.

Aspirin and not P2Y12 inhibitors, such as clopidogrel, have been used alongside
warfarin in clinical trials for Food and Drug Administration approval of currently available
mechanical valves and this combination remains recommended for all patients with
mechanical valves in either the aortic or mitral position.

In a patient with a history of gastrointestinal bleeding that has been appropriately

treated, it would not be appropriate to withhold antiplatelet or anticoagulant agents.
Answer A
Key Point
Aspirin alone or direct anticoagulants are not an alternative to warfarin for patients with
mechanical prosthetic valves.

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Question 9
9-A 48-year-old male presents for evaluation of bicuspid aortic stenosis because of
worsening shortness of breath over the past few months. He is an avid motorcyclist and
despite a recent accident, would never consider not riding. His echocardiogram 6
months ago showed normal biventricular function, a peak transaortic velocity of 4.2
m/sec, peak transaortic gradient of 72 mm Hg, mean gradient of 48 mm Hg, and
calculated aortic valve area of 0.8 cm2. His aortic root is a normal size. His past medical
history is significant for hypothyroidism. On exam today his blood pressure is 128/66
mm Hg, heart rate is 72 bpm, and he has a normal S1 and a late peaking harsh systolic
murmur. His lungs are clear to auscultation bilaterally and he has no peripheral edema.

Which of the following is the next most appropriate step in the care of this patient?
A. Transesophageal echocardiogram.
B. Repeat transthoracic echocardiogram in 1 year.
C. Bioprosthetic aortic valve replacement.
D. Exercise stress testing.
E. Mechanical aortic valve replacement.

This patient has severe aortic stenosis, has now developed symptoms and should
undergo aortic valve replacement (AVR). The choice of which type of valve,
bioprosthetic versus mechanical, is an individual one and should be based on the
individual's preferences, risks of long-term anticoagulation, and contraindications to
anticoagulation. A bioprosthesis is recommended in patients of any age for whom
anticoagulant therapy is contraindicated, cannot be managed appropriately, or is not
desired (Class 1, Level of Evidence C). Per the 2017 update to the valvular heart
disease guideline, a bioprosthetic valve is reasonable for patients 50-70 years of age
based on individual patient factors and preferences. While the risk for reoperation with
bioprosthetic valves is greater in younger patients, this must be balanced against the
risk of bleeding such as in this patient who is an avid motorcyclist. Transesophageal
echocardiography would not alter the management of this patient. Since this patient is
now symptomatic, AVR is indicated. Exercise stress testing is reasonable to assess
physiological changes with exercise and to confirm the absence of symptoms in
reportedly asymptomatic patients with a calcified aortic valve and an aortic velocity ≥4.0
m/sec or a mean pressure gradient of ≥40 mm Hg (stage C). This patient reports
symptoms, and exercise stress testing is contraindicated in this setting (Class III).
Answer C

Key Point
Selection of valve procedure (repair vs. replacement, surgical vs. transcatheter) and
prosthetic valve type (mechanical vs. bioprosthetic) has to be individualized according
to the patient’s characteristics, including age, surgical risk, and personal preferences
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Question 10

10- A 71-year-old male with a history of a bioprosthetic aortic valve replacement 16

years ago for endocarditis returns for routine follow-up. He denies any symptoms of
exertional dyspnea, chest pressure, or dizziness.

His examination reveals a grade 3/6 mid-peaking systolic ejection murmur and a mild
diastolic murmur. An S4 is present.

His echocardiogram shows a left ventricular (LV) ejection fraction of 55%, an LV end-
diastolic diameter of 4.9 cm, an LV end-systolic diameter of 2.3 cm, and LV wall
thickness of 1.1 cm. Peak aortic velocity is 4.3 m/sec and mean aortic gradient is 44
mm Hg. The aortic valve area is 0.9 cm2 with mild aortic regurgitation and there is no
tricuspid regurgitation jet to estimate pulmonary pressures.

Based on these data, which of the following is the next best step in the management of
this patient?
A. Dobutamine stress echocardiogram.
B. Exercise testing.
C. Transesophageal echocardiogram.
D. Refer for aortic valve replacement.

The indications for intervention in prosthetic valve stenosis are the same as those for
native stenosis of the aortic or mitral valve. This patient has severe prosthetic aortic
stenosis (AS) without symptoms. Thus, exercise stress testing is as Class IIa indication
in this setting. The inability to augment systolic blood pressure by 20 mm Hg or the
development of symptoms are adverse prognostic signs that would merit intervention.

Patients with prosthetic valve AS merit referral to a heart valve team for consideration of
percutaneous or surgical intervention.

Neither transesophageal echocardiogram nor dobutamine stress echocardiogram are

indicated when the transthoracic echocardiogram already demonstrates severe AS.

Aortic valve replacement is not indicated in the absence of symptoms or high-risk

findings on stress testing. Answer B
Key Point
Doppler echocardiography (transthoracic echocardiography and transesophageal
echocardiography) is the method of choice for the diagnosis of prosthetic valve
dysfunction (stenosis and regurgitation).
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Question 11
11-You are seeing a 40-year-old female with flushing and diarrhea as well as
progressive ankle swelling. She has gained 20 pounds in the last 6 months and says
her clothes are fitting tight around the waist. She has no other medical problems and
takes no medications. Her physical exam reveals a blood pressure of 106/70 mm Hg, a
pulse of 88 bpm, and respirations of 14 per minute. Her jugular veins are distended with
prominent "v" waves. There is a soft, early systolic murmur along her right lower sternal
border. Her liver is pulsatile and palpable two finger-breadths below the costal margin.
There is 3+ bilateral lower extremity edema up to the thighs. Her laboratory evaluations
include a normal thyroid stimulating hormone, normal electrolytes and renal function,
elevated total bilirubin, and elevated 5-HIAA.

Which of the following echocardiographic findings would confirm your clinical diagnosis?
A. Hepatic vein flow reversal.
B. A peak tricuspid regurgitant velocity of 4 m/s.
C. A systolic notch on the pulmonary valve.
D. Pulmonary vein flow reversal.
E. Inferior vena cava collapse with inspiration.

Causes of primary tricuspid regurgitation (TR) include radiation, Ebstein's anomaly,

infective endocarditis, cardiovascular implanted electronic devices, and carcinoid
syndrome, which is what this patient's clinical presentation suggests. Advanced degrees
of TR may be detected on physical examination by the appearance of elevated “c-V”
waves in the jugular venous pulse (JVP), a systolic murmur at the lower sternal border
that increases in intensity with inspiration, and a pulsatile liver edge. In many patients,
characteristic findings in the JVP are the only clues to the presence of advanced TR,
because a murmur may be inaudible even with severe TR. Symptoms include fatigue
from low cardiac output, abdominal fullness, edema, and palpitations, particularly if atrial
fibrillation is also present. Progressive hepatic dysfunction may occur due to the
elevated right atrial pressure, and thus assessment of liver function is useful in patients
with advanced degrees of TR.

Transothoracic echocardiography can distinguish primary from functional TR, define any
associated left-sided valvular and/or myocardial disease, and provide an estimate of
pulmonary artery systolic pressure. Characterization of severity of TR relies on an
integrative assessment of multiple parameters.

Hepatic vein flow reversal is seen in severe TR, which is the correct answer.

Systolic notching of the pulmonic valve is seen in severe pulmonary hypertension and
not expected in cases of primary TR related to carcinoid syndrome.

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The inferior vena cava would be plethoric, not collapsed, in severe TR.

Pulmonary vein flow reversal is a sign of severe MR and would not be expected from a
right-sided valvular lesion.

The peak tricuspid regurgitant velocity reflects the right ventricular-right atrial pressure
gradient and does not reflect the severity of TR. Answer A
Key Point
In addition to Doppler color jet area, quantitative measures should be integrated to
determine tricuspid regurgitation (TR) severity, including Doppler characteristics of the
TR jet, jet density, contour by continuous wave, width of the vena contracta, systolic
hepatic vein flow, proximal isovelocity surface area (PISA)–derived regurgitant fraction
and effective regurgitant orifice area (EROA), as well as right atrium and ventricle size
and function. Systolic hepatic vein flow reversal is the strongest correlate for severe TR.

Question 12
12-A 55-year-old female presents with a 6-month history of progressive dyspnea on
exertion. She has a history of hypertension and hyperlipidemia. Her medications include
amlodipine 5 mg daily and atorvastatin 20 mg daily. On examination, her heart rate is 76
bpm and irregular; her blood pressure is 126/70 mm Hg. Her jugular venous pressure is
8 cm H20 with prominent v waves. Her lungs are clear. Her cardiac exam reveals a brief,
high-pitched sound after S2 followed by a low-pitched rumble, best heard at the apex at
held expiration. Her extremities have no edema.

A transthoracic echocardiogram (TTE) reveals an ejection fractrion of 60% with

thickened mitral leaflets, reduced motion during diastole, and doming. The mean mitral
valve area is 1.2 cm2. There is moderate tricuspid regurgitation with an estimated right
ventricular systolic pressure of 55 mm Hg.

Which of the following studies is necessary to determine the correct treatment approach
for this patient?
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A. Cardiopulmonary exercise stress test.

B. Exercise stress echocardiogram.
C. Transesophageal echocardiogram.
D. Cardiac magnetic resonance imaging.
E. Right heart catheterization.

This patient has severe symptomatic mitral stenosis (MS) with atrial fibrillation and
pulmonary hypertension. Percutaneous balloon mitral commissurotomy is the treatment
of choice for severe symptomatic MS over surgical commissurotomy as long as the
valve is mobile, relatively thin, and free of calcium and there is no left atrial clot or more
than mild mitral regurgitation (MR).

The 2014 American College of Cardiology/American Heart Association guideline on the

management of valvular heart disease note that a transesophageal echocardiogram
(TEE) should be performed in patients considered for percutaneous mitral balloon
commissurotomy (PMBC) to assess the presence or absence of left atrial thrombus and
to further evaluate the severity of MR. In the vast majority of patients with MS, valve
morphology and lesion severity can be obtained with TTE. A key exception is in patients
being considered for PMBC, in whom left atrial cavity and appendage thrombi must be
excluded. Although a TTE may identify risk factors for thrombus formation, TTE has
poor sensitivity for detecting such thrombi, thus mandating a TEE before PMBC.
Although TTE is generally accurate in grading MR, TEE may offer additional
quantitation and assurance that MR >2+ is not present, which generally precludes
PMBC.

In the contemporary era, adequate assessment of MS and associated lesions can be

obtained in the vast majority of patients by TTE, occasionally supplemented by TEE,
and thus cardiac catheterization is rarely indicated. Even if it were to be performed, right
heart catheterization alone would not be the correct approach, as simultaneous
pressure measurements in the left ventricle (LV) and left atrium, via transseptal
catheterization, are necessary because the LV to pulmonary wedge gradient will
overestimate the true transmitral gradient due to phase delay and delayed transmission
of pressure changes.

Exercise testing with Doppler or invasive hemodynamic assessment is recommended to

evaluate the response of the mean mitral gradient and pulmonary artery pressure in
patients with MS when there is a discrepancy between resting Doppler
echocardiographic findings and clinical symptoms or signs. However, there is no
discrepancy in this case and thus exercise testing is not indicated.

A cardiopulmonary exercise stress test measures maximum oxygen consumption and is

useful in differentiating cardiac from pulmonary causes of dyspnea and offers prognostic
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information for end-stage heart failure patients contemplating heart transplantation.

However, this test is not useful in the evaluation or management of patients with MS.

While cardiac magnetic resonance imaging is useful in many cardiac conditions, it plays
little role in the evaluation of patients with MS. Answer C
Key Point
Percutaneous mitral balloon commissurotomy is indicated in symptomatic patients or
asymptomatic patients with pulmonary hypertension, moderate or severe stenosis, and
favorable valve morphology in the absence of left atrial thrombus or moderate to severe
mitral regurgitation.

Question 13
13- A 40-year-old male with a history of bicuspid aortic valve (BAV) presents to your
office with complaints of exertional dyspnea. He recently joined a gym to help him lose
weight. He has started jogging on a treadmill, however he notes shortness of breath
when running at an incline. He denies chest pain and is able to run on level ground
without difficulty. His physical exam includes a height of 70 inches, weight of 225 lbs,
blood pressure of 112/73 mm Hg, and a heart rate of 82 bpm. His carotid pulses are
prominent and lungs are clear. His heart exam is notable for a holodiastolic murmur.
There is no lower extremity edema. A transthoracic echocardiogram is performed. His
left ventricular ejection fraction is 60%. There is a BAV with aortic regurgitation
(pressure half-time [PHT] 280 msec, vena contracta width 0.5 cm, and effective
regurgitant oriface [ERO] 0.26 cm2). His ascending aorta measures 4.8 cm. These
findings are similar to his echocardiogram performed last year.

What is the next best step in the management of this patient?

A. Genetic testing.
B. Echocardiogram in 6 months.
C. Cardiac surgery referral.
D. Hydralazine 10 mg three times a day.
E. Computed tomography scan in 12 months.

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This patient has moderate aortic regurgitation (PHT 200-500 msec, vena contracta
width 0.3-0.6 cm, and ERO 0.1-0.29 cm2). His exertional dyspnea is mild, only occurring
when running at an incline and is likely due to deconditioning given that he has only
recently started exercising and is overweight. The most notable finding in his
presentation is the dilated ascending aorta at 4.8 cm. BAVs are frequently associated
with aortic dilation either at the level of the sinuses of Valsalva or, more frequently, in
the ascending aorta. The incidence of aortic dilation is higher in patients with fusion of
the right and noncoronary cusps. Aortic imaging is recommended annually in patients
with a BAV and significant aortic dilation (>4.5 cm), a rapid rate of change in aortic
diameter (increase of >0.5 cm in a year, and in those with a family history of aortic
dissection (Class I recommendation, Level of Evidence C). Aortic imaging can be by
echocardiogram if there is adequate image quality with visualization of the aorta up to 4
cm distal to the valve. Alternatively computed tomography or magnetic resonance
imaging can provide better spatial resolution and is preferred in patients with poor
echocardiographic windows.

This patient has no indication for cardiac surgery at this point. Surgical intervention is
recommended at a dimension of 5.5 cm or at 5.1-5.5 cm in patients with rapid growth or
a family history of aortic dissection. An echocardiogram at 6 months is too soon. Given
his normal blood pressure, there is no benefit of adding hydralazine. At present, there
are no proven drug therapies that have been shown to reduce the rate of progression of
aortic dilation in patients with aortopathy associated with BAV. In patients with
hypertension, control of blood pressure is warranted. A specific genetic cause has not
been identified yet in patients with BAV and aortopathy and therefore genetic testing is
not recommended. Answer E
Key Point
Patients with aortic regurgitation on the basis of a bicuspid aortic valve should be
evaluated and followed for abnormalities of the aorta.

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Question 14
14-You are seeing a 58-year-old female in clinic for follow-up of an abnormal computed
tomography (CT) scan of her chest. She had pleuritic chest pain and was diagnosed
with a pulmonary embolus 2 weeks ago. Since starting on anticoagulant therapy she
feels well. She is a life-long nonsmoker. She has two adult children. Her vital signs are a
pulse of 78 bpm, respirations 12 per minute, and blood pressure of 168/92 mm Hg in
the right arm and 162/91 mm Hg in the left arm. Her height is 64 inches (162 cm) and
weight is 178 pounds (80.7 kg). Her carotid pulses are brisk. The apical impulse is not
displaced; S1 and S2 are normally split without any clicks. The remainder of her exam is
unremarkable. A CT angiogram of her chest demonstrated that her aorta was dilated,
measuring 4.2 cm at the mid-ascending aorta, without a visible dissection flap.

Which of the following is the most appropriate next test for this patient?
A. Transthoracic echocardiogram.
B. Repeat CT in one year.
C. Carotid ultrasound.
D. Magnetic resonance angiography.
E. Coronary angiography.

Patients with dilation of the aortic root or ascending thoracic aorta should be evaluated
for bicuspid aortic valve and/or aortic regurgitation with transthoracic echocardiography.

There are no data to support screening for carotid disease in asymptomatic patients.

Magnetic resonance angiography is a reasonable alternative to CT angiography for

surveillance of aortic diameter, but first any associated valve disease must be identified
before arranging surveillance imaging.

Repeat imaging for stability of the aortic size is appropriate, but an assessment of aortic
valve function is needed prior to planning the schedule and best imaging modality for
that surveillance.

Coronary angiography would be appropriate in patients with aneurysms large enough to

warrant elective repair, generally indicated at aortic diameters of >5.5 cm in sporadic
thoracic aneurysms such as this, but not in this patient without risk factors for rupture.
Answer A
Key Point
Aortic regurgitation may be due to abnormalities of the aortic valve and/or aorta.

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Question 15
15-A 29-year-old female presented for prenatal counseling. She exercises by running
three miles 5 days per week without limitations. She has a history of a heart murmur
since childhood and a previous echocardiogram suggesting ‘enlargement of the heart'.

Her physical exam reveals normal jugular venous pressure and contour. Precordial
palpation is unremarkable. Auscultation along the left sternal border reveals a high-
pitched sound just after S1 followed by a murmur.

Which of the following maneuvers will best identify the associated valvular abnormality
in this patient?
A. Standing.
B. Inspiration.
C. Hand grip.
D. Valsalva.
E. Squatting.

This patient has a right-sided ejection click and ejection murmur. Ejection clicks are
high-pitched sounds that occur at the moment of maximal opening of the aortic or
pulmonary valves. They are heard just after the first heart sound. The sounds occur in
the presence of a dilated aorta or pulmonary artery or in the presence of a bicuspid or
flexible stenotic aortic or pulmonary valve. In this case, the ejection click and murmur
are heard only at the left sternal border, consistent with pulmonic rather than aortic
stenosis.

The most helpful distinguishing feature of a pulmonary ejection sound is its decreased
intensity, or even its disappearance during the inspiratory phase of respiration. During
expiration, the valve opens rapidly from its fully closed position; sudden "halting" of this
rapid opening movement is associated with a maximal intensity of the ejection sound.
With inspiration, the increased venous return to the right ventricle (RV) augments the
effect of right atrial systole and causes partial opening of the pulmonary valve prior to
ventricular systole. The lack of a sharp opening movement of the pulmonary valve
explains the decreased intensity of the pulmonary ejection sound during inspiration.
Thus, the best way to confirm that the patient has pulmonic stenosis is to assess the
intensity of the murmur on inspiration.

The other maneuvers listed would not be expected to affect the auscultation of a right-
sided murmur.

Sustained hand grip for 20-30 seconds leads to an increase in systemic vascular
resistance, arterial pressure, cardiac output, and left ventricular (LV) volume and filling
pressure. Hand grip is most useful in differentiating between the ejection systolic
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murmur of aortic stenosis and the regurgitant murmur of mitral regurgitation (MR).
Intensity of the murmur of aortic stenosis tends to decrease along with a decreased
transvalvular pressure gradient, while the severity and murmur of MR increase.

During the straining phase, phase 2, of Valsalva there is a decrease in venous return,
RV and LV volumes, stroke volumes, mean arterial pressure, and pulse pressure; this is
associated with a reflex increase in heart rate. The murmur of hypertrophic
cardiomyopathy (HCM) increases in intensity as the LV outflow size decreases with a
decreased venous return. In mitral valve prolapse (MVP) there is an early onset of the
click and murmur due to the decrease in LV volume.

Abrupt standing from the supine position decreases venous return to the heart and,
consequently, RV and LV diastolic volumes and stroke volumes decline. There also
may be a fall in arterial pressure and a reflex increase in heart rate. This will cause
similar effects to phase 2 of a Valsalva maneuver.

Squatting from a standing position is associated with a simultaneous increase in venous

return (preload) and systemic vascular resistance (afterload) and a rise in arterial
pressure. In HCM, intensity of the ejection systolic murmur declines because of an
increased LV volume and arterial pressure, which increase the effective orifice size of
the outflow tract. In patients with MVP there is a delay in the onset of the click and a
shortening of the late systolic murmur. These changes reflect the delay in prolapse
induced by the increase in preload. However, as MR becomes more severe, the
murmur may increase in intensity with squatting because of the increase in afterload.
Answer B
Key Point
The only right-sided auscultatory event that diminishes with inspiration is the pulmonary
ejection click associated with pulmonary valve stenosis.

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Question 16
16-A 76-year-old male with a past history of coronary artery bypass grafting 20 years
ago is admitted with progressive dyspnea on exertion. He has diabetes mellitus, stage 3
chronic kidney disease, hypertension, dyslipidemia, and had a stroke 5 years ago that
left him with residual left-sided weakness. His echocardiogram shows a mildly dilated
left ventricle, a left ventricular ejection fraction of 25-30% with regional variations, and
severe aortic stenosis (AS) with a mean gradient of 50 mm Hg and a calculated valve
area of 0.8 cm2. Coronary angiography demonstrates patent grafts and severe native
vessel disease.

What is the most appropriate next step in the management of this patient?
A. Transcatheter aortic valve replacement.
B. Valved apical-aortic conduit.
C. Balloon aortic valvuloplasty.
D. Left ventricular assist device.
E. Dobutamine infusion.

This patient has severe symptomatic AS. The recommendation for either surgical aortic
valve replacement (AVR) or transcather AVR among high-risk patients with severe,
symptomatic AS (stage D), after consideration by a heart valve team, was changed from
Class IIa (Level of Evidence [LOE] B) to Class I (LOE A) in the 2017 guideline for
patients with valvular heart disease.

Percutaneous aortic balloon dilation has an important role in treating children,

adolescents, and young adults with AS, but its role in treating older patients is very
limited. The mechanism by which balloon dilation modestly reduces the severity of
stenosis in older patients is by fracturing calcific deposits within the valve leaflets and, to
a minor degree, stretching the annulus and separating the calcified or fused
commissures. Immediate hemodynamic results include a moderate reduction in the
transvalvular pressure gradient, but the postdilation valve area rarely exceeds 1.0 cm 2.
Despite the modest change in valve area, an early symptomatic improvement usually
occurs. However, serious acute complications, including acute severe aortic
regurgitation, restenosis, and clinical deterioration, occur within 6-12 months in most
patients. Therefore, in patients with AS, percutaneous aortic balloon dilation is not a
substitute for AVR.

Apico-aortic conduits to bypass the diseased aortic valve are a historical curiosity and
no longer performed in the era of transcatheter aortic valve therapy.

Left ventricular assist devices (LVAD) are indicated for end-stage heart failure patients
only when all other therapeutic options have been exhausted. In this case AVR would
be expected to result in improvement in symptoms, myocardial performance, and
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mortality, so AVR would be preferred to LVAD.

Palliative care with inotropic support would not be expected to improve quality of life or
mortality in a patient with severe symptomatic AS. Answer A
Key Point
The primary indication for aortic valve replacement (AVR) is symptoms due to severe
aortic stenosis (AS). AVR is also recommended in asymptomatic patients with severe
AS and a reduction in ejection fraction (<50%), and may be considered with very severe
AS, rapid AS progression, or at the time of other cardiac surgery.

Question 17
17-A 52-year-old male with a history of Marfan syndrome and remote Type A aortic
dissection status post mechanical aortic valve replacement and root repair presents to
the emergency department with 1 week of progressive dyspnea and orthopnea. On
physical exam, he is afebrile, his heart rate is 92 bpm, blood pressure is 148/92 mm Hg,
respiratory rate is 22 breaths per minute, and pulse oximetry is 90% on room air. His
jugular venous pressure is elevated. His lung exam is notable for rales and scattered
wheezing. His heart sounds are distant. There is 1+ bilateral lower extremity edema. His
electrocardiogram (ECG) shows sinus rhythm with 0.5 mm lateral ST depressions. His
laboratory results reveal a creatinine of 1.2 mg/dl, hemoglobin of 12 g/dl, international
normalized ratio (INR) of 2.2, brain natriuretic peptide of 1200 pg/ml (normal <100), and
troponin I of 0.4 ng/ml (normal <0.04). His transthoracic echocardiogram (TTE) shows a
grossly normal left ventricular ejection fraction with poor Doppler windows. His chest X-
ray shows bilateral patchy infiltrates.

Which of the following is the next best step in the management of this patient?
A. Two sets of blood cultures.
B. Right heart catheterization with thermodilution.
C. Ventilation-perfusion scan.
D. Transesophageal echocardiogram.
E. Coronary angiogram.

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The patient presents with new onset left-sided heart failure (HF) with pulmonary edema.
Given his history of mechanical aortic valve replacement, it is essential to evaluate for
valve thrombosis (even in the presence of a therapeutic INR at present as he may have
been subtherapeutic in the past month). Echocardiography holds a Class I indication for
the evaluation of suspected prosthetic valve dysfunction. TTE allows for the evaluation
of the valve hemodynamics and the detection of valve stenosis or regurgitation. In this
case, the TTE was unable to adequately assess the aortic prosthesis. Leaflet motion
and thrombus may be visualized by TTE in some patients, but transesophageal
echocardiography is more sensitive for the detection of valve dysfunction and
thrombosis. By the data provided, the patient has elevated filling pressures and a right
heart catheterization would not be the best initial step. A gated chest computed
tomography scan or fluroscopy may also be useful to evaluate mechanical prosthesis
motion (Class IIa). A ventilation-perfusion scan is useful in the evaluation of pulmonary
embolism not acute HF. Blood cultures are indicated for suspected endocarditis or
unexplained fever in the setting of a prosthetic valve; however, there is a low suspicion
for infection, and valve thrombus should be evaluated first. The elevated troponin and
mild ST abnormality by ECG is most likely due to demand ischemia secondary to acute
HF. He has no chest pain, therefore coronary angiography should not be the initial
study. Answer D
Key Point
Doppler echocardiography (transthoracic echocardiography and transesophageal
echocardiography) is the method of choice for the diagnosis of prosthetic valve
dysfunction (stenosis and regurgitation).

Question 18
18- A 56-year-old female presents for evaluation of a 2-year history of progressive
dyspnea on exertion, which has worsened over the past 4 months. Presently she cannot
climb a flight of stairs without dyspnea. Her medical history is significant for
hypertension. Her medications include metoprolol succinate 50 mg daily.

On examination her heart rate is 80 bpm and her blood pressure is 136/80 mm Hg. Her
jugular venous pressure is elevated to 9 cm H20 with prominent V waves. A left
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parasternal lift is present. The first heart sound is normal in intensity. The pulmonic
component of the second heart sound is increased. Both a soft grade 2/4 diastolic
rumble and a 3/6 holosystolic murmur are heard at the apex. At the left sternal border a
grade 2/6 systolic ejection murmur is present and increases with inspiration. A pulsatile
liver is evident with mild lower extremity edema.

A transthoracic echocardiogram demonstrates an ejection fraction of 65% and fusion of

the mitral valve (MV) commissures. The mean transmitral gradient is 14 mm Hg at a
heart rate of 72 bpm. The calculated valve area by the pressure half-time equation is
1.0 cm2. The MV is pliable with no commissural calcification. Moderate to severe mitral
regurgitation and severe tricuspid regurgitation are present. The right ventricle is mildly
dilated with generalized hypokinesis. The pulmonary artery systolic pressure is
estimated at 60 mm Hg.

Which of the following is the next best step in the management of this patient?
A. MV replacement.
B. Metoprolol succinate 100 mg daily.
C. Exercise stress echocardiogram.
D. Balloon mitral valvuloplasty.

The patient has severe symptomatic mitral stenosis (MS) and regurgitation. Although
her MV is pliable, the presence of moderate or worse mitral regurgitation is a
contraindication to balloon valvuloplasty (Figure 1).

Surgical MV replacement is a Class I indication in patients with severe symptomatic MS

(New York Heart Association Class III-IV) who are not a high risk for surgery and who
are not candidates for mitral valvuloplasty.

Increase in beta-blocker therapy is not adequate for management of severe

symptomatic MS.

An exercise stress echocardiogram would be indicated only if there was a discrepancy

between the clinical findings and the results of the echocardiogram. Answer A

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Key Point
Percutaneous mitral balloon commissurotomy is indicated in symptomatic patients or
asymptomatic patients with pulmonary hypertension, moderate or severe stenosis, and
favorable valve morphology in the absence of left atrial thrombus or moderate to severe
mitral regurgitation.

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Question 19
19- A 34-year-old male is admitted with a fever. He has felt poorly for several months.
He has had a 10-lb weight loss and recurring night sweats. In the past few weeks, he
has experienced progressive dyspnea. He is on no medications at this time. His social
history includes ongoing intravenous drug use.

His temperature is 38.5° Celcius, heart rate is 103 bpm, blood pressure is 95/50 mm
Hg, and resting oxygen saturation on room air is 96%. His lungs reveal bibasilar fine
rales. His jugular venous pressure is not elevated. His apex is hyperdynamic, and there
is a grade 2/6 systolic murmur and grade 2/4 diastolic murmur along the left sternal
border. The first heart sound (S1) is soft and there is an S3. He has no edema. There
are splinter hemorrhages under the nailbeds on his left fingers.

His echocardiogram reveals a hyperdynamic left ventricle with evidence for severe
aortic regurgitation (AR) and vegetations on his aortic valve (Figure 1).

In addition to broad spectrum antibiotics, which of the following is the most appropriate
next step in the management of this patient?

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(Figure 1)
A. Aortic valve replacement.
B. Intravenous esmolol 0.1 mg/kg/min.
C. Intra-aortic balloon pump.
D. Phenylephrine 40 mcg/min.

This patient has evidence of infective endocarditis (IE) of the aortic valve resulting in
severe AR and heart failure (HF). His exam is consistent with AR further supported by
premature closure of the mitral valve (MV) on the m-mode echocardiogram shown.
Early surgical intervention is a Class I indication for patients with IE and valve
dysfunction causing HF.

His echocardiography reveals preclosure of the MV due to the rapidly rising left
ventricular diastolic pressure as a result of his severe AR. This also results in the soft
S1 observed. As opposed to chronic AR, the difference between the aortic and left
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ventricular pressures in diastole may be small in acute AR and there may be little
diastolic murmur. Likewise the pulse pressure may not be wide, and there may be none
of the classic hemodynamic findings of chronic AR. Schematic hemodynamic tracings of
chronic versus acute AR are shown in Figure 2.

A transesophageal echocardiogram would be indicated in patients with aortic valve

endocarditis to better assess the vegetation sizes and to help define the presence of an
aortic abscess, but the procedure should not delay surgical intervention and can be
performed intraoperatively.

Severe AR is a contraindication to the intra-aortic balloon pump. His tachycardia is

appropriate for his serious hemodynamic state and beta-blockers therefore would not be
appropriate. In addition, the reduced heart rate from a beta-blocker would increase
diastolic time and the duration of AR per beat. Phenylephrine would increase afterload,
which is contraindicated in severe AR. Answer A

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Key Point
Transesophageal echocardiography is indicated for diagnosis and evaluation of
vegetation size, abscess formation, fistula formation, leaflet perforation, or prosthetic
valve dehiscence.

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Question 20
20-A 75-year-old male presents with 3 days of chest pain and worsening dyspnea on
exertion. His past medical history includes hypertension and osteoarthritis. His
medications include aspirin 81 mg daily, chlorthalidone 25 mg daily, and amlodipine 5
mg daily.

On examination his blood pressure is 125/60 mm Hg, heart rate is 95 bpm, and jugular
venous pressure is 12 cm. His lungs have bibasilar crackles. There is a harsh systolic
murmur heard throughout the precordium without inspiratory augmentation. At the apex,
there is a musical murmur that is louder after a premature ventricular contraction (PVC).
There is no peripheral edema.

His electrocardiogram shows Q waves in the inferior leads.

Which of the following is most likely to be seen on cardiac catheterization of this

patient?
A. Large right ventricular outflow tract to pulmonary artery pressure gradient.
B. Large V wave on right atrial pressure tracing.
C. Large V wave on pulmonary capillary wedge pressure tracing.
D. Increased pulmonary artery oxygen saturation.
E. Large left ventricle to aorta gradient.

The patient has degenerative or age-related aortic stenosis (AS), the most common
etiology of AS. On physical examination, he demonstrates Gallavardin phenomenon—a
harsh murmur at the base with a musical murmur at the apex. This is due to the high-
frequency components of the AS murmur radiating to the left ventricular (LV) apex. It
can be confused with the murmur of mitral regurgitation (MR) except that the murmur of
AS is a systolic ejection murmur rather than holosystolic. The murmur of AS also
increases with bradycardia or after a pause, such as after a PVC; an MR murmur would
not change.

This is not tricuspid regurgitation, which would be holosystolic and best heard at the left
lower sternal border. Nor is this pulmonic stenosis, which would typically best be heard
at the left upper sternal border. Both of these right-sided murmurs would become louder
with inspiration.

If cardiac catheterization were performed, it would demonstrate a large LV to aorta

pressure gradient, consistent with AS. A large V wave on pulmonary capillary wedge
pressure is consistent with significant MR. A large V wave on the right atrium is
consistent with significant tricuspid regurgitation. Increased pulmonary artery (PA)
oxygen saturation suggests the presence of a left-to-right shunt, such as a ventricular
septal defect (VSD). A VSD murmur is typically continuous and heard at the sternal

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border, not the apex. A large right ventricular outflow tract to PA pressure gradient is
consistent with pulmonic stenosis. Answer E
Key Point
As the murmur of aortic stenosis (AS) worsens, the ejection sound and the intensity of
A2 diminish; the murmur peaks later in systole. The AS murmur increases after a
premature ventricular contraction.

Question 21
21-A 43-year-old female with a history of endocarditis status post mechanical aortic
valve replacement (AVR) 3 years prior presents with a 2-week history of progressive
dyspnea on exertion. She has a history of nonadherence to medications.

On examination, she is anxious and short of breath. Her temperature is 36.8 degrees
Celcius, blood pressure is 96/60 mm Hg, and heart rate is 106 bpm. Her oxygen
saturation is 93% (2L nasal cannula). Her lungs have crackles halfway up the lung
fields. There is a soft S1 and S2. There is a 2/6 systolic ejection murmur and a 3/4
diastolic murmur at the left sternal border with an S3 gallop. There is no peripheral
edema.

Her interantional normalized ratio is 1.4.

Her transesophageal echocardiogram (TEE) reveals normal left ventricular (LV) size
with preserved LV function, LV ejection fraction of 60%. The peak velocity is 3.8 m/sec
with a mean aortic gradient of 32 mm Hg. There is severe aortic regurgitation by color
Doppler with a steep pressure half-time slope. Cinefluoroscopy is shown in Video 1.

Intravenous (IV) heparin is started and furosemide 40 mg IV is given.

Which of the following is the next best step in the management of this patient?

Vedio consistent with stuck valve

A. Transcatheter AVR.
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B. Tissue plasminogen activator 10 mg IV bolus.

C. Surgical AVR.
D. Vancomycin 1 g IV.

This patient has prosthetic valve thrombosis with heart failure symptoms, a Class I
indication for emergent surgery. Cineflouroscopy or computed tomography is often used
as an adjunct to TEE for the diagnosis of prosthetic valve dysfunction.

Fibrinolytic therapy is a Class IIa indication if there is a right-sided thrombus or for a

small left-sided prosthetic valve thrombus in the setting of New York Heart Association
class I-II symptoms of <14 days onset.

There is no indication of infective endocarditis so empiric vancomycin is not appropriate.

Transcatheter AVR is not indicated for prosthetic valve thrombosis. Answer C

Key Point
Doppler echocardiography (transthoracic echocardiography and transesophageal
echocardiography) is the method of choice for the diagnosis of prosthetic valve
dysfunction (stenosis and regurgitation).

Question 22
22-A 34-year-old female is referred to you with shortness of breath and the new onset of
atrial fibrillation (AF). She has no past medical history and takes no medications. Her
vital signs are a pulse of 100 bpm that is irregular, a blood pressure of 110/66 mm Hg,
and respirations of 14 breaths per minute. Her physical exam reveals a 2/4 blowing
diastolic murmur best heard at the apex in end expiration, with an opening snap in
diastole, and a 3/6 holosystolic murmur heard at the apex and radiating to the axilla.

Transthoracic echocardiography demonstrates normal left ventricular (LV) size with a

LV ejection fraction of 66%. There is mitral stenosis (MS; mean gradient of 15 mm Hg at
98 bpm and a mitral valve area of 1.3 cm2) and mild mitral regurgitation (MR). Her

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estimated pulmonary systolic pressures are elevated at 64 mm Hg and her left atrial
size is 49 ml/m2.

Which of the following is the next most appropriate study for this patient?
A. Repeat transthoracic echocardiogram in 6 months.
B. Exercise stress echocardiogram.
C. Transesophageal echocardiography.
D. Right heart catheterization.

Transesophageal echocardiography should be performed in patients planning

percutaneous balloon mitral commissurotomy (PBMC) to assess for the presence and
degree of MR and to rule out left atrial or left atrial appendage thrombus prior to the
procedure (Class I). PBMC is indicated in symptomatic patients with severe MS (Class
I) and in asymptomatic patients with new onset AF and favorable valve morphology
(Class IIB) (Figure 1).

The guideline says that exercise testing or hemodynamic assessment is indicated when
there is a discrepancy between resting echocardiogram and clinical symptoms and
signs in the evaluation of MS. There is no discrepancy regarding the severity of MS in
this case and so no further testing is required prior to planning therapy.

Periodic monitoring would only be appropriate for a patient with asymptomatic severe
MS. Answer C

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Key Point
Percutaneous mitral balloon commissurotomy is indicated in symptomatic patients or
asymptomatic patients with pulmonary hypertension, moderate or severe stenosis, and
favorable valve morphology in the absence of left atrial thrombus or moderate to severe
mitral regurgitation.

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Question 23
23-A 52-year-old female presents to the emergency department with acute shortness of
breath.

Her vital signs include a heart rate of 110 bpm, a blood pressure of 90/60 mm Hg, and a
room air saturation of 90%. She is tachypneic sitting upright. There are rales bilaterally
and a grade 2/6 holosystolic murmur with an associated S3.

Her echocardiogram is shown (Videos 1 and 2).

She undergoes cardiac catheterization with the following pertinent findings:

• Normal coronaries
• Right atrial pressure mean of 10 mm Hg
• Pulmonary artery pressure 65/35 mm Hg (mean 45 mm Hg)
• Pulmonary capillary wedge pressure V wave of 40 mm Hg (mean of 33 mm Hg)
• Aortic gradient 100/80 mm Hg
• Left ventricular pressure 100/29 mm Hg
• Cardiac output 3.2 L/min
• Cardiac index 1.8 L/min/m2

Which of the following is the next best step in the management of this patient?
Video 1 : TTE Apical 4ch , flail post leaflet
Video 2: With colour consistent with severe MR anteriorly directed
A. Norepinephrine 5 mcg/kg/min.
B. Inhaled nitric oxygen 10 ppm.
C. Percutaneous mitral valve repair.
D. Surgical mitral valve repair/replacement.
E. Percutaneous left ventricular assist device.

This woman has acute severe mitral regurgitation from chordal rupture. The correct
answer is urgent surgical intervention.

Medical management may include diuresis and afterload reduction with vasodilators or
an intra-aortic balloon pump, but should be done while preparing for urgent surgical
intervention. Norepinephrine is not indicated because it offers inotropic support with
vasoconstriction. Neither percutaneous left ventricular assist device nor percutaneous
mitral valve repair have been studied in this setting.

Her pulmonary hypertension is secondary to her high pulmonary capillary wedge

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pressure (group II pulmonary hypertension) so there is no indication for pulmonary

vasodilator therapy. Answer D

Question 24
24-The patient is an 85-year-old male who presents to the emergency department with
dyspnea and malaise. He has a past medical history of severe aortic stenosis status
post transcatheter aortic valve replacement approximately 5 years ago. He describes
increasing fatigue, dyspnea, and a low grade fever for the past 7 days. On exam, his
temperature is 38.1 degrees Celsius (100.6 degrees Fahrenheit), heart rate is 98 bpm,
blood pressure is 112/50 mm Hg, respiratory rate is 18 respirations per minute, pulse
oximetry is 96% on room air. He has normal S1 and S2 heart sounds with a 2/4
decrescendo diastolic murmur present along the left sternal border. His lungs are clear.
The remainder of his physical examination is normal.

In addition to a transthoracic echocardiogram, which of the following is the most

appropriate next step in the management of this patient?
A. Empiric antibiotics.
B. Obtain two sets of blood cultures.
C. Administer intravenous furosemide.
D. An influenza nasal swab.
E. A chest X-ray.

The patient has a prosthetic aortic valve and is at risk for infective endocarditis (IE). It is
important that two sets of blood cultures are obtained in patients who are at risk for IE,
including those with congenital or acquired valvular heart disease, previous IE,
prosthetic heart valves, certain congenital or heritable heart malformations,
immunodeficiency states, or who are injection drug users. Blood cultures are positive in
>90% of patients with IE and should be obtained at separate time intervals prior to the
administration of antibiotics. This patient has an unexplained fever for >48 hours and a
left-sided regurgitant murmur with a high likelihood for IE. Thus, the most appropriate
next step in management is to obtain two sets of blood cultures.

Blood cultures must be obtained before starting empiric antibiotics. He has no evidence
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of pulmonary edema and therefore furosemide is not warranted. While a chest X-ray
and a flu swab may be considered, neither are the essential first steps in management
of this patient. Answer B
Key Point
The diagnosis of infective endocarditis is based on a constellation of history, clinical
findings, laboratory studies (particularly blood cultures), and echocardiography. Use of
an imaging algorithm reduces unnecessary use of echocardiography.

Question 25
25-A 36-year-old female presents to you for evaluation. She has a known history of
mitral valve prolapse (MVP). An echocardiogram one month prior shows an ejection
fraction of 70% with bileaflet MVP and moderate mitral regurgitation (MR). She is able
to hike one hour on the weekends without limitations.

On examination, her body mass index is 30 kg/m2, heart rate is 70 bpm, blood pressure
is 128/70 mm Hg, jugular venous pressure is 2 cm H 20, and her lungs are clear. Her
cardiac exam shows a mid-systolic click and late-systolic murmur radiating to the axilla.
Her extremities are warm without edema.

Which of the following do you recommend for this patient?

A. Losartan 25 mg daily.
B. Lisinopril 5 mg daily.
C. Nifedipine sustained release 30 mg daily.
D. Sacubitril/valsartan 24/26 mg twice daily.
E. No change in therapy.

Vasodilator therapy is not indicated for normotensive asymptomatic patients with

chronic primary MR and normal left ventricular (LV) systolic function.

Because vasodilator therapy appears to be effective in acute severe symptomatic MR, it

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seems reasonable to attempt afterload reduction in chronic asymptomatic MR with

normal LV function in an effort to forestall the need for surgery. However, the results
from the limited number of trials addressing this therapy have been disappointing,
demonstrating little or no clinically important benefit. Conversely, because vasodilators
decrease LV size and mitral closing force, they may increase MVP, worsening rather
than decreasing the severity of MR. The foregoing does not apply to patients with
concomitant hypertension. Hypertension must be treated because of the well known
morbidity and mortality associated with that condition and because increased LV
systolic pressure by itself increases the systolic transmitral gradient and worsens the
severity of MR.

The correct answer is no therapy as she is not hypertensive so vasodilator therapy with
an angiotensin-converting enzyme inhibitor, angiotensin-receptor blocker, angiotensin
receptor–neprilysin inhibitor, or nifedipine is not indicated.
Answer E
Key Point
Echocardiography is the most useful diagnostic test to inform the etiology and severity
of mitral regurgitation. Echocardiographic grading of more-than-trivial mitral
regurgitation should include quantitative criteria beyond color jet dimensions, particularly
if jets are nonholosystolic or eccentric.

Question 26
26-A 22-year-old female presents for routine follow-up of mitral stenosis (MS). She was
hospitalized with a prolonged febrile illness at the age of 13. She currently runs 4 miles
in 35 minutes several times per week. She denies palpitations. She is on no medication
except birth control pills.

On physical examination she is 67 inches tall and weighs 127 lbs. Her blood pressure is
122/72 mm Hg and her heart rate is regular at 76 bpm. Her lungs are clear. Her jugular
venous pressure is 4 cm H2O. The first heart sound is increased. Both components of
the second heart sound are normal. A soft early diastolic rumble is present that is
preceded by a crisp opening snap. No peripheral edema is present.
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Her echocardiogram shows fusion of the mitral commissures. The mean transmitral
gradient is 4 mm Hg at a heart rate of 88 bpm. The calculated mitral valve (MV) area is
2.3 cm2 by the pressure half-time method. Trivial mitral regurgitation is present. The MV
appears pliable (echocardiographic score = 4).

Which of the following is the next best step in the care of this patient?
A. Vitamin K antagonist therapy.
B. Percutaneous balloon mitral valvotomy.
C. Oral penicillin V 250 mg twice daily.
D. Surgical MV replacement.
E. Transesophageal echocardiography.

The correct answer is penicillin prophylaxis which should be initiated the time of
presentation.

Figure 1 outlines the guideline options that include oral penicillin V twice daily, monthly
benzathine penicillin G intramuscular injection, or daily sulfadiazine. Figure 2 outlines
the duration of therapy. For those patients with residual valvular disease, the
recommended duration is to continue penicillin prophylaxis for 10 years from the last
episode of acute rheumatic fever or until the age of 40. If there was acute carditis, but
no residual valve disease, the recommendation is for 10 years or until age 21
(whichever is longer). If there was rheumatic fever without carditis, the recommendation
is for 5 years or until the age of 21 (whichever is longer).

The patient has evidence of rheumatic MS on physical examination and

echocardiography. However, the MS is mild and she is asymptomatic with no evidence
of atrial fibrillation or pulmonary hypertension. Thus, there is no indication for either
percutaneous or surgical intervention.

A transesophageal echocardiogram would be indicated to rule out left atrial thrombus in

patients being considered for percutaneous balloon mitral valvotomy and is thus not
indicated in this case.

A vitamin K antagonist would be required if atrial arrhythmias were present or there was
evidence for emboli or an atrial thrombus. Answer C

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Question 27
27-A 52-year-old male with a history of hypertension and remote occult gastrointestinal
bleeding presumed to be secondary to atrioventricular malformation is referred to your
clinic for evaluation of a new murmur. His physical examination is notable for a blood
pressure of 126/74 mm Hg, heart rate of 72 bpm, and a late peaking systolic murmur.
His echocardiogram reveals a bicuspid aortic valve (BAV) with moderate to severe
aortic stenosis and a dilated ascending aorta. He is referred for surgical evaluation.
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What is the next best step in the management of this patient?

A. BAV replacement.
B. Transcatheter aortic valve replacement.
C. Mechanical aortic valve replacement.
D. Ascending aorta.
E. Medical management.

In patients who are being treated with long-term vitamin K antagonist (VKA)
anticoagulation before valve surgery, a mechanical valve may be appropriate, given its
greater durability compared with a bioprosthetic valve and the need for continued VKA
anticoagulation even if a bioprosthetic valve is implanted. However, if interruption of
VKA therapy is necessary for noncardiac procedures, bridging therapy with other
anticoagulants may be needed if a mechanical valve is present, whereas stopping and
restarting VKA therapy for other indications may be simpler. Specific clinical
circumstances, comorbid conditions, and patient preferences should be considered
when deciding between a bioprosthetic and mechanical valve in patients receiving VKA
therapy for indications other than the prosthetic valve itself. Answer A
Key Point
Selection of valve procedure (repair vs. replacement, surgical vs. transcatheter) and
prosthetic valve type (mechanical vs. bioprosthetic) has to be individualized according
to the patient’s characteristics, including age, surgical risk, and personal preferences.

Question 28
28-A 55-year-old female is referred to you for evaluation and treatment of aortic
regurgitation (AR). She has a known history of bicuspid aortic valve (BAV). She
exercises four to five times per week on an upright stationary bicycle and reports no
symptoms. Her medical history includes hyperlipidemia. Her current medications include
atorvastatin 40 mg.

On physical examination her blood pressure is 126/54 mm Hg with a heart rate of 84

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bpm. Her lungs are clear to auscultation. The left ventricular (LV) apical impulse is
normal and nondisplaced. A soft, grade 2/4 diastolic decrescendo murmur is present at
the left sternal border. A systolic ejection click is noted. There is no S 3 or S4, and no
peripheral edema.

Transthoracic echocardiogram demonstrates an LV ejection fraction of 59% with an

end-systolic dimension of 33 mm and an end-diastolic dimension of 50 mm. The aortic
root diameter is 3.9 cm at the sinuses of Valsalva. There is a BAV with severe AR.

Which of the following is the next best step the treatment of this patient?
A. Metoprolol succinate 25 mg.
B. No additional medications.
C. Enteric coated aspirin 81 mg.
D. Extended release nifedipine 30 mg.
E. Enalapril 5 mg.

In the absence of other indications (e.g., hypertension), vasodilator therapy is not

indicated in asymptomatic patients with AR and normal systolic function. It is a Class I
indication to use vasodilators, preferably dihydropyridine calcium channel blockers,
angiotensin-converting enzyme inhibitors, or angiotensin receptor blockers, to treat
hypertension when present in patients with AR.

For patients with AR, long-term therapy with vasodilators is indicated in those with
severe regurgitation and symptoms or LV dysfunction only when surgical intervention
cannot be performed due to comorbidities (Class IIa).

There is no indication for aspirin or beta-blockers for modification of her risk for eventual
aortic valve replacement. Answer B
Key Point
Surgery remains the mainstay of therapy. Medical therapy is limited to individuals with
chronic aortic regurgitation and hypertension, and it is limited in those who are not
surgical candidates because of comorbidities.

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Question 29
29-A 67-year-old male with chronic severe aortic regurgitation (AR) and trileaflet aortic
valve presents to the cardiology clinic for routine outpatient follow-up. He denies any
dyspnea with walking at a moderate pace, mowing his lawn, or other usual activities.
His exam is notable for a II/IV diastolic decrescendo murmur along the left sternal
border. His jugular venous pressure is normal, his lungs are clear, and there is no
peripheral edema. An echocardiogram is obtained.

If present, which of the following echocardiographic findings would support surgical

aortic valve replacement (AVR) for this patient?
A. A left ventricular ejection fraction of 55%.
B. An aortic root of 4.8 cm at the sinuses of Valsalva.
C. A left ventricular end systolic dimension of 52 mm.
D. A left ventricular end diastolic dimension of 61 mm.
E. Holodiastolic flow reversal in the abdominal aorta.

In asymptomatic patients with chronic severe AR, indications for AVR include: a left
ventricular (LV) ejection fraction of <50% (Class I), an LV end systolic dimension of >50
mm (Class IIa), progressive LV enlargement with an LV end diastolic dimension of >65
mm if the patient is a low surgical risk (Class IIb), and if undergoing cardiac surgery for
other indications (Class I). Holodiastolic flow reversal in the abdominal aorta is a highly
specific sign for severe aortic insufficiency, but is not by itself an indication for surgery.
In patients with bicuspid aortic valve who have an indication for AVR, the ascending
aorta should be replaced if its diameter is >4.5 cm. Otherwise, the indication for
ascending aorta replacement is a diameter of >5.5 cm. This patient has a trileaflet aortic
valve. Answer C
Key Point
Left ventricular dysfunction and dilatation can be reversible if valve replacement is
performed in a timely fashion. Therefore, serial follow-up with clinical examinations and
echocardiography is recommended to identify patients who require intervention before
symptoms develop.

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Question 30
30- A 48-year-old male with a history of myxomatous valve disease and moderate mitral
regurgitation (MR) on echocardiogram presents today for his annual reassessment. He
has a history of hyperlipidemia and remote endocarditis. He feels well and rides his road
bike most days of the week without limiting symptoms. His exam is notable for a heart
rate 52 bpm, blood pressure of 118/74 mm Hg, and pulse oximetry of 98%. His lungs
are clear without rales. His cardiac exam reveals a soft, late systolic murmur at the
apex. There is no lower extremity edema.

Which of the following would be recommended for this patient?

A. Antibiotics prior to dental cleaning.
B. Aspirin 81 mg daily.
C. Cardiac magnetic resonance imaging.
D. Transesophageal echocardiogram.
E. Losartan 25 mg daily.

Although myxomatous mitral valve disease alone is not an indication for endocarditis
prophylaxis in the current guideline, this patient has a history of prior endocarditis and
therefore should receive antibiotic prophylaxis before dental procedures.

Losartan or other vasodilators are not indicated in MR unless the patient has
hypertension. A transesophageal echocardiogram or magentic resonance can be
helpful to better delineate the severity of the regurgitation and the mechanism.
However, this patient is asymptomatic with only moderate MR and therefore additional
imaging would not change management. Although patients with MR have an increased
risk of developing atrial fibrillation, there is no evidence to support prophylactic aspirin.
He is not in the age range for aspirin prophylaxis for primary prevention.Answer A

Question 31
31- A 48-year-old male with a history of myxomatous valve disease and moderate mitral
regurgitation (MR) on echocardiogram presents today for his annual reassessment. He
has a history of hyperlipidemia and remote endocarditis. He feels well and rides his road
bike most days of the week without limiting symptoms. His exam is notable for a heart
rate 52 bpm, blood pressure of 118/74 mm Hg, and pulse oximetry of 98%. His lungs

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are clear without rales. His cardiac exam reveals a soft, late systolic murmur at the
apex. There is no lower extremity edema.

Which of the following would be recommended for this patient?

A. Antibiotics prior to dental cleaning.
B. Aspirin 81 mg daily.
C. Cardiac magnetic resonance imaging.
D. Transesophageal echocardiogram.
E. Losartan 25 mg daily.

Although myxomatous mitral valve disease alone is not an indication for endocarditis
prophylaxis in the current guideline, this patient has a history of prior endocarditis and
therefore should receive antibiotic prophylaxis before dental procedures.

Losartan or other vasodilators are not indicated in MR unless the patient has
hypertension. A transesophageal echocardiogram or magentic resonance can be
helpful to better delineate the severity of the regurgitation and the mechanism.
However, this patient is asymptomatic with only moderate MR and therefore additional
imaging would not change management. Although patients with MR have an increased
risk of developing atrial fibrillation, there is no evidence to support prophylactic aspirin.
He is not in the age range for aspirin prophylaxis for primary prevention. Answer A

Question 32
32- A 44-year-old male with a history of bicuspid aortic valve (BAV) comes in to your
office for initial evaluation. He plays basketball regularly and reports no cardiovascular
symptoms. His family history is significant for aortic dissection and repair in his father.
On exam his blood pressure is 132/72 mm Hg, heart rate is 68 bpm, and body mass
index is 26 kg/m2. He has a 3/6 mid-to-late peaking systolic murmur heard best at the
base. He undergoes an echocardiogram which reveals normal biventricular size and
function. His peak transaortic velocity is 3.4 m/sec, peak transaortic gradient is 46 mm
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Hg, and mean trans aortic gradient is 29 mm Hg. His calculated aortic valve area is 1.1
cm2. His ascending aorta measures 5.1 cm.

Which of the following is the next most appropriate step in the care of this patient?
A. Transesophageal echocardiogram.
B. Cardiac magnetic resonance imaging.
C. Repeat the echocardiogram in 1 year.
D. Exercise stress testing.
E. Surgical referral.

In this patient with BAV and aortopathy, consideration of surgical repair should occur
when the aortic size is >5.5 cm, there is rapidly increasing size (>0.5 cm/year), or as in
this patient, a family history of dissection (Class IIa). If the transthoracic imaging quality
is adequate, imaging with magnetic resonance or transesophageal imaging is not
indicated. If the patient does not elect to undergo surgery, then repeating the
echocardiogram in 1 year is indicated. Given his high level of activity, exercise stress
testing is not likely to add additional information about his functional status. Answer E

Question 33
33- A 70-year-old female presents for a follow-up office visit following recent cardiac
testing for fatigue, abdominal distention, and lower extremity swelling. She has a history
of paroxysmal atrial fibrillation and sick sinus syndrome requiring a dual chamber
pacemaker. On exam, she has jugular venous distension, normal heart sounds, no
murmur, abdominal distention with a palpable free liver edge, and significant lower
extremity swelling.

A right heart catheterization was performed revealing a right atrial (RA) pressure of 12
mm Hg (v waves 35 mm Hg), right ventricular (RV) pressure of 35/15 mm Hg,
pulmonary artery pressure of 35/12 mm Hg, and pulmonary capillary wedge pressure of
12 mm Hg.

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A transthoracic echocardiogram revealed severe eccentric tricuspid regurgitation (TR)

with holosystolic flow reversal in the hepatic veins. Tricuspid valve leaflets were not
thickened and without apical displacement. Anterior and posterior leaflets exhibited
normal motion. Restricted septal leaflet motion was present. RV basal diastolic diameter
was 45 mm.

What is the most likely cause of this patient's TR?

A. Carcinoid syndrome.
B. Ebstein's anomaly.
C. Rheumatic heart disease.
D. Pacemaker lead.
E. Pulmonary arterial hypertension.

This patient has symptomatic severe tricuspid regurgitation (TR) with evidence of right
heart failure on examination. The etiology of severe TR can typically be established
using clinical history and echocardiographic findings. Though not performed for this
patient, transesophageal echocardiography can help to determine the cause of severe
TR. Primary disorders causing TR include rheumatic heart disease, prolapse, congenital
disease (Ebstein's), infective endocarditis, radiation, carcinoid syndrome, blunt chest
wall trauma, RV endomyocardial biopsy-related trauma, and intra-annular RV
pacemaker or implantable cardioverter defibrillator leads. Approximately 80% of cases
of significant TR are functional in nature and related to tricuspid annular dilation and
leaflet tethering in the setting of RV remodeling due to pressure and/or volume
overload.

For this patient, the right heart catheterization reveals significantly elevated RA
pressures with elevated v waves in the absence of pulmonary hypertension. The valve
leaflets are not thickened which excludes carcinoid and rheumatic disease. Rheumatic
tricuspid valve disease typically includes diffuse leaflet thickening with restriction of
opening due to commissural fusion, chordal shortening, and calcification with
characteristic diastolic doming. In carcinoid syndrome, liver metastases produce 5-
hydroxyindoleacetic acid which causes a unique echocardiographic appearance of the
RV including leaflets that are short, thick, and with systolic and diastolic restriction. The
patient does not have echocardiographic characteristics of Ebstein's anomaly, a
congenital disease characterized by apical displacement of the tricuspid valve septal
and posterior leaflets from the atrioventricular ring. The septal and posterior leaflets are
typically adherent to the underlying myocardium with an apical displacement of ≥8
mm/m2 compared with the mitral valve annulus.

Thus, the most likely cause of the patient's severe TR is restriction caused by the
patient's pacemaker lead. Endocardial leads can impair the structure and function of the
tricuspid valve. Injury can also occur during implantation or extraction. Chronic
interaction between the endocardial leads and tricuspid valve leaflets and/or chords can
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result in inflammation and fibrosis leading to entrapment of the lead. The resulting TR
will typically exhibit eccentric rather than a central trajectory, with the septal leaflet being
the most common leaflet to be entrapped. Answer D
Key Point
The main cause of tricuspid regurgitation (TR) is secondary or secondary TR due to
annulus dilation.

Question 34
34- A 65-year-old male presents to your office for routine follow-up. He has a past
medical history of symptomatic severe aortic stenosis secondary to bicuspid aortic valve
(AV) disease. Approximately 1 year ago, he underwent surgical AV replacement using a
21 mm bioprosthetic valve. His postoperative course was uneventful.

He now presents to your clinic 1 year later and reports exertional fatigue with
ambulating one block that has been present since surgery. On exam, he has a normal
first and second heart sound with a 3/6 mid-late peaking systolic murmur that is best
heard at the right upper sternal border. Body surface area is 2.18 m2. His remaining
physical examination is unremarkable.

The following AV Doppler measurements were obtained at 2 and 12 months: 2 month

left ventricular outflow tract (LVOT) diameter 20 mm; LVOT velocity time integral (VTI)
22 cm; AV VTI 65 cm; effective orifice area (EOA) 1.06 cm2; AV mean gradient 28 mm
Hg; acceleration time 92 msec; 12-month LVOT diameter 20 mm; LVOT VTI 25 cm; AV
VTI 70 cm; EOA 1.12 cm2; AV mean gradient 36 mm Hg; acceleration time 97 msec.

Which of the following is the most likely cause for this patient's elevated transaortic
gradients?
A. Pannus formation.
B. Patient-prosthesis mismatch.
C. Hemolytic anemia.
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D. Pressure recovery.
E. Valve thrombosis.

The patient has elevated gradients across his AV prosthesis, with a higher than
expected mean gradient and a smaller than expected EOA. Thus, it is important to
consider prosthetic valve obstruction as a cause for elevated prosthetic valve gradients.
Obstruction can be functional (i.e., patient-prosthesis mismatch [PPM]) or pathologic
(e.g., thrombus or pannus formation). It is important to calculate the indexed EOA by
dividing the EOA by the body surface area. The indexed EOA at 2 and 12 months are
0.49 and 0.52 cm2/m2, respectively. When the indexed EOA is ≤0.65 cm2/m2, it suggests
severe PPM. Additionally, an acceleration time is usually ≤100 msec in PPM, whereas it
is >100 msec with pathologic obstruction. The Doppler velocity index ([DVI]; i.e., LVOT
VTI/AV VTI) is 0.34 and 0.36 at 2 and 12 months respectively. PPM will typically have a
DVI >0.25, whereas in pathological obstruction it will be <0.25. Additionally, the Doppler
gradients are stable over time without a significant change, making pathological
obstruction less likely. Taken together, the correct answer is PPM, which is the most
common cause of elevated gradients across a surgical bioprosthetic valve.

Hemolytic anemia can can cause increased cardiac output and therefore increased
gradients, however it will not affect the DVI or indexed EOA. Pressure recovery
phenomenon causing elevated gradients is more common in mechanical prostheses.
Answer B
Key Point
Patient prosthesis mismatch (PPM) is the most frequent cause of high transprosthetic
gradients following aortic valve replacement or mitral valve replacement. The differential
diagnosis between PPM and acquired prosthetic valve stenosis can be made by: 1)
assessing leaflet morphology and mobility; 2) comparing measured echocardiographic
parameters of prosthetic valve function to normal reference values; and 3) assessing
serial changes in the echocardiographic parameters during follow-up.

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Question 35
35-A 58-year-old female presents to your office with a 6-month history of palpitations
and dyspnea on exertion. She has no medical problems and takes no medications.

Her examination reveals a body mass index of 30 kg/m2, a heart rate of 68 bpm, and a
blood pressure 160/80 mm Hg. Her jugular venous pressure is 6 cm H20. Her cardiac
exam reveals a systolic murmur with a single component S2.

What test is most likely to elucidate the diagnosis?

A. Injection of agitated saline contrast.
B. Chest X-ray.
C. Electrocardiogram.
D. Doppler tricuspid regurgitation velocity.
E. Doppler aortic velocity across the aortic valve.

This patient presents with dyspnea on exertion as well as a systolic murmur, but the
telltale finding on examination is the single S2. A single S2 can be caused by pulmonic
stenosis, severe aortic stenosis, congenital absence of the pulmonic valve, or in
patients in whom cardiac auscultation is hindered by body habitus, emphysema, or
pericardial effusion. Doppler velocity across the aortic valve would assess for aortic
stenosis and thus is the correct answer.

An electrocardiogram (ECG) would show a right or left bundle branch block, but the
former would result in a widely split S2 and the latter a paradoxically split S2. Thus, ECG
is not the correct answer.

Agitated saline contrast would assess for an atrial septal defect which would cause a
fixed split S2.

Doppler of the tricuspid regurgitant jet would assess the estimated right ventricular
systolic pressure, an indicator of pulmonary hypertension, which would cause a fixed
split S2 with increased intensity of P2.

A chest X-ray may show rib notching as seen in aortic coarctation. This would not result
in an abnormality of the second heart sound and thus is not the correct answer. Answer
E
Key Point
A single S2 may be present in severe pulmonary stenosis or aortic stenosis,
transposition of the great arteries, or pulmonary hypertension.

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Question 36
36-A 67-year-old female presents for follow-up for her valvular heart disease. Her
history is significant for hypertension, hyperlipidemia, and aortic insufficiency. Her
medications include hydrochlorothiazide 25 mg daily and aspirin 81 mg. Her exam is
significant for a blood pressure (BP) of 160/90 mm Hg, a heart rate of 72 bpm, a 2/4
holodiastolic murmur heard best at the apex, and a soft 1/6 holosystolic murmur heard
best at the apex. Her creatinine is 0.8 mg/dl. She undergoes echocardiography which
revealed normal left ventricular cavity size and function, moderate aortic insufficiency,
and mild mitral regurgitation.

Which of the following is most appropriate in the care of this patient?

A. The addition of verapamil extended release 120 mg daily.
B. Increase hydrochlorothiazide to 50 mg daily.
C. The addition of metoprolol succinate 50 mg daily.
D. The addition of losartan 25 mg daily.
E. The addition of hydralazine 10 mg three times daily.

This patient has stage B aortic regurgitation (AR) and hypertension. Treatment of
hypertension (systolic BP >140 mm Hg) is recommended in patients with chronic AR
(stages B and C), preferably with dihydropyridine calcium channel blockers or
angiotensin-converting enzyme inhibitors/angiotensin-receptor blockers (e.g., losartan;
Class 1, Level of Evidence B). Beta-blockers may be less effective because the
reduction in heart rate is associated with an even higher stroke volume, which
contributes to the elevated systolic pressure in patients with chronic severe AR. While
each of the other answer options may help with BP control, they are not the next best
choice of antihypertensive therapy.
Answer D
Key Point
Surgery remains the mainstay of therapy. Medical therapy is limited to individuals with
chronic aortic regurgitation and hypertension, and it is limited in those who are not
surgical candidates because of comorbidities.

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Question 37
37- A 50-year-old male is hospitalized following an inferior ST-segment elevation
myocardial infarction (MI). He had late revascularization of an occluded, dominant right
coronary artery. On day 3 he developed acute breathlessness. His vital signs are
noteworthy for a heart rate of 110 bpm, a blood pressure of 102/80 mm Hg, and a
respiratory rate of 28 breaths per minute with an oxygen saturation of 90% on high flow
oxygen. His physical exam reveals wet rales bilaterally. He is tachycardic, but cardiac
auscultation reveals no murmur. His 12-lead electrocardiogram shows sinus tachycardia
with inferior Q waves and <1 mm ST-segment depression.

What is the most appropriate next step in the management of this patient?
A. Procalcitonin.
B. B-type natriuretic peptide.
C. Coronary angiography.
D. Pulmonary artery catheter placement.
E. Echocardiogram.

This patient is in extremis from acute mitral valve regurgitation (MR), possibly as a
consequence of papillary muscle rupture. In this scenario, inferior MR is likely
associated with posteromedial papillary muscle rupture. Acute severe MR may produce
a brief systolic murmur or no murmur at all. Rapid diagnosis is essential. A delay in the
time to surgery appears to increase the risk of further myocardial injury, organ failure,
and death.

To confirm the diagnosis, an urgent echocardiogram is needed. Transthoracic

echocardiography may be sufficient, however eccentric MR may be under-appreciated
on transthoracic imaging, in which case transesophageal imaging would be needed.

The other answer options would fail to diagnose the problem in a timely manner.
Pulmonary artery catheter placement might reveal an elevated wedge pressure tracing
with a V-wave, but this is supportive data as opposed to direct confirmation of MR. One
might consider right heart catheterization data useful in the setting of an ischemic
ventricular septal rupture (VSR), where an oxygenation step-up would be noted,
however the clinical vignette doesn't include features of a VSR such as a loud murmur;
and in the case of VSR, pulmonary edema would be an uncommon feature. Coronary
angiography would be warranted if there was concern for reinfarction, but the clinical
vignette is not supportive of reinfarction. B-type natriuretic peptide and procalcitonin
levels are not likely to be helpful in the acute management of a patient with a suspected
mechanical complication of MI. Answer E
Key Point
The murmur of acute mitral regurgitation (MR) differs from that of chronic MR because

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of the rapid rise in left atrium pressures in the acute setting. The acute MR murmur may
be short.

Question 38
38-A 45-year-old female with history of a bileaflet mechanical aortic valve replacement
for bicuspid aortic valve disease 10 years ago is admitted with complaints of dyspnea
on exertion. She has had sporadic medical care over the past year. She reports
occasional recent subjective fevers. Her past medical history is otherwise
unremarkable. On examination she is afebrile with a heart rate of 90 bpm and a blood
pressure of 110/75 mm Hg. She has clear lung fields. On cardiac auscultation there is a
soft systolic murmur.

Which of the following would be the most likely finding on this patient's
echocardiogram?
A. An aortic valve acceleration time 70 msec.
B. A dimensionless index of 0.44.
C. A left ventricular outflow tract gradient of 40 mm Hg.
D. A peak aortic velocity 4.1 m/sec.
E. An effective orifice area 1.5 cm2.

This patient has prosthetic aortic valve stenosis in the setting of sporadic medical care,
suspicious for valve thrombosis from inadequate anticoagulation. Transthoracic
echocardiography is the first-line test for diagnosing prosthetic valve dysfunction.
Expected findings would be an elevated transvalvular velocity and gradient; a prolonged
(>100 msec) acceleration time; a reduced effective orifice area (<1 cm2); and a reduced
dimensionless index (<0.3).
A left ventricular outflow tract gradient would not be expected to occur in this setting and
would also be expected to cause a dynamic murmur. Answer D

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Question 39
39-An 88-year-old female presents to clinic for progressive dyspnea on exertion and
lower extremity edema. Her past history includes osteoarthritis and hypertension. She
takes aspirin 81 mg daily, metoprolol succinate 25 mg daily, amlodipine 5 mg daily, and
naproxen as needed.

On exam, her blood pressure is 130/60 mm Hg, pulse is 70 bpm, and respirations are
16 breaths per minute. Her jugular venous pressure is 10 cm H 20. There are bibasilar
crackles. She has a late-peaking, harsh systolic murmur along the right upper sternal
border with a single S2. There is 2+ bilateral lower extremity edema.

Her echocardiogram shows a severely calcified aortic valve with reduced leaflet
excursion. Peak velocity across the valve is 3.3 m/sec with a mean gradient of 28 mm
Hg. The calculated valve area was 0.8 cm2 and dimensionless index is 0.22. The left
ventricle (LV) is mildly dilated with a left ventricular ejection fraction (LVEF) of 25-30%
and global hypokinesis.

Which of the following is the most appropriate next step in the management of this
patient?
A. Exercise myocardial perfusion scan.
B. Right and left heart catheterization.
C. Cardiac magnetic resonance imaging.
D. Transesophageal echocardiography.
E. Dobutamine stress echocardiography.

Most patients with severe aortic stenosis (AS) present with a high transvalvular gradient
and velocity. However, a subset present with severe AS despite a low gradient and
velocity due either to concurrent LV systolic dysfunction (LVEF of <50%) or a low
transaortic stroke volume with preserved LV systolic function.

Outcomes in severe low-flow, low-gradient AS are improved with aortic valve

replacement (AVR) compared with medical therapy particularly when contractile reserve
is present. The American Society of Echocardiography/European Association of
Echocardiography recommendations for clinical practice defines severe AS on
dobutamine stress testing as a maximum velocity of >4.0 m/sec with a valve area of 1.0
cm2 at any point during the test protocol, with a maximum dobutamine dose of 20
mcg/kg per minute. On the basis of outcome data in several prospective nonrandomized
studies, AVR is reasonable in these patients (Class IIa). LVEF typically increases by 10
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LVEF units and may return to normal if afterload mismatch was the cause of the LV
systolic dysfunction. Some patients without contractile reserve may also benefit from
AVR, but decisions in these high-risk patients must be individualized because there are
no data indicating who will have a better outcome with surgery.

Exercise stress testing is contraindicated in patients with severe symptomatic AS and

nuclear perfusion imaging would not be expected to add useful information in this
setting.

Right and left heart catheterization are invasive procedures and generally reserved for
cases with indeterminate noninvasive test results.

The role of cardiac magnetic resonance imaging for low-flow, low-gradient AS has not
been established.

Transesophageal echocardiography would not be expected to add additional

information. Answer E

Key Point
With low-flow, low-gradient aortic stenosis (AS) and a reduced left ventricular ejection
fraction (<50%), severe AS is defined as a velocity of 4 m/sec and a valve area of ≤1.0
cm2, at any flow rate, on low-dose dobutamine stress echocardiogram.

Question 40
40- A 51-year-old male is referred for evaluation of a chronic murmur. At 20 years of
age, he underwent surgical repair of bicuspid aortic valve (BAV) stenosis. The patient
has been followed intermittently and has no other significant medical history. He is
physically active and asymptomatic, regularly running 5 miles without limitation.

On physical examination his heart rate is regular at 60 bpm and he has a blood
pressure of 135/55 mm Hg. His lungs are clear to auscultation. Left ventricular (LV)
apical impulse is enlarged and laterally displaced to the anterior axillary line. A grade
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2/6 early-peaking systolic murmur and a decrescendo grade 2/4 diastolic murmur both
are present along the left sternal border. The S2 is soft. There is no S3or S4. Peripheral
examination demonstrates a pulsatile uvula and a collapsing water hammer radial
pulse.
The patient undergoes a transthoracic echocardiogram that demonstrates BAV. LV
end-diastolic dimension is 6.5 cm, LV end-systolic dimension is 4.2 cm, and LV ejection
fraction is 57%. There is severe aortic regurgitation by color Doppler. Atrioventricular
(AV) regurgitant volume is 70 ml, AV regurgitant fraction is 60%, and aortic root
diameter is 4.2 cm.

Which of the following is the next best step in the management of this patient?
A. Symptom-limited exercise stress test.
B. Serial echocardiography.
C. Surgical aortic valve replacement.
D. Transcatheter aortic valve replacement.

This patient has asymptomatic severe aortic regurgitation (AR). Surgical aortic valve
replacement (AVR) is a Class I indication if the LV ejection fraction is <50% or if the
patient is undergoing cardiac surgery for another indication. For an asymptomatic
patient with severe AR, surgical AVR is a Class IIa recommendation when there is an
end-systolic dimension of >5.0 cm and a Class IIb indication when there is an LV end-
diastolic dimension of >6.5 cm (Figure 1).

As intervention is not currently indicated for this patient, the most appropriate
recommendation is to repeat the echocardiogram in 6-12 months.

A symptom-limited exercise stress test is reasonable to clarify functional capacity in

patients with equivocal symptoms, but is not indicated in a patient who can run 5 miles
without limitations.

Transcatheter AVR is not indicated in this asymptomatic patient and is also not
indicated for management of AR. Answer B

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Key Point
Left ventricular dysfunction and dilatation can be reversible if valve replacement is
performed in a timely fashion. Therefore, serial follow-up with clinical examinations and
echocardiography is recommended to identify patients who require intervention before
symptoms develop.

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Question 41
41- A 66-year-old female is postoperative Day 2 from cardiac surgery for symptomatic
mitral regurgitation. A 31 mm mechanical bileaflet mitral prosthesis was placed and a
tricuspid annuloplasty was performed. She had postoperative atrial fibrillation which
spontaneously resolved within 24 hours. Her medical history is significant only for
hypertension. A preoperative coronary angiogram demonstrated normal coronary
arteries.

Which of the following is the most appropriate long-term antithrombotic therapy for this
patient?
A. Warfarin with an international normalized ratio goal of 3.0-4.0.
B. Warfarin with an international normalized ratio goal of 2.5-3.5.
C. Aspirin 81 mg daily and warfarin with an international normalized ratio goal of 2.0-3.0.
D. Aspirin 81 mg daily and warfarin with an international normalized ratio goal of 2.5-3.5.

For patients with a mechanical mitral valve prosthesis, warfarin with an with an
international normalized ratio (INR) goal of 3 (2.5-3.5) in addition to aspirin 75-100 mg
daily is a Class I recommendation.

Higher INR goals may be considered in patients who have thromboembolic events while
anticoagulated within this INR range. This patient has not had a thromboembolic event
therefore a higher INR goal would not be indicated.

Warfarin alone is not adequate. Randomized trials have demonstrated a reduction of

thromboembolic events with the addition of aspirin (75-100 mg daily) to warfarin
anticoagulation in patients with mechanical valves.
Answer D

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Question 42
42- You are asked to consult on a 70-year-old male with shortness of breath and a
murmur. His past medical history includes hypertension, hypercholesterolemia, and
arthritis. His current medications include aspirin 81 mg, atenolol 50 mg, atorvastatin 80
mg, and naproxen 375 mg as needed. His vital signs are a pulse rate of 70 bpm, blood
pressure of 110/76 mm Hg, and his respiration rate is 16 breaths per minute. His
physical exam is remarkable for jugular venous distension to 8 cm above the sternal
angle, right basilar crackles, a soft holosytolic murmur, and 1+ bilateral ankle edema.

An echocardiogram is performed (Videos 1, 2, 3).

What is the most likely etiology of this patient's mitral valve disease?
Long axis view : RWMA at inferolateral wall
Long axis view, with color : Severe MR posterior directed
Short axis view : RWMA at inferolateral wall
A. Coronary atherosclerosis.
B. Postinfectious inflammation.
C. Congenital deformation.
D. Dystrophic calcification.
E. Myxoid degeneration.

This patient has functional or secondary mitral regurgitation (MR) caused by

inferolateral hypokinesis of the left ventricle with tethering of the lateral papillary muscle;
the most likely cause is right coronary or left circumflex territory coronary artery disease.
MR can be classified according to leaflet motion, which gives clues to the differential
diagnosis of the cause (Figure 1).

Type I MR involves normal leaflet motion. This can be caused by endocarditis with
perforation of a leaflet or by a cleft mitral valve (congenital).

Type II MR is due to excessive leaflet motion, either flail or prolapse, due to fibroelastic
deficiency or mitral valve prolapse syndrome.

Type III MR is due to restricted leaflet motion. Type IIIa MR is due to restricted diastolic
and systolic motion and may be caused by mitral annular calcification or rheumatic
disease (post-streptococcal infection). Type IIIb MR is due to restricted systolic motion
and is caused by left ventricular dysfunction, as in this case. Answer A

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Key Point
Anatomic or functional abnormalities of the mitral annulus, leaflets, subvalvular
apparatus, or left ventricle may result in mitral regurgitation.

Question 43
43- A 52-year-old male with no prior medical history presents to the emergency
department with 2 weeks of progressive dyspnea, lower extremity edema, and
orthopnea. Exam is notable for a temperature 39.1 degrees Celsius, an irregular heart
rate of 125 bmp, a blood pressure of 110/85 mm Hg, a respiratory rate 33 breaths per
minute, and a pulse oximetry of 85% on room air. His echocardiogram shows a left
ventricular ejection fraction (LVEF) of 72% and an 0.8 cm mass on the anterior mitral
valve leaflet with severe eccentric mitral regurgitation due to leaflet perforation. Cultures
are obtained and he is started on intravenous antibiotics and furosemide. He is taken to
the operating room where a 25 mm mechanical mitral valve is placed. His postoperative
course is complicated by acute kidney injury and anemia requiring blood transfusions.

He is now 2 months postoperative and is being seen in your office. He denies shortness
of breath, but does admit to fatigue. On exam his heart rate is 78 bpm and blood
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pressure is 125/72 mm Hg. A crisp mechanical S1 is heard. His lungs are clear and
there is no edema. His current medications include aspirin 81 mg daily, warfarin, and
metoprolol tartrate 50 mg twice a day. His laboratory results are notable for hemoglobin
8.8 g/dl, creatinine 1.1 mg/dl, and international normalized ratio 3.5 (INR; range of 1.7-
4.2 over the past month).

What is the next best step in management of this patient?

A. Switch to metoprolol succinate 100 mg daily.
B. Stop aspirin.
C. Perform transthoracic echocardiogram.
D. Switch to rivaroxaban.
E. Change INR target to 2-2.5.

An echocardiographic examination performed 6 weeks to 3 months after valve

implantation is essential to establish a baseline for comparison should complications or
deterioration occur later (Class I recommendation, Level of Evidence B). Despite the
anemia, the aspirin should not be stopped as it is indicated along with a vitamin K
antagonist in patients with mechanical prosthetic valves. While metoprolol succinate is
preferred in patients with heart failure with reduced ejection fraction, there is no clear
benefit in patients following valve replacement with normal LVEF. Anticoagulant therapy
with oral direct thrombin or anti-Xa agents should not be used in patients with
mechanical valve prostheses (Class III recommendation), therefore switching to
rivaroxaban is incorrect. Answer C
Key Point
Periodic clinical and echocardiographic evaluation of patients with a prosthetic valve is
essential for early detection of prosthetic valve dysfunction. Careful medical
management of patients with prosthetic valves includes careful control of antithrombotic
therapy and prescription of infective endocarditis prophylaxis.

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Question 44
44- A 56-year-old female with a history of mitral valve prolapse (MVP) and a recent
dental procedure presents to the emergency department with fevers and shortness of
breath. An echocardiogram one year prior showed bileaflet MVP with trace mitral
regurgitation (MR). She has no other medical problems and takes no medications. On
examination her temperature was 102.4 F, heart rate was 110 bpm, and blood pressure
was 102/70 mm Hg. She is diaphoretic and appears to be in mild distress. Her jugular
venous pressure is 6 cm H20. Her lungs have bibasilar fine inspiratory crackles. Her
heart is regular with a soft systolic murmur and soft S3 gallop. Her abdomen is soft and
nontender; her extremities are warm without edema. Her echocardiogram shows an
ejection fraction of 65% and thickening of the mitral valve leaflets with moderate MR.

Development of which the following would be an indication to perform a

transesophageal echocardiogram?
A. Atrial fibrillation.
B. Streptococcus bovis bacteremia.
C. Worsening pulmonary edema.
D. Persistent fever after 48 hours.
E. Acute deep vein thrombosis of the femoral vein.

Transthoracic and/or transesophageal echocardiograpy (TEE) are recommended for the

re-evaluation of patients with infective endocarditis (IE) who have a change in clinical
signs or symptoms (e.g., new murmur, embolism, persistent fever, heart failure [HF],
abscess, or atrioventricular heart block) and in patients at a high risk of complications
(e.g., extensive infected tissue/large vegetation on initial echocardiogram
or staphylococcal, enterococcal, fungal infections).

HF, perivalvular extension, and embolic events represent three of the most frequent and
severe complications of IE. They are also the three main indications for early surgery,
which is performed in almost 50% of cases. If signs or symptoms consistent with any of
these complications exist, there should be a very low threshold for repeat imaging in
these patients. Conversely, in the absence of clinical deterioration or new
signs/symptoms, routine follow-up echocardiography is probably of only limited clinical
utility.

In this patient, worsening HF is the indication to perform TEE to assess for progressive
valvular destruction that may be an indication for urgent surgery. Perivalvular abscesses
can extend into adjacent cardiac conduction tissues, leading to heart block. Involvement
of the conducting system is most common in the setting of aortic valve infection,
especially when there is involvement of the valve ring between the right and
noncoronary cusp; this anatomic site overlies the intraventricular septum that contains
the proximal ventricular conduction system.
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However, atrial fibrillation would not be considered a sign of a perivalvular abscess and
thus not an indication for repeat imaging. Fever associated with IE should resolve after
3-5 days of antibiotic therapy. A fever noted after 48 hours in the absence of other
symptoms or signs would not be considered a failure of therapy and thus further
imaging would not be indicated. Streptococcus bovis (S. bovis) is a common cause of
IE, but unlike IE caused by Staphylococcus species, enterococcus, and fungal
species, S. bovis IE usually responds to antibiotic therapy so the growth of S. bovis from
blood cultures alone would not be a reason to perform repeat imaging. Septic emboli
can occur with IE resulting in stroke, renal or splenic infarcts, ischemia of the
extremities, myocardial infarction or, in the case of right-sided IE, pulmonary embolism.
However, deep vein thrombosis (DVT) would not be a form of septic emboli and thus
the presence of an acute DVT would not warrant further imaging. Answer C
Key Point
Transesophageal echocardiography is indicated for diagnosis and evaluation of
vegetation size, abscess formation, fistula formation, leaflet perforation, or prosthetic
valve dehiscence.

Question 45
45- A 60-year-old male presents for evaluation of a murmur. He exercises by walking
his dog two miles daily without limitations. He has a history of hypertension and
hyperlipidemia. His medications include amlodipine 5 mg daily and atorvastatin 20 mg
daily.

On examination, his heart rate is 70 bpm, blood pressure is 128/80 mm Hg, and jugular
venous pressure is 4 cm H20. His lungs are clear. His cardiac exam shows a soft
systolic ejection murmur radiating to the carotid arteries. His extremities have no
edema.

His echocardiogram shows an ejection fraction of 60% and aortic stenosis (AS) with a
peak velocity of 2.3 m/sec, mean gradient of 13 mm Hg, and a valve area of 1.8 cm2.

In the absence of new symptoms, what is the appropriate interval for a repeat
echocardiogram in this patient?
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A. 2 years.
B. 6 months.
C. 3 years.
D. 6 years.
E. 1 year.

The timing of periodic clinical evaluation of patients with severe asymptomatic AS

depends on comorbidities and patient-specific factors. Transesophageal
echocardiogram for re-evaluation of asymptomatic patients with AS with normal left
ventricular systolic function who have no change in signs or symptoms is performed at
intervals of 6 months to 1 year when aortic velocity is 4.0 m/sec (stage C), 1-2 years
when aortic velocity is 3.0-3.9 m/sec (stage B), and 3-5 years when aortic velocity is
2.0-2.9 m/sec (stage B; Table 1).

Valvular AS is a progressive disease, and an increase in hemodynamic severity is

inevitable once even mild AS is present. The rate of progression of the stenotic lesion
has been estimated in a variety of invasive and noninvasive studies. When severe AS is
present (aortic velocity of 4.0 m/sec), the rate of progression to symptoms is high with
an event-free survival of only 30-50% at 2 years. Therefore, patients with asymptomatic
severe AS require frequent monitoring for progressive disease because symptom onset
may be insidious and not recognized by the patient.

Once even moderate AS is present (aortic velocity between 3.0-3.9 m/sec), the average
rate of progression is an increase in velocity of 0.3 m/sec per year, an increase in mean
pressure gradient of 7 mm Hg per year, and a decrease in valve area of 0.1 cm2 per
year. There is marked individual variability in the rate of hemodynamic change.
Progression of AS can be more rapid in older patients and in those with more severe
leaflet calcification. Because it is not possible to predict the exact rate of progression in
an individual patient, regular clinical and echocardiographic follow-up is mandatory in all
patients with asymptomatic mild-to-moderate AS.

In his patient with mild (Stage B) AS, a repeat echocardiogram in 3-5 years is
appropriate. Answer C

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Question 46
46- You are seeing an 80-year-old female for shortness of breath. She reports dyspnea
with usual activites such as walking in the grocery store. She has hypertension, stage 3
chronic kidney disease, hyperlipidemia, and osteoporosis. Her current medications
include lisinopril, simvastatin, calcium plus vitamin D, and aspirin. Her cardiac exam
reveals a soft systolic ejection murmur with a normally split S2. She has arthritic
changes of the joints of both of her hands. Her exam is otherwise unremarkable.

An echocardiogram shows a mean transmitral gradient 6 mm Hg at heart rate of 70

bpm.

Which of the following most likely represents the appearance of this patient's mitral
valve?

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Although the vast majority of mitral stenosis (MS) in the world results from rheumatic
heart disease, senile calcific MS is found with increasing frequency in the elderly
population in North America. This is due to calcification of the mitral annulus and
calcification that extends into the leaflets, which cause both a narrowing of the annulus
and a rigidity of the leaflets without commissural fusion. Mitral annular calcification
(MAC) has been associated with decreased renal function and inflammatory markers
like C-reactive protein; however, senile calcific MS is common in the elderly population
with normal renal function and is associated with senile aortic stenosis (AS).

Indications for intervention in patients with senile calcific MS are different from those for
rheumatic MS for the following reasons: first, because calcification involves the annulus
and base of the leaflets without commissural fusion, there is no role for percutaneous
mitral balloon or surgical commissurotomy; and second, the presence of severe MAC
can be quite challenging for the surgeon because it causes problems in securely
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attaching the prosthetic valve and narrowing of the orifice. Supra-annular insertion and
other innovative techniques can be used, such as placement of a felt patch around the
valve orifice to anchor the prosthesis; however, this only works if the mitral orifice is
adequate. If the annular calcification narrows the orifice, it has to be debrided. The other
alternative is left atrial to ventricular bypass with a valved conduit in extreme cases of
calcification both of the leaflet and the annulus. Finally, patients with calcification are
often elderly and debilitated, have multiple comorbidities, and are at high risk for
surgery. For these reasons, intervention should be delayed until symptoms are severely
limiting and cannot be managed with diuresis and heart rate control.

A subset of patients have mitral inflow obstruction due to other causes, such as
congenital malformations, tumors, or other masses. Congenital MS usually takes the
form of a parachute mitral valve, where the mitral chordae are attached to a single or
dominant papillary muscle and often form a component of the Shone complex, which
can include supramitral rings, valvular or subvalvular AS, and aortic coarctation. For MS
caused by tumors or other obstructive lesions, intervention is aimed at reducing or
removing the mass, with efforts made to preserve the valve.

This patient has a history consistent with senile calcific MS as she is older and has renal
dysfunction. Thus, the image with echodensity along the posterior mitral annulus is the
best response. Answer C
Key Point
Mitral annular calcium is common, with 45% of patients >65 years of age exhibiting
evidence for this. It can occasionally lead to both mitral regurgitation and mitral stenosis.

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Question 47
47- A 50-year-old female with a history of a murmur was referred to the valve clinic at a
large tertiary referral hospital. She denies any symptoms. Her exam is normal except for
a blowing 3/6 holosystolic murmur at the apex that radiates to the base of the heart. Her
transthoracic echocardiogram was of good quality and revealed a normal ejection
fraction (EF; 68%) with normal left ventricular (LV) dimensions and severe eccentric
anteriorly-directed mitral regurgitation (MR) secondary to posterior leaflet prolapse. Her
predicted risk of mortality with surgical mitral valve (MV) repair or replacement is <1%.

Which of the following is the most appropriate next step in the management of this
patient?
A. Cardiac magnetic resonance imaging.
B. Repeat transthoracic echocardiogram in 2 years.
C. Transcatheter MV repair.
D. Surgical MV replacement.
E. Surgical MV repair.

This patient has asymptomatic primary severe MR (stage C1) with a normal EF and
normal LV dimensions. Patients with asymptomatic severe primary MR with a surgical
risk of <1% and and a high likelihood of successful MV repair (>95%) may be
considered for surgical MV repair to prevent long-term sequela and adverse remodeling
(Class IIa). MV repair by an experienced surgeon is preferred over replacement. There
is no indication for transcather repair as the patient is at a low risk for surgery. If
observation is chosen, a repeat echocardiogram should be obtained in 6-12 months for
severe MR. As the echocardiogram was of good diagnostic quality, cardiac magnetic
resonance imaging is not indicated. Answer E
Key Point
Surgical repair rates and operative mortality rates are strongly influenced by operator
and center experience.

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Question 48
48- A 28-year-old female who is 26 weeks pregnant presents to the emergency
department with a 2-week history of progressive dyspnea and palpitations. She has a
history of a prolonged febrile illness as a child. Her examination reveals an irregular
heart rate of 140 bpm with a blood pressure of 95/60 mm Hg. Her jugular venous
pressure is 4 cm above the sternal angle. A grade 2/4 diastolic rumble is heard at the
apex. Her lungs are clear to auscultation with no peripheral edema.
Her electrocardiogram shows atrial fibrillation (AF) at 144 bpm.
She is given intravenous metoprolol 5 mg and feels somewhat better.
Her transthoracic echocardiogram shows a left ventricular ejection fraction of 60%. The
mean mitral valve (MV) gradient is 12 mm Hg at a heart rate of 88 bpm. The calculated
MV area by the pressure half-time equation is 1.3 cm2. There is mild mitral regurgitation.
The peak pulmonary artery pressure is 50 mm Hg.
In addition to anticoagulation, which of the following is the next best step in the
management of this patient?

A. Urgent direct current cardioversion.

B. Metoprolol tartrate 12.5 mg every 6 hours.
C. Sotalol 80 mg twice daily.
D. Percutaneous balloon valvuloplasty.

This pregnant woman presents with rheumatic mitral stenosis and AF with rapid
ventricular response. The initial treatment will be medical therapy to lower her heart rate
and anticoagulation. Thus, metoprolol is the correct answer.

There is no role for cardioversion at this time, as she now has appropriate rate control
with associated improvement in symptoms. In addition, chemical or electrical
cardioversion is not indicated as she has not been previously anticoagulated and the
duration of AF is unknown; she has been symptomatic for 2 weeks.

If symptoms fail to improve with additional medical therapy, future options include
transesophageal echocardiogram-guided direct current cardioversion. Indications for
intervention (either percutaneous or surgical) are symptoms refractory to medical
management. If medical management fails, balloon valvuloplasty (with pelvic shielding)
would be the procedure of choice if the valve morphology is appropriate. Surgical MV
replacement during pregnancy carries a 30% risk of fetal loss and is therefore a last
resort. Answer B
Key Point
Percutaneous mitral balloon commissurotomy is indicated in symptomatic patients or
asymptomatic patients with pulmonary hypertension, moderate or severe stenosis, and
favorable valve morphology in the absence of left atrial thrombus or moderate to severe
mitral regurgitation.
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Question 49
49-A 30-year-old female presents to your clinic with progressive shortness of breath.
The symptoms started 2 years ago and now occur with minimal activity. She has no
significant past medical history. She is on no medications. Her physical exam reveals an
early opening snap followed by a low frequency murmur.

A cardiac echocardiogram showed thickening of the mitral valve leaflets with doming.
The mean gradient was 5 mm Hg at a ventricular rate of 60 bpm.

What is the next best step in the management of this patient?

A. Exercise stress myocardial perfusion imaging.
B. Transesophageal echocardiography.
C. Right heart catheterization.
D. Exercise stress echocardiography.
E. Polysomnography.

This patient has symptoms and exam findings consistent with significant mitral stenosis
(MS), but her resting echocardiographic findings are suggestive of mild MS. Exercise
testing with Doppler or invasive hemodynamic assessment is recommended to evaluate
the response of the mean mitral gradient and pulmonary artery pressure in patients with
MS when there is a discrepancy between resting Doppler echocardiographic findings
and clinical symptoms or signs.

Right heart catheterization is an invasive test and should be reserved for cases of
indeterminate noninvasive test findings. Furthermore, direct measurement of left
ventricular diastolic pressure would be required to assess the mitral valve; right heart
catheterization alone would be inadequate in this case.

She does not report any daytime somnolence that point to sleep apnea, so
polysomnography would not be the next best step.

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Stress perfusion imaging to test for coronary ischemia would not be expected to explain
her valve findings.

Transesophageal echocardiography will be required to plan therapy once a diagnosis of

severe MS is made, but will not resolve the discrepancy between her resting
echocardiogram findings and her symptoms. Answer D
Key Point
The echocardiogram is the diagnostic mainstay and is used to assess the etiology,
morphology, and severity of the mitral stenosis. The analysis of the morphology of the
mitral valve apparatus (including leaflet thickness and mobility, leaflet calcification,
subvalvular thickening, and chordal fusion) and the appearance of the commissures are
key features for the diagnosis of rheumatic MS. They also have important implications
for the choice of the most appropriate intervention.

Question 50
50- A 50-year-old male presents to your office for follow-up of aortic stenosis (AS) and
an aortic aneurysm. He is active and exercises regularly without symptoms. He takes no
medications.

On physical examination his blood pressure is 110/70 mm Hg with a heart rate of 66

bpm. The carotid contour is normal. A systolic click and grade 2/6 early-peaking systolic
ejection murmur are heard at the right upper sternal border. The aortic component of
the S2 is preserved. There is no diastolic murmur and no S3 or S4. His peripheral
examination shows no edema.
His transthoracic echocardiogram demonstrates bicuspid aortic valve (BAV) and the
following:

• Left ventricular ejection fraction of 60%

• Left ventricular end-diastolic dimension of 5.0 cm
• Ascending aorta dimension of 4.6 cm
• Aortic root diameter of 3.8 cm
• Left atrial volume index of 32 ml/m2
• Mean aortic valve gradient of 10 mm Hg
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• Aortic valve area of 2.0 cm2

When should the next echocardiogram for surveillance be performed on this

patient?

A. 2 years.
B. 1 year.
C. 3 years.
D. 5 years.

For patients with a BAV and an ascending aortic aneurysm >4.5 cm, annual imaging of
the aneurysm is recommended (Class I). For patients with mild AS without an
ascending aortic aneurysm, serial echocardiography every 3-5 years is recommended
in the absence of a change in clinical status or physical findings. Echocardiography is
recommended annually for asymptomatic patients with severe AS and every 1-2 years
in those with moderate AS. Patients should be advised to report changes in symptoms
promptly. Answer B
Key Point
Careful monitoring with periodic clinical evaluation for symptoms and serial
echocardiogram for disease severity is essential to determine appropriate timing for
intervention.

51- A 60-year-old male with a past medical history significant for hypertension and
diabetes mellitus presents with fever, chills , malaise, and shortness of breath for 5
days. On presentation the patient was febrile with a blood pressure of 100/40 mm Hg.
An early, soft, high-pitched, decrescendo diastolic murmur was heard on his exam. His
blood cultures were positive for Streptococcus bovis. A cardiac echocardiogram
revealed a bicuspid aortic valve with severe aortic regurgitation and an aortic valve
replacement procedure is scheduled.

Prior to this patient's surgery, which of the following studies should be performed first?
A. Computed tomography of the abdomen and chest.
B. Fecal immunochemical test.
C. No further testing is needed.
D. Guaiac fecal occult blood test.
E. Colonoscopy.

Colon cancer screening is mandatory if the pathogen is Streptococcus (S.) bovis. The
association between colonic carcinoma and endocarditis was reported as early as 1951,
but it was only in 1977 that S. gallolyticus (previously S. bovis) was recognized by Klein
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et al. as the pathogen agent specifically related to the presence of a colonic cancer.
Answer E
Key Point
Streptococcus bovis endocarditis is often associated with malignancy of the
gastrointestinal track.

Question 52
52- A 40-year-old female is noted to have a late systolic murmur on routine outpatient
follow-up. She is physically active and enjoys running and cycling. She denies any
symptoms related to physical exertion. A transthoracic echocardiogram is performed.
This demonstrates normal left ventricular (LV) size and function with an ejection fraction
of 65%. Left atrial (LA) size is normal. There is prolapse of the P2 segment of the of the
mitral valve. A proximal isovelocity surface area (PISA) radius of 1 cm is measured at
an aliasing velocity of 40 cm/sec. The maximum velocity of the regurgitant jet is 5
m/sec. The velocity time integral (VTI) of the regurgitant jet is 30 cm.

Which of the following quantitative echocardiographic features most accurately reflects

this patient's mitral regurgitation (MR) severity?
A. MR jet area.
B. Vena contracta width.
C. Regurgitant volume.
D. MR jet maximum velocity.
E. Effective regurgitant orifice area.

This question highlights the importance that systolic duration of MR plays when
assessing the severity of MR. Parameters which are measured in a single frame like
PISA, vena contracta width (VCW), or jet area can lead to significant overestimation of
the severity of MR, especially in the setting of late systolic MR. The presence of normal
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LV and LA size should raise the possibility of overestimation by quantitative measure in

this asymptomatic patient.

When calculated using the data from the echocardiogram, the instaneous effective
regurgitant orifice area (EROA) is 0.5 cm2, which is consistent with severe MR (see
calculations below). However, the calculated regurgitant volume is in the mild range.
This is most likely secondary to late systolic MR as opposed to holosystolic MR. This is
commonly seen in mitral prolapse where there is late systolic regurgitation yielding a
small regurgitant volume. MR duration is a common reason for discrepancy between the
calculated EROA and regurgitant volume. The VCW profile of the MR regurgitant will be
helpful in defining the duration of MR.

The MR jet would be expected to be anteriorly directed in this patient with posterior
leaflet prolapse.

The regurgitant fraction cannot be calculated based on the information provided but
would be expected to be low with a regurgitant volume of 15 ml/beat.

EROA = [(2) x (3.14) x (PISA r2) x (Aliasing velocity)]/Vmax MR jet

EROA = [2 x 3.14 x 12 x 40] / 500

EROA = 0.5 cm2

Regurgitant volume is then calculated according the formula:

regurgitant volume = EROA x VTI of MR jet

regurgitant volume = 0.5 cm2 x 30 cm

regurgitant volume = 15 cm3.Answer C

Key Point
Echocardiography is the most useful diagnostic test to inform the etiology and severity
of mitral regurgitation. Echocardiographic grading of more-than-trivial mitral
regurgitation should include quantitative criteria beyond color jet dimensions, particularly
if jets are nonholosystolic or eccentric.

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Question 53
53- A 27-year-old female who is 30 weeks pregnant presents to the emergency
department with several days of progressive dyspnea on exertion. She had a similar
presentation to the emergency department 3 weeks ago, and was given prescriptions
for furosemide 40 mg once daily and metoprolol 25 mg twice daily.

Her vital signs are a heart rate of 102 bpm, a blood pressure 100/60 mm Hg, and an
oxygen saturation 100% on 2 liters nasal cannula oxygen. Her physical examination
reveals bilateral rales two-thirds of the way up the posterior lung fields. There is an
elevated jugular venous pressure to the angle of the mandible, a 1/6 holosystolic
murmur at the apex with a 2/4 diastolic murmur, and an opening snap. There is 1+
pitting lower extremity edema.

Her electrocardiogram shows sinus tachycardia. Her echocardiogram reveals normal

left ventricular function with fusion of the mitral commissures and a mean transmitral
valve gradient of 16 mm Hg at a heart rate of 90 bpm with trivial mitral regurgitation. The
valve leaflet tips are thickened with minimal involvement of the subvalvular chords and
no significant calcification. The calculated mitral valve (MV) area by the pressure half-
time equation is 1.5 cm2. The estimated right ventricular systolic pressure is 50 mm Hg.

In addition to diuresis, which of the following is the best next step for the management
of this patient?
A. Percutaneous mitral commissurotomy.
B. Intravenous metoprolol 5 mg every 6 hours.
C. Delivery followed by MV replacement.
D. Percutaneous edge-to-edge MV repair.
E. MV replacement.

Percutaneous MV commissurotomy (valvuloplasty) is a Class IIa recommendation in the

absence of significant mitral regurgitation (MR) and a favorable Wilkins Score in the
2014 American Heart Association/American College of Cardiology valvular disease
guidelines for pregnant patients with Class III or IV heart failure symptoms despite
medical therapy. If she were not pregnant, percutaneous balloon MV commissurotomy
would be a Class I indication for severe symptomatic mitral stenosis (MS) and favorable
valve morphology.

Delivery and then surgery would not be optimal management at this point, unless for a
specific obstetric indication, due to issues related to prematurity.

Her symptoms have progressed despite diuretic and beta-blocker therapy, thus current
medical therapy would not be the best choice.

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Percutaneous mitral edge-to-edge MV repair (MitraClip) is not indicated as the patient

has MS, not MR. Answer A
Key Point
Percutaneous mitral balloon commissurotomy is indicated in symptomatic patients or
asymptomatic patients with pulmonary hypertension, moderate or severe stenosis, and
favorable valve morphology in the absence of left atrial thrombus or moderate to severe
mitral regurgitation.

Question 54
54- A 55-year-old female presents to the emergency department with fever and
shortness of breath 3 months after mitral valve replacement with a bioprosthesis. Her
past medical history includes mitral regurgitation due to Barlow's disease, hypertension,
and hypothyroidism. Her current medications include aspirin, warfarin, lisinopril, and
levothyroxine. On physical exam her vital signs are a temperature of 101 degrees
Fahrenheit, a blood pressure of 100/64 mm Hg, a pulse of 100 bpm, and an oxygen
saturation of 90% on 6 liters of O2. She appears to be uncomfortable. Her neck veins
are flat. There are scattered rales throughout both lung fields. The precordium is
hyperdynamic with a soft systolic murmur and an S3 gallop. Her extremities are warm
without edema.

A transthoracic echocardiogram (TTE) demonstrates hyperdynamic left venticular (LV)

systolic function, a LV ejection fraction of 75%, and elevated gradients across the mitral
prosthesis.

Which of the following is the next best step in in the management of this patient?
A. Computed tomography pulmonary angiogram.
B. Cardiac catheterization.
C. Cardiac magnetic resonance imaging.
D. Cinefluoroscopy.
E. Transesophageal echocardiography.

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This patient appears to have prosthetic valve endocarditis, possibly with a paravalvular
leak that is causing heart failure.

In patients with bioprosthetic valves who show evidence of prosthetic valve

regurgitation, TTE is used to monitor the appearance of the valve leaflets, valve
hemodynamics, LV size and systolic function, and to estimate pulmonary pressures.
The initial approach is TTE for evaluation of antegrade valve velocities and pressure
gradients. However, transesophageal echocardiography (TEE) is essential for the
evaluation of suspected or known prosthetic mitral valve regurgitation. On TTE imaging,
the left atrium is shadowed by the valve prosthesis, obscuring evidence of prosthetic
regurgitation. TEE imaging provides clear images of the left atrial side of the mitral
prosthesis and is particularly useful for delineation of the site and severity of
paravalvular regurgitation, evaluation of suitability for a percutaneous approach, and
guidance during percutaneous closure procedures.

Thus, TEE is the correct approach in this patient.

Cardiac catheterization and computed tomography pulmonary angiography would not

offer additional insight into the cause of this patient's symptoms, management, or
prosthetic valve regurgitation. While magnetic resonance imaging can be useful for
quantifying mitral regurgitation or paravalvular leaks, it would not be the first choice in
this unstable patient. Notably, cinefluoroscopy is not indicated as she has a
bioprosthetic valve. Answer E
Key Point
Doppler echocardiography (transthoracic echocardiography and transesophageal
echocardiography) is the method of choice for the diagnosis of prosthetic valve
dysfunction (stenosis and regurgitation).

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Question 55
55- You are asked to see a 59-year-old female with aortic stenosis (AS). For the past 3
months she has noted progressive shortness of breath with daily household chores. Her
medical history is significant for hypertension. She currently takes losartan 50 mg and
aspirin 81 mg daily.

Her vital signs are a blood pressure of 124/72 mm Hg with a heart rate of 84 bpm. Her
jugular veins are not distended. Carotid upstrokes are delayed. Her lungs are clear to
auscultation. The apical impulse is sustained and displaced 2 cm to the left of the mid-
clavicular line. A grade 3/6 harsh, late-peaking systolic ejection murmur is heard
throughout the precordium, loudest at the right upper sternal border, and radiates to the
carotids. There is no diastolic murmur. The rest of her physical examination is normal.

Her laboratory studies include a hemoglobin of 13.1 g/dl and a creatinine of 0.8 mg/dl.
Her echocardiogram demonstrates concentric left ventricular hypertrophy with an
ejection fraction of 65%. There is a bicuspid aortic valve with AS; peak velocity is 4.2
m/sec with a mean gradient of 42 mm Hg and a calculated valve area of 0.7 cm 2.
Coronary angiography reveals a 60% angiographic stenosis of the mid left anterior
descending (LAD) artery; fractional flow reserve (FFR) was 0.89. Her estimated
operative mortality for cardiac surgery is calculated at 1.6%.

Which of the following is the next best step in the management of this patient?
A. Surgical aortic valve replacement plus coronary artery bypass grafting.
B. Transcatheter aortic valve replacement plus percutaneous coronary intervention.
C. Surgical aortic valve replacement.
D. Transcatheter aortic valve replacement.

The patient has symptomatic, severe AS and nonobstructive coronary artery disease
involving her LAD artery based on an FFR of >0.8. Her surgical risk is low (<4%). The
most appropriate therapy is surgical aortic valve replacement (AVR; Class I) (Figure 1).
Transcather AVR is a Class I indication for treating AS in patients at prohibitive surgical
risk as well as high surgical risk (Society of Thoracic Surgeons Predicted Risk of
Mortality score >8%), depending on patient values and preferences. Transcatheter AVR
is not currently recommended for the treatment of patients with AS and low surgical risk.
She needs medical therapy for her coronary disease. Answer C

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Question 56
56- A 63-year-old male with history of ST-segment elevation myocardial infarction (MI)
of the right coronary artery presents to the cardiology clinic with progressive exertional
dyspnea. He denies any chest discomfort with activity. He has not been seen for 18
months. His medications include aspirin 81 mg daily, metoprolol tartrate 25 mg twice a
day, and lisinopril 2.5 mg daily. On exam, his blood pressure is 122/78 mm Hg, heart
rate is 95 beats per minute, respiratory rate is 18 breaths/min, and oxygen saturation is
93% while breathing ambient air. His examination reveals a III/VI blowing holosystolic
murmur loudest at the apex and radiating to the axilla. He has trace peripheral edema.
His electrocardiogram shows sinus rhythm, inferior Q waves, and a left bundle branch
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block (LBBB) with a QRS duration of 143 ms. Echocardiography reveals severe inferior
and inferolateral left ventricular (LV) hypokinesis, a LV ejection fraction of 34%, and
severe mitral regurgitation (MR).

Which of the following is the next best step in the management of this patient?
A. Optimization of medical therapy.
B. Cardiac resynchronization therapy.
C. Mitral valve annuloplasty with repair.
D. Mitral clip placement.
E. Mitral valve replacement.

MR may be primary (due to intrinsic valve dysfunction) or secondary (due to adverse LV

remodeling). This patient presents with chronic symptomatic heart failure (HF) in the
setting of a prior MI, likely ischemic cardiomyopathy, and most likely secondary MR due
to papillary muscle displacement with posterior mitral valve (MV) leaflet tethering. For
patients with secondary MR, treatment is initially aimed at improving ventricular function
and volume status.

This patient is on metoprolol tartrate rather than metoprolol succinate and is on a low-
dose of an angiotensin-converting enzyme inhibitor, so there is still opportunity to
optimize guideline-directed medical therapy (GDMT) for HF. If he had persistent LV
systolic dysfunction and HF symptoms after optimization of GDMT, then cardiac
resynchronization therapy may be considered given his LBBB and prolonged QRS
duration. For secondary MR, MV replacement and repair (Class IIb) were shown to
have similar mortality and LV remodeling outcomes in a randomized trial, but MV
replacement was associated with a lower rate of recurrent moderate or severe MR and
lower incidence of both HF and repeat hospitalization. Both surgical groups had a high
mortality rate at 2 years, emphasizing the poor prognosis with secondary MR.

Neither of the surgical MV treatments nor transcatheter MV repair would be a first-line

therapy for this patient who is receiving suboptimal medical management. Answer A
Key Point
Causes of mitral regurgitation may be broadly differentiated as primary (predominantly
degenerative) or secondary (functional), a classification that has significant impact on
prognosis and management.

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Question 57
57- A 74-year-old female with a history of hypertension and moderate aortic stenosis
(AS) presents to your outpatient clinic for a routine follow-up evaluation. She reports
worsening fatigue and dyspnea, but no chest pain, palpitations, presyncope, or
syncope. Her blood pressure is 112/80 mm Hg and heart rate is 66 bpm. Her last
echocardiogram, 1 year prior, demonstrated normal biventricular function, mild mitral
regurgitation, and aortic peak velocity 3.1 m/sec with a calculated aortic valve area 1.3
cm2.

What physical examination finding would suggest progression of this patient's aortic
stenosis?
A. Increased opening snap-S2 interval.
B. Ejection click.
C. Widened splitting of S2.
D. Enhanced A2.
E. Late-peaking murmur.

Aortic valve stenosis is associated with reduced leaflet mobility and delayed closure.
Auscultatory findings in severe AS include a soft, single S2 sound since the
A2 component of the second heart sound, which is due to aortic valve closure, is
delayed and tends to occur simultaneously with the pulmonic component (P 2), which is
due to pulmonic valve closure. The S2 may become paradoxically split when the
stenosis is severe and associated with left venricular dysfunction. With increasingly
severe, fixed AS, the A2 closing sound may disappear. The presence of a normally split
S2 is the most reliable finding to exclude severe AS in adults.

The first heart sound (S1) is usually normal. However, an aortic ejection click, which is
more commonly heard with a congenital bicuspid valve, may be heard after S 1 early in
AS when the leaflets are stiff but still somewhat compliant and mobile. Vigorous left
atrial contraction against a stiff, noncompliant ventricle can produce an S4.

The murmur associated with AS is described as a systolic "ejection" murmur, typically

heard best at the base of the heart in the right intercostal space, with a harsh quality.
The murmur may be heard at the apex especially in elderly patients with radiation of the
high frequency sounds (Gallavardin phenomenon). The murmur generally begins after
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S1 and ends before S2. The intensity of the murmur reflects the amount and velocity of
blood flow across the valve and the turbulence produced by the stenosis. A loud
murmur (grade 4 or greater) has a high specificity for severe AS. However, most
patients with severe stenosis have a grade 3 murmur, and many have only a grade 1 or
2 murmur. In patients with low flow, low gradient AS the murmur may be soft and almost
inaudible.

The timing of the murmur also correlates with the severity of the stenosis, similar to the
timing of the carotid pulse. An early-peaking murmur is typical for mild to moderate AS,
while a late-peaking murmur is consistent with severe AS.

The opening snap is a finding of mitral stenosis and not pertinent to AS. Answer E
Key Point
As the murmur of aortic stenosis (AS) worsens, the ejection sound and the intensity of
A2 diminish; the murmur peaks later in systole. The AS murmur increases after a
premature ventricular contraction.

Question 58
58- A 45-year-old female with mitral valve prolapse (MVP) for many years presents to
your clinic for re-evaluation. She feels well. She teaches a spinning class at her gym.
She takes no medications.

On exam her blood pressure is 100/60 mm Hg and pulse is 62 bpm. She has a mid
systolic click followed by a loud murmur radiating to the axilla.

Her echocardiogram demonstrates a left ventricular end systolic diameter (LVESD) of

4.1 cm and left ventricular ejection fraction (LVEF) of 60%. There is posterior MVP
resulting in severe mitral regurgitation (MR) with a calculated effective regurgitant orifice
area of 0.45 cm2 and a regurgitant volume of 70 ml.

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Which of the following is the best strategy for managing this patient's mitral valve
disease?
A. Bioprosthetic valve replacement.
B. Mechanical valve replacement.
C. Surgical valve repair.
D. Watchful waiting.
E. Transcatheter valve repair.

Mitral valve (MV) surgery is recommended for asymptomatic patients with chronic
severe primary MR and left ventricular (LV) dysfunction (LVEF of 30-60% and/or
LVESD of 40 mm, stage C2).

The goal of therapy in MR is to correct it before the onset of LV systolic dysfunction and
the subsequent adverse effect on patient outcomes. Ideally, MV surgery should be
performed when the patient's LV approaches, but has not yet reached the parameters
that indicate systolic dysfunction (LVEF of 60% or LVESD of 40 mm). Because
symptoms do not always coincide with LV dysfunction, imaging surveillance is used to
plan surgery before severe dysfunction has occurred. If moderate LV dysfunction is
already present, prognosis is reduced following MV operation. Thus, further delay (even
though symptoms are absent) will lead to greater LV dysfunction and a still worse
prognosis. Because the loading conditions in MR allow continued late ejection into a
lower-impedance left atrium, a higher cutoff for “normal” LVEF is used in MR than in
other types of heart disease. Although it is clearly inadvisable to allow patients' LV
function to deteriorate beyond the benchmarks of an LVEF of 60% and/or LVESD of 40
mm, some recovery of LV function can still occur even if these thresholds have been
crossed.

MV repair is recommended in preference to MV replacement when surgical treatment is

indicated for patients with chronic severe primary MR involving the anterior leaflet or
both leaflets when a successful and durable repair can be accomplished, thus MV repair
is the preferred choice in this patient.

Transcatheter MV repair may be considered for severely symptomatic patients (New

York Heart Association [NYHA] class III-IV) with chronic severe primary MR (stage D)
who have favorable anatomy for the repair procedure and a reasonable life expectancy,
but who have a prohibitive surgical risk because of severe comorbidities and remain
severely symptomatic despite optimal guideline directed medical therapy (GDMT) for
heart failure (HF; Level of Evidence B). A randomized controlled trial of percutaneous
MV repair using the MitraClip device versus surgical MV repair was conducted in the
United States. The clip was found to be safe, but less effective than surgical repair
because residual MR was more prevalent in the percutaneous group. However, the clip
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reduced the severity of MR, improved symptoms, and led to reverse LV remodeling.
Percutaneous MV repair should only be considered for patients with chronic primary MR
who remain severely symptomatic with NYHA class III-IV HF symptoms despite optimal
GDMT for HF and who are considered inoperable.

Degenerative MV disease consisting of more than posterior leaflet disease requires a

more complex and extensive repair. When the anterior leaflet or both leaflets require
repair, durability of the repair is less certain, with a freedom from reoperation of
approximately 80% and a freedom from recurrent moderate or severe MR of 60% at 15-
20 years. These results are superior to the results of MV replacement, even in elderly
patients. Repair should also be attempted if possible with other causes of severe MR,
such as papillary muscle rupture, infective endocarditis, and cleft MV. As the repair
becomes more complex however, results of very complex repair in younger patients
may be matched by results of durable mechanical MV replacement with careful
management of anticoagulation. Answer C
Key Point
For primary mitral regurgitation, surgical mitral valve (MV) repair is recommended over
MV replacement because of preserved left ventricular (LV) function, lower operative
mortality rate, and lower rate of complications associated with prosthetic valves in the
long term.

Question 59
59- A 65-year-old male calls your office with questions regarding his mitral valve
prosthesis (MVP). Two years ago, the patient underwent surgery with placement of a 31
mm porcine MVP for symptomatic, severe regurgitation due to myxomatous disease. He
has done well since surgery, with no symptoms or complications in follow-up. His last
echocardiogram, which was performed 2 months ago, demonstrated a mean transmitral
gradient of 2 mm Hg and trivial mitral regurgitation.

For which of the following procedures should he take antibiotics as prophylaxis against
infective endocarditis?
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A. Fat pad biopsy.

B. Esophagogastroduodenoscopy.
C. Cystoscopy with urethral dilation.
D. Dental cleaning.
E. Colonoscopy with biopsy.

Antibiotic prophylaxis against endocarditis is indicated for those patients undergoing

dental procedures involving manipulation of gingival tissues who are at highest risk of
complications. These high-risk patients include those with a prosthetic cardiac valve or
prosthetic valve repair material, a prior history of infective endocarditis, cardiac
transplant with valvulopathy, completely repaired congenital heart disease (CHD) with
percutaneous or surgical repair occurring within the previous 6 months, repaired CHD
with residual shunts or defects that impair endothelialization of prosthetic material, and
unrepaired cyanotic CHD.

Antibiotic prophylaxis is required in these patients who are undergoing dental

procedures that involve manipulation of the gingival tissues, the periapical region of the
teeth, or perforation of oral mucosa. There are no prospective studies to suggest benefit
from antibiotic prophylaxis in nondental procedures. Answer D .

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Congenital Heart Disease

Question 1 of 30

A 54-year-old man with a history of tetralogy of Fallot (TOF) status initially palliated with a Blalock-
Taussig shunt as an infant followed by transannular patch repair at 10 years of age presents for
evaluation. He reports a syncopal episode 2 weeks prior. He was sitting in a

chair and awoke on the floor. There were no witnesses and he estimates he was out for a few minutes.
He denies recent fevers, chills, or sweats. He walks 30 minutes daily for exercise. His daily medications
include aspirin 81 mg and metoprolol succinate 25 mg. On examination his temperature is 98.4 degrees
Fahrenheit, heart rate is 64 bpm, blood pressure is 118/70 mm Hg, and oxygen saturation is 98% on
room air. His jugular venous pulse is 6 cm H20, his lungs are clear, and heart sounds are regular with a
widely split S2, a soft 2/6 systolic ejection murmur, and a I/IV diastolic murmur at the right upper sternal
border. His abdomen is soft and nontender without organomegaly and his extremities are warm without
edema.

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His electrocardiogram is shown (Figure 1). His echocardiogram shows left ventricular (LV) dysfunction
with an ejection fraction of 42%, mild right ventricular (RV) dilatation and dysfunction, a bioprosthetic
valve in the pulmonary position with a mean transvalvular gradient of 10 mm Hg, and mild pulmonary
regurgitation. The estimated RV systolic pressure is 28 mm Hg. A Holter monitor shows frequent
premature ventricular contractions and short runs of nonsustained ventricular tachycardia (NSVT).

Reproduced with permission from ttps://thephysiologist.org/2016/07/31/tetralogy-of-fallot/.

What is the next best step in the care of this patient?

A. Implantable loop recorder placement.

B. Implantable cardioverter-defibrillator placement.

C. Amiodarone.

D. A tilt table test.

E. A transesophageal echocardiogram.
TOF is a conotruncal anomaly resulting from anterior and leftward deviation of the infundibular
septum. It is characterized by varying degrees of RV outflow tract obstruction, a ventricular septal
defect, overriding aorta, and RV hypertrophy. The majority of adults with TOF will have undergone
repair in childhood. In the late 1950s and 1960s, patients with TOF often underwent palliative
procedures such as a systemic-to-pulmonary artery shunt (i.e., the Blalock-Taussig shunt) to
augment pulmonary blood flow prior to definitive repair. Since the 1980s, primary repair without initial
palliation is the standard of care. In adults with repaired TOF, prevalence rates for atrial (20%) and
ventricular (15%) arrhythmias have noted to have steep increases after 45 years of age. The
incidence of sudden cardiac death (SCD) after surgical repair of TOF is approximately 2% per
decade.

Implantable cardioverter-defibrillator (ICD) therapy is reasonable in selected adults with TOF and
multiple risk factors for SCD, such as LV systolic or diastolic dysfunction, NSVT, QRS duration ≥180
msec, extensive RV scarring, or inducible sustained ventricular tachycardia at electrophysiologic
study. In this patient with syncope consistent with an arrhythmic cause prior palliative shunt, late
complete repair, NSVT on Holter, and right bundle branch block >180 msec, ICD is the best choice.

Amiodarone is not indicated to reduce the risk of SCD, but could be considered if the patient had a
high burden of ventricular arrhythmias after ICD placement. An implantable loop recorder to assess
for ventricular arrhythmias is not indicated as this study would not change management. A tilt table
test would not be useful as the patient's history is not consistent with neurocardiogenic syncope.
While further imaging may be useful to quantify RV size and function, quantify PR, and identify RV
outflow tract aneurysms, the best imaging test for this would be cardiac magnetic resonance
imaging, not transesophageal echocardiogram.

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Answer : B
Key Point
A resting QRS duration of >180 msec is associated with ventricular arrhythmias in patients with
repaired tetralogy of Fallot. Left ventricular dysfunction and prior arrhythmia events are also
associated with sudden death in this population.

Question 2 of 30

A healthy 18-year-old woman presents for an athletic screening prior to starting soccer practice with her
college team. She reports no known medical issues and takes no medications.

On examination her heart rate is 52 bpm and blood pressure is 108/70 mm Hg. An ejection click is noted
at the left second intercostal space. The click occurs in early systole and the intensity does not vary with
inspiration or maneuvers.

Which of the following is the most likely diagnosis?

A. Secundum atrial septal defect.

B. Mitral stenosis.

C. Bicuspid aortic valve.

D. Mitral valve prolapse.

E. Hypertrophic cardiomyopathy.

Systolic ejection clicks occur early in systole and result from the opening of stiff, but still mobile aortic
valve leaflets most commonly seen in bicuspid aortic valve. Aortic ejection clicks are most commonly
heard at the left second interspace and the apex. They do not vary with inspiration. Mitral valve
prolapse generally produces a mid-systolic click heard best in the left lower sternal border. It is heard
later in systole than an aortic click and produces a higher frequency sound. Flow across the atrial septal
defect itself does not result in a murmur; it is related to the augmented flow through the right heart
which produces a pulmonary flow murmur. Hypertrophic cardiomyopathy, when obstructive, causes a
harsh systolic crescendo-decrescendo murmur heard best at the left lower sternal border radiating to
the apex. Maneuvers that enhance the degree of obstruction (Valsalva, squat-to-stand maneuver) will
increase the intensity of the murmur. Mitral stenosis typically causes a diastolic opening snap followed
by a diastolic rumble. It is heard best closest to the left lower sternal border in the left lateral decubitus
position.

Answer : C
Key Point
Patients with unexplained right heart enlargement but no atrial septal defect seen on transthoracic
echocardiography should undergo alternative imaging (cardiac magnetic resonance, computed
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tomography, or transesophageal echocardiography) to find other causes of pretricuspid shunt such

as partial anomalous pulmonary venous return, sinus venosus defect, or unroofed coronary sinus,

Question 3 of 30

A 42-year-old woman presents with complaints of progressive shortness of breath. She recently
immigrated to the United States and has not previously seen a cardiologist. She has three children, the
youngest of which was born 8 years ago. She reports progressive fatigue and dyspnea over the past 6
months and over the last 2 weeks has noted the onset of peripheral edema. On review of systems she
notes dizziness and near-syncope. On physical examination her vital signs are a blood pressure of
108/78 mm Hg, a heart rate of 95 bpm, and 92% oxygen saturation by finger oximetry. Cardiac
auscultation is notable for a loud P2 component and a diastolic murmur heard best in the left upper
sternal border. Cyanosis and clubbing are apparent in the toes but not the fingers. A chest X-ray shows
cardiomegaly and prominence of the proximal pulmonary arteries. A 12-lead electrocardiogram shows
normal sinus rhythm, biatrial enlargement, and biventricular hypertrophy.

Which of the following is the most likely cause of this patient's symptoms?

A. Secundum atrial septal defect.

B. Patent ductus arteriosus.

C. Ventricular septal defect.

D. Congenitally corrected transposition of the great arteries.

E. Tetralogy of Fallot.

The patient in this vignette has Eisenmenger syndrome as the result of an unrepaired patent ductus
arteriosus (PDA). Clues to the presence of Eisenmenger syndrome in this vignette include features
of pulmonary hypertension with a loud P2 sound on cardiac auscultation, radiographic prominence of
the pulmonary arteries, and electrocardiographic evidence of right ventricular hypertrophy. Initially a
PDA will produce a continuous "machinery" type murmur, however with the onset of shunt reversal
this murmur may no longer be evident - as is the case in this patient. The finding of differential
cyanosis (cyanosis and clubbing of the toes but not the fingers) is pathognomonic for PDA-level
shunting. This is due to the fact that the right-to-left shunt at the ductus is distal to the subclavian
arteries. Eisenmenger syndrome may result from any initial left-to-right shunt evolving over time to
produce pulmonary vascular remodeling and pulmonary arterial hypertension, which then reverses
the shunt to a right-to-left physiology. Examples include but are not limited to atrial septal defects,
ventricular septal defects, and PDA.

Congenitally corrected transposition of the great arteries if it presents in adulthood would not result
in shunting in the absence of other defects. Tetralogy of Fallot presenting in adulthood would result
in cyanosis/Eisenmenger syndrome if not repaired, but not differential cyanosis.

Answer : B

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Key Point
Patients with Eisenmenger physiology due to a patent ductus arteriosus may present with pink
fingers on both hands, but blue and clubbed toes due to reversal of the shunt toward the lower
extremities

Question 4 of 30

A 52-year-old woman with tetralogy of Fallot presents for routine follow-up. She had surgical repair of
her pulmonary valve at the age of 4 with a transannular patch and has had few medical issues since
then. She denies dyspnea on exertion or palpitations. On examination her blood pressure is 161/90 mm
Hg, heart rate is 69 bpm, and oxygen saturation rate is 99 % on room air.

Cardiac auscultation is significant for a widely split S2 with respiratory variation, an early diastolic
murmur at the left upper and lower sternal borders, and a soft systolic murmur at the left lower sternal
border. Her jugular venous pressure is estimated at 8 cm H20. There is no lower extremity edema. Her
electrocardiogram shows normal sinus rhythm with a right bundle branch block of 165 msec.

Cardiac magnetic resonance imaging performed today is significant for a right ventricular (RV) end-
diastolic volume of 130 ml/m2 with a RV ejection fraction of 55%, left ventricular (LV) ejection fraction
of 55%, and mild LV hypertrophy with an RV:LV diastolic volume ratio of 1.5:1. Echocardiographic
assessment of the pulmonary valve is shown (Figure 1; Video 1).

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Video 1(echo colordoppler of short axis L parasternal view showed sever PR)

Which of the following is the most appropriate next step in the management of this patient?

A. Percutaneous pulmonary valve replacement.

B. Implantable cardioverter-defibrillator placement.

C. Antihypertensive therapy.

D. A fasting lipid panel.

E. Coronary artery computed tomography.

This patient has significant pulmonary regurgitation on echocardiogram, but does not have any other
criteria to suggest that pulmonary valve replacement is indicated. Specifically, RV volumes do not meet
the 2018 Adult Congenital Heart Disease (ACHD) guideline recommendations for replacement.
Moreover, data for native outflow tract percutaneous pulmonary valve replacement is limited. Definitive
data for primary prevention implantable cardioverter-defibrillator indications are limited. Risk factors
for sudden cardiac death include LV systolic or diastolic dysfunction, a QRS duration above 180 msec,
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and nonsustained or inducible ventricular tachycardia. Management of atherosclerotic cardiovascular

disease risk factors are critical in ACHD patients and should be included in the overall treatment of
congenital heart disease patients.

Answer : C

Question 5 of 30

A 49-year-old asymptomatic man who had an extensive yearly executive physical is referred to you for
evaluation. The patient had been doing well with no cardiovascular symptoms. He exercises regularly
and has normal lipids. As part of his workup he underwent an exercise stress test which was ischemic so
he was referred for exercise SPECT which suggests inferior ischemia. At cardiac catheterization there
were no intracoronary lesions, but the right coronary origin was in the left cusp. Intravascular
ultrasound was normal as was a chest wall echocardiogram and resting ECG. You order a coronary
computed tomography angiography that shows an interarterial course of the right coronary artery from
the left sinus.

Based on the information you have in this asymptomatic patient, what is the best next step?

A. Stenting of the right coronary artery orifice.

B. Repeat stress test after the initiation of beta-blockers.

C. Add a statin.

D. Follow-up in 6 months.

E. Surgical intervention.

The correct answer is to proceed with surgical intervention. The current guideline suggests that any time
an anomalous right coronary from the opposite sinus or a left coronary from the opposite sinus travels
between the aorta and the pulmonary artery, and there is evidence for ischemia, it is a Class I indication
for intervention regardless of symptoms. Figure 1 outlines the current guideline recommendations. Any
left coronary from the opposite sinus of Valsalva should be operated upon if there is ischemia (Class I) or
no ischemia (Class IIa) with an interarterial course. Any right coronary from the opposite sinus should be
operated upon if there is ischemia (Class I) or evidence for ventricular arrhythmias (Class IIa). If neither
are present, then the clinical scenario is to be considered and either surgery (Class IIb) or watchful
observation (Class IIb) is acceptable.

Answer : E

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Key Point
The most important coronary anomaly of potential clinical significance is anomalous coronary artery
(RCA) from the opposite sinus of Valsalva. It is rare, being observed in 0.1% of cardiac
catheterizations. This anomaly may be the RCA from the left sinus of Valsalva or the left main or left
anterior descending (LAD) artery from the right sinus of Valsalva. Studies of sudden death in college
athletes have reported that 4-14% of events are attributable to an anomalous coronary artery from
the opposite sinus of Valsalva. The mechanism of sudden death seems to be related to decreased
blood flow, leading to myocardial ischemia from an abnormal slit-like orifice of the anomalous
coronary ostium. Surgical unroofing is recommended in cases of the left main or LAD from the right
sinus of Valsalva with or without documented ischemia or the RCA from the left sinus of Valsalva
with symptoms or documented ischemia. Computed tomography angiography (CTA) is the best test
to demonstrate the anatomy and magnetic resonance imaging (MRI) can be used as well. The role
of stress testing in coronary anomalies is controversial.

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Question 6 of 30

A 52-year-old man presents to urgent care with indigestion and chest heaviness. He is mildly obese with
a history of hypertension. However, his blood pressure has been well-controlled for many years. His
father died of a heart attack at the age of 78. His mother is alive and healthy at age 92. His last
cholesterol panel included low-density lipoprotein 124 of mg/dl, high-density lipoprotein 39 mg/dl, and
triglycerides of 199 mg/dl. His only medication is lisinopril 20 mg daily. His electrocardiogram shows
nonspecific ST changes. His troponin T is 0.08 ng/dl. Due to ongoing chest discomfort, coronary
angiography is performed.

Video

Coronary angiography in this patient reveals which of the following?

A. Anomalous left coronary artery from the right sinus.

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B. Diffuse microvascular dysfunction.

C. Diagonal artery myocardial bridging.

D. Left circumflex coronary artery occlusion.

E. Dual left anterior descending coronary artery.

This angiogram shows an anomalous left coronary artery with an origin from the right sinus. The
angiogram reveals an injection of a left coronary artery with an additional vessel originating adjacent to
the origin of the left main. This is the right coronary artery as identified by its course and posterior
descending artery branch. Computed tomography angiography is recommended to delineate that the
anatomic course of this is interarterial, as was the case for this patient. Since an interarterial course was
identified, it is associated with increased risk for sudden cardiac death. In general the left coronary from
the right sinus appears to be a more significant risk for sudden death and ischemia than the right, and in
most cases it should undergo repair. If it travels in the anterior aortic media it can often be unroofed.

Answer : A
Key Point
The most important coronary anomaly of potential clinical significance is anomalous coronary artery
(RCA) from the opposite sinus of Valsalva. It is rare, being observed in 0.1% of cardiac
catheterizations. This anomaly may be the RCA from the left sinus of Valsalva or the left main or left
anterior descending (LAD) artery from the right sinus of Valsalva. Studies of sudden death in college
athletes have reported that 4-14% of events are attributable to an anomalous coronary artery from
the opposite sinus of Valsalva. The mechanism of sudden death seems to be related to decreased
blood flow, leading to myocardial ischemia from an abnormal slit-like orifice of the anomalous
coronary ostium. Surgical unroofing is recommended in cases of the left main or LAD from the right
sinus of Valsalva with or without documented ischemia or the RCA from the left sinus of Valsalva
with symptoms or documented ischemia. Computed tomography angiography (CTA) is the best test
to demonstrate the anatomy and magnetic resonance imaging (MRI) can be used as well. The role
of stress testing in coronary anomalies is controversial.

Question 7 of 30

You are seeing a 34-year-old man for follow-up of a ventricular septal defect. He has mild headaches
most days and has leg cramps that are worse at night. He occasionally experiences blurry spots in his
vision. He denies chest pain or shortness of breath. His current medications include amoxicillin 2,000 mg
prior to dental work. His heart rate is 90 bpm, blood pressure is 102/62 mm Hg, and oxygen saturation is
93% on room air. His physical examination reveals a thin adult man. Mucous membranes are moist. His
cardiac examination reveals a 3/6 holosystolic murmur. There is mild clubbing of the fingers. His
hematocrit is 63%, hemoglobin is 21 g/dl, and platelet count is 101,000. His echocardiogram shows a
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large ventricular septal defect with bidirectional flow. Both ventricles are dilated with low-normal
systolic function. The tricuspid regurgitant velocity is 4 m/s. The inferior vena cava is 2.1 cm in diameter,
but does not collapse with inspiration.

Which of the following is the next best step in the care of this patient?

A. Phlebotomy.

B. Aspirin.

C. Uric acid.

D. Iron studies.

E. Hydroxyurea.

This patient has Eisenmenger's syndrome. This syndrome is associated with secondary erythrocytosis,
thromboembolic events, cerebrovascular complications (stroke and brain abscesses), hyper viscosity
syndrome, hypertrophic osteoarthropathy, and renal dysfunction. Treatment consists of supplemental
oxygen therapy (if it increases arterial oxygen saturation), pulmonary vasodilator therapy, and iron
supplementation when iron deficiency is present. Iron studies will allow you to diagnose and calculate
the iron deficit for the appropriate replacement if indicated.

For many years, cerebrovascular complications were presumed to be due to erythrocytosis and
phlebotomy was performed to maintain normal or near-normal hematocrit levels. However, a large
study failed to demonstrate a relationship between hematocrit and stroke risk in cyanotic patients.
Atrial fibrillation, systemic hypertension, and microcytosis, which can be worsened by phlebotomy and
iron deficiency, are the strongest predictors of stroke in Eisenmenger patients. Phlebotomy, along with
volume resuscitation and iron supplementation, may have a role in treating acute, severe symptoms of
hyperviscosity, but is not indicated in this patient with mild symptoms (headaches, leg cramps, scotoma)
that may be due to iron deficiency rather than hyperviscosity.

Hydroxyurea is used to suppress myeloproliferative activity (i.e., overproduction of all cell lines) in
patients with polycythemia vera. In contradistinction to polycythemia patients, cyanotic patients have
high levels of erythrocytes in proportion to their hypoxemia, but often have low platelet levels.

Suppression of bone marrow activity would not be appropriate in patients with secondary
erythropoiesis. Eisenmenger syndrome is associated with both thrombocytopenia and thromasthenia as
well as abnormal coagulation parameters, so that patients are at risk for both thrombotic events and
bleeding events. Systemic anticoagulation or antiplatelet therapies are generally reserved for specific
indications (clinically apparent thrombosis, atrial fibrillation, the presence of artificial heart valves, or
conduits).

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Uric acid levels are often elevated in Eisenmenger patients and are associated with worse outcomes.

Answer: D
Key Point
Patients with Eisenmenger physiology due to a patent ductus arteriosus may present with pink
fingers on both hands, but blue and clubbed toes due to reversal of the shunt toward the lower
extremities.

Question 8 of 30

A 43-year-old woman is referred to your clinic for the recent diagnosis of an atrial septal defect (ASD).
She describes shortness of breath and leg swelling. Her blood pressure is 108/72 mm Hg. An
echocardiogram reveals a secundum ASD.

Which of the following findings would be a contraindication to ASD closure in this patient?

A. Right ventricular dilatation.

B. A pulmonary artery systolic pressure of 80 mm Hg.

C. A Qp:Qs ratio of 1.6.

D. Bidirectional shunting.

E. Severe tricuspid regurgitation.

ASD surgical or device closure is a Class I indication (Figure 1) in symptomatic patients with a left-to-right
shunt, pulmonary vascular resistance <1/3 of systemic vascular resistance, pulmonary arterial systolic
pressure (PASP) of <50% systemic, and a large shunt (Qp:Qs >1.5). A pulmonary systolic pressure of 80
mm Hg with a systolic blood pressure of 108 mm Hg is >2/3 systemic and is a contraindication of closure.
It can still be considered in patients with higher PASP, bidirectional shunt (net equal or left-to-right),
and/or pulmonary vascular resistance in consultation with adult congenital and pulmonary hypertension
experts (Class IIb). However, a net right-to-left shunt (e.g., Eisenmenger syndrome) would be a
contraindication to closure as these patients have prohibitively high peri-operative morbidity and
mortality. Right ventricular dilatation is a consequence of a significant ASD not a contraindication to ASD
closure. Resultant significant tricuspid regurgitation can be seen and sometimes improves after ASD
closure; it is not a contraindication to the procedure.

Answer : B

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Key Point
Mild pulmonary hypertension (pulmonary vascular resistance [PVR] <1/3 systemic vascular
resistance) is not a contraindication to closure. Patients with intermediate levels of pulmonary
hypertension should be carefully evaluated before closure of an atrial septal defect is attempted, and
medical therapy should ideally be initiated to lower PVR.

Question 9 of 30

A 19-year-old man presents for evaluation of a systolic murmur noted on a sports physical. He is a
Division I golfer without exertional symptoms. He reports occasional palpitations, but denies syncope or
presyncope. He has no family history of cardiac disease. His echocardiogram is shown (Video 1).

Echo

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Which of the following is the most likely location of the defect seen?

A. Perimembranous.

B. Inlet/atrioventricular canal.

C. Supracristal/outlet.

D. Left ventricle-right atrium (Gerbode).

E. Muscular.

This patient has a muscular ventricular septal defect (VSD), a common type of VSD. It is most easily seen
in the short-axis views with color-flow Doppler (11 o'clock in the current echocardiogram). The
components of the ventricular septal wall are shown in Figure 1, as well as in the corresponding
echocardiogram views. The most common types of VSDs are shown in Figure 2. Initial evaluation should
include assessment of left ventricular (LV) size and function as VSD flow typically causes volume loading
of the left-sided structures. LV volume overload and hemodynamically significant shunts (Qp:Qs =1.5:1)
should undergo VSD closure if pulmonary arterial systolic pressure is <50% systemic and pulmonary
vascular resistance is <1/3 systemic. Additionally evaluation for location within the muscular septum and
proximity to valvular structures is helpful for determination as most muscular defects can be closed
percutaneously.

Answer : E

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Question 10 of 30

A 66-year-old woman is seen in the office with shortness of breath that has been ongoing for about a
year; the condition worsens when she is standing and improves with lying down. Her past medical
history is notable for hypertension that is well controlled on amlodipine. On examination, she has
marked kyphoscoliosis. Her resting oxygen saturation while breathing ambient air is 84% while seated
and 93% when supine. A transthoracic echocardiogram reveals aneurysmal dilation of the ascending
aorta with compression of the right atrium.

Which of the following is the next best step in the management of this patient?

A. An agitated saline (bubble) study.

B. Cardiac magnetic resonance imaging.

C. Right heart catheterization.

D. A cardiopulmonary exercise stress test.

E. Pulmonary embolism protocol chest computed tomography.

This patient has findings of platypnea-orthodeoxia syndrome (POS), characterized by dyspnea and
hypoxemia in the upright position that resolve when lying supine. Potential etiologies of POS include
intracardiac shunt caused by patent foramen ovale (PFO), atrial septal defect, or atrial septal aneurysm
with fenestration as well as pulmonary conditions with ventilation-perfusion mismatch such as
interstitial lung disease, hepatopulmonary syndrome, and pulmonary arteriovenous malformations. The
most commonly described condition associated with POS is PFO. In order for a PFO to cause POS there
must usually be an associated condition that causes distortion of the interatrial septum while in the
upright position. Such conditions include post-pneumonectomy or lung transplantation, right
hemidiaphragm paralysis, kyphoscoliosis, and ascending aortic dilation.

An agitated saline (bubble) study is the next best step to evaluate for a right-to-left shunt and should
ideally be performed in both the supine and upright positions in a patient with suspected POS. While
cardiac magnetic resonance imaging or transesophageal echocardiogram could provide additional
anatomic information, an agitated saline echocardiogram is the most cost-effective, non-invasive
diagnostic study.

Although chest computed tomography and right heart catheterization may demonstrate evidence of
intracardiac shunting or help to quantify the degree of shunt, these are not the next best steps. A
cardiopulmonary exercise stress test is not needed to make the diagnosis of POS or to establish the
etiology. In patients with POS due to interatrial shunt without pulmonary hypertension, closure of this
defect provides relief of symptoms.

Answer : A
Key Point
Patent foramen ovale occurs in approximately 25% of the population, does not result in left-to-right
shunt-related complications, and is only rarely associated with any pathologic sequelae (stroke,
migraine, platypnea-orthodeoxia, or provoked exercise desaturation).
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Question 11 of 30

A 24-year-old male medical student has an echocardiogram during a practice session to acquaint
students with the technology. The student has no prior medical history, takes no medications, and has
no symptoms. The technician notices an enlarged right ventricle and measures it to be 36 mm at the
base and 40 mm at the mid-cavity level. No atrial septal defect is noted on the transthoracic
echocardiogram. The echocardiography attending confirms the findings and measures the right
ventricular systolic pressure at 50 mm Hg. Agitated saline is administered and shows no interatrial
shunting.

Which of the following is the next best step in the management of this student's care?

A. Repeat echocardiogram in one year.

B. An exercise stress test.

C. Right heart catheterization.

D. Cardiac magnetic resonance imaging.

E. V/Q scan.

Although a bubble study shows no evidence of shunting, this does not exclude all types of atrial level
shunting or partial anomalous pulmonary venous return. Given his enlarged right ventricle, further
investigation to exclude these is warranted. The best next step would be a cardiac magnetic resonance
imaging (MRI) study and therefore a repeat echocardiogram in 1 year is not appropriate. Cardiac MRI
would provide both physiologic and anatomic information including a Qp:Qs to guide further
management. The definitive anatomy and diagnosis should be defined before proceeding with right
heart catheterization. A V/Q scan would be indicated for the workup of pulmonary hypertension if a
shunt lesion is not identified. An exercise stress test is not indicated in this asymptomatic individual for
the evaluation of right heart enlargement.

Answer : D
Key Point
Partial anomalous pulmonary venous return is almost always present in patients with sinus venosus
atrial septal defect.

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Question 12 of 30

A 21-year-old woman presents to your clinic for the management of hypertension. She is on two
antihypertensive medications, but without adequate blood pressure control. She reports mild fatigue
with exercise and lower extremity cramping with exercise. Otherwise she feels well. Her physical
examination is significant for a height of 4'11", weight of 110 lbs, blood pressure of 141/89 mm Hg, and
a heart rate of 61 bpm. Her pulse examination is consistent with a radiofemoral delay and right upper
extremity lymphedema.

Her echocardiographic assessment is shown (Figure 1; Video 1).

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Video 1

Based on the clinical history provided, which genetic syndrome does this patient most likely have?

A. Noonan syndrome.

B. Holt-Oram syndrome.

C. DiGeorge syndrome.

D. William syndrome.

E. Turner syndrome.

Turner syndrome is characterized by the (X,O) genotype. Women have short stature, a webbed neck,
lymphedema, and premature ovarian failure. Congenital heart disease occurs in up to 50% of patients
with left-sided obstructive lesions being the most common. Bicuspid aortic valve and coarctation are the
most common issues. The images show that the patient has evidence of coarctation, a diagnosis that is
most commonly made on echocardiogram with diastolic forward flow in the abdominal aorta. While
coarctation can be seen in other genetic syndromes, coarctation is most common in Turner syndrome.

Noonan syndrome patients often have pulmonary stenosis while Holt-Oram patients exhibit with atrial
septal defects, DiGeorge patients with tetralogy of Fallot, and William syndrome patients with supra
aortic stenosis.

Answer : E
Key Point
Turner syndrome is associated with bicuspid aortic valve, aortic coarctation, and ascending aortic
aneurysm; it is a risk factor for aortic dissection.

Question 13 of 30

A 26-year-old man is referred to your office for the evaluation of hypertension. He feels well, but admits
to leading a sedentary lifestyle. He was recently started on amlodipine 5 mg daily. He has never smoked
cigarettes and drinks 3-4 beers on the weekends. On examination, his heart rate is 78 bpm, blood
pressure is 158/100 mm Hg on the right upper extremity, pulse oximetry is 99% on room air, and body
mass index is 29 kg/m2. His cardiac examination reveals a systolic ejection click and an early peaking 1/6
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systolic murmur at the base. His lungs are clear to auscultation and his abdomen is soft with no
hepatomegaly. There is no edema or clubbing. An electrocardiogram shows normal sinus rhythm with
left ventricular hypertrophy. An echocardiogram is performed (Video 1).

Video 1

Which of the following tests will confirm the cause of this patient's hypertension?

A. Renal artery duplex ultrasound.

B. Plasma renin activity.

C. A chest computed tomography angiogram.

D. Urine toxicology.

E. Thyroid stimulating hormone level.

The echocardiogram shown demonstrates a bicuspid aortic valve (BAV) with fusion of the right and left
coronary cusps. A BAV can be associated with aortopathies and coarctation of the aorta. Untreated
coarctation can cause secondary hypertension, left ventricular hypertrophy, and significantly increases
the risk of aortic dissection. Coarctation can be diagnosed with transthoracic echocardiogram in the
suprasternal and subcostal window with two-dimensional and Doppler imaging. Alternatively,
coarctation can be well visualized on chest computed tomography angiography.

Although the other tests can identify other secondary causes of hypertension, the BAV seen in this
patient should prompt evaluation of the aorta. Additionally, physical examination assessment should
include radiofemoral delay assessment, upper and lower extremity blood pressures, as well as
auscultation for murmurs of both the anterior and posterior chest. Renal artery duplex can be used to
diagnose renal artery stenosis or fibromuscular dysplasia. Plasma renin activity will be low in patients
with hyperaldosteronism. Urine toxicology for illicit drugs including cocaine, marijuana, and
amphetamines that can all cause hypertension. Typically hyperthyroidism, but also hypothyroidism, can
lead to hypertension.

Answer : c
Key Point
Large atrial septal defects lead to right heart enlargement, atrial arrhythmias, and right heart failure if
unrepaired.

Question 14 of 30
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You are asked to see a 22-year-old college student who has had a known murmur since infancy. She has
been active, but did not play any sports because her local physician felt the murmur was too loud. She
currently is feeling well and has no limitations to her activity. Her only medications are birth control pills.
Her examination reveals a healthy appearing young woman with no cyanosis or clubbing. Her vital signs
are normal including saturations. Her examination reveals no jugular venous pressure or evidence of an
elevated right heart pressure. Her lungs are clear. She has a loud grade 4/6 holosystolic murmur at the
third intercostal space along the left sternal border with an accompanying thrill. There is no gallop.
There are no diastolic murmurs. The remainder of the examination is normal.

Her electrocardiogram (ECG) and chest X-ray are normal.

You obtain an echocardiogram (Video 1). All chamber sizes are normal as is the estimated right
ventricular systolic pressure.

Video 1

Which of the following additional studies would you recommend for this patient at today's visit?

A. Cardiac magnetic resonance imaging.

B. Cardiac catheterization.

C. A cardiopulmonary stress test.

D. Cardiac computed tomography

E. No additional studies.

The correct answer is no additional studies. This patient should have a clinical follow-up in 2 years. At
that time, echocardiography and ECG should be repeated. Echocardiogram remains the imaging
modality of choice to assess changes in left ventricular size and function. Given the fact that patient is
asymptomatic, exercise testing would not be indicated. The echocardiogram shows a muscular
ventricular septal defect (VSD) and color-flow Doppler suggests it is restrictive. The murmur is very loud
suggesting a high gradient across the muscular VSD. She is asymptomatic and meets no requirement for
intervention for this muscular VSD at this time. The current guideline defines physiologic stages (Figure
1). She would be anatomic class I, physiologic stage B as defined by her trivial or small shunt.

The current guideline suggests that small VSDs without significant shunt can be reassessed every 24
months with ECG and echocardiography. Further studies and potential intervention would be warranted
if she demonstrates an increase in physiologic stage as defined (Figures 1a, b). Indications for closure
include the following: 1) any symptoms, 2) evidence for left heart enlargement from a volume overload
(in adults this means evidence of at least a 1.5:1 shunt and enlarged left ventricle and left atrium), 3)
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evidence for pulmonary hypertension (as long as the pulmonary arterial systolic pressure is <50%
systemic and the pulmonary vascular resistance is <1/3 systemic vascular resistance), or 4) endocarditis
(Figure 2).

While further imaging or hemodynamic characterization of her small VSD might be considered useful,
she has no indication of any concern regarding surgical or percutaneous device intervention, and she
can be followed with serial echocardiography and examinations. Were the VSD to be high in the septum
(doubly committed or "supracristal"), then it is possible that aortic regurgitation may ensue from right
aortic cusp prolapse and, if severe, this would represent another indication for further studies and
potential intervention.

Answer : E

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Question 15 of 30

A 48-year-old woman with a prior history of a transient ischemic attack (TIA) presents to the emergency
department with 1 hour of left-sided weakness and decreased sensation as well as a left-sided visual
field defect. Her current daily medications include aspirin 81 mg and atorvastatin 40 mg. She is a
nonsmoker. Her prior work-up included a negative hypercoagulable evaluation, echocardiogram, and a
30-day electrocardiogram monitoring.

The stroke team is activated. Her head and neck computed tomography angiogram (CTA) shows an
acute right middle cerebral artery occlusion without evidence of other atherosclerotic disease. There is

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no hemorrhage. She is taken emergently for intracerebral thrombectomy with improvement in

symptoms.

The echocardiogram performed at the time of the previous TIA is shown (Video 1).

Video 1

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Which of the following is the next best step in the management of this patient?

A. Agitated saline injection.

B. Carotid duplex ultrasound.

C. Inferior vena cava filter.

D. Oral anticoagulation.

E. Percutaneous closure.

The patient presented with an acute embolic stroke. The echocardiogram shows a shunt across the atrial
septum most suggestive of a patent foramen ovale ([PFO] or possibly a small atrial septal defect). This
can be a cause of an ischemic stroke due to paradoxical emboli from the venous system traversing the
PFO and entering the cerebral arterial circulation. Given that this is her second event (prior history of a
TIA and on aspirin) and she had an extensive negative work-up for other causes of stroke, this is a
cryptogenic PFO mediated event; therefore she should be referred for percutaneous closure of the PFO.

The shunt is clearly seen on color flow Doppler imaging, therefore an agitated saline study is not
necessary. Ruling out carotid disease is important in the evaluation of an ischemic stroke, however this
patient had a neck CTA on presentation and therefore a carotid duplex ultrasound is redundant. An
inferior vena cava filter can be used for treatment of lower extremity deep vein thrombosis when
anticoagulation is contraindicated, however it is not indicated for the long-term prevention of recurrent
strokes in patients with a PFO. There is no clear-cut benefit of anticoagulation over aspirin in patients
with a cryptogenic stroke in the absence of atrial fibrillation.

Answer : E
Key Point
Patent foramen ovale (PFO) is more common in patients with cryptogenic stroke. In patients with
cryptogenic stroke, randomized trials suggest that PFO closure, when added to medical therapy,
reduces the risk of subsequent stroke.

Question 16 of 30

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A 24-year-old asymptomatic female (X,O) presents to your office to establish care. She last saw a
pediatric cardiologist when she was 16 years old. Her vital signs are blood pressure of 108/70 mm Hg
and heart rate 70 bpm. Her echocardiogram is shown (Figure 1; Videos 1 and 2).

Video 1

What is the next best step in the management of this patient?

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A. Aortic magnetic resonance angiography.

B. A transesophageal echocardiogram.

C. Computed tomography angiography of the head.

D. No further diagnostic testing.

E. A Holter monitor.

Turner syndrome is the most common chromosomal abnormality affecting females (occurring in 1 in
2,500 live births). Congenital heart abnormalities occur in up to 50% of affected individuals, the most
common abnormality being a bicuspid aortic valve (BAV; prevalence estimated at 15-30%). Aortic
magnetic resonance angiography or computed tomography (CT) angiography is indicated in patients
with a BAV when the ascending aorta cannot be fully assessed and/or if the transthoracic
echocardiogram (TTE) cannot exclude aortic coarctation (as is the case with this patient's TTE). TTE can
be helpful to further evaluate the aortic valve, but is not adequate to evaluate the dimensions of the
transverse and initial descending aortic arch. CT angiography of head may be appropriate if the finding
of coarctation is established. There is not a higher prevalence of dysrhythmias in Turner's syndrome
making a Holter monitor an incorrect test.

Answer :A
Key Point
Initial and follow-up aortic imaging using cardiac magnetic resonance imaging or computed
tomography angiography is recommended in adults with coarctation of the aorta including those who
had surgical or catheter intervention.

Question 17 of 30

A 36-year-old man is referred to you for the evaluation of hypertension. He was diagnosed by his
internist 6 months prior and despite compliance with chlorthalidone 50 mg daily, amlodipine 10 mg
daily, lisinopril 40 mg daily, and carvedilol 25 mg twice daily, his blood pressure remains elevated. He
has no symptoms and walks briskly for 30 minutes 5 days a week without limitations. He takes no other
medications or supplements. On examination his heart rate is 58 bpm and blood pressure is 150/90 mm
Hg in both arms. His jugular venous pulse is 6 cm H20 and his lungs are clear. Heart sounds are regular
with an ejection systolic click and a systolic ejection murmur at the left sternal border.

An echocardiogram is obtained. There is a bicuspid aortic valve (BAV) with a mean transaortic gradient
of 10 mm Hg and a calculated aortic valve area of 2.2 cm2. Peak descending aortic velocity is 3.8 m/sec.
There is diastolic forward flow documented in the abdominal aorta.

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What is the next best step in this ptient's care?

A. Aortic balloon angioplasty.

B. Renal artery ultrasound.

C. Repeat echocardiogram in 1 year.

D. Clonidine 0.1 mg twice daily.

E. Aortic stent placement.

This patient has coarctation of the aorta (CoA) which most commonly involves a discrete narrowing of
the descending aorta at the insertion of the ductus arteriosus. This classically presents with refractory
hypertension in patients with a BAV. Other findings on physical examination would include systolic
hypertension in the upper extremities, diminished or delayed femoral pulses (brachial-femoral delay),
and low or unobtainable arterial blood pressure in the lower extremities. In addition, the systolic
murmur can extend beyond the second heart sound, at the left paravertebral interscapular area, due to
flow across the narrow coarctation area. Typical echocardiographic findings include accelerated flow
across the aortic isthmus with an elevated peak velocity and diastolic forward flow in the abdominal
aorta.

Corrective intervention (i.e., surgery or transcatheter intervention) should be performed in patients with
CoA with any of the following: a CoA gradient >20 mm Hg, radiologic evidence of clinically significant
collateral flow, systemic hypertension attributable to CoA, or heart failure attributable to CoA.

Cardiac magnetic resonance imaging or computed tomography angiography is often required to

precisely define the location of the CoA and guide the choice of intervention, transcatheter versus
surgical. Of the two transcatheter approaches, aortic stent placement is the preferred option because
balloon angioplasty alone is associated with a higher rate of intimal tears and aneurysm formation
compared with stent placement. As CoA intervention is warranted based on the presence of an elevated
gradient and hypertension, rechecking an echocardiogram in 1 year or just increasing antihypertensive
therapy are not the best answers. Given the patient's physical examination findings of BAV and
echocardiogram findings of coarctation, additional testing for secondary causes of secondary
hypertension (i.e., renal artery ultrasound) is not warranted.

Answer : E
Key Point
Classic examination findings of coarctation of the aorta are systemic hypertension, brachial-femoral
pulse delay, and decreased pulsatility with blunting of the abdominal aortic Doppler pattern. A
gradient of 20 mm Hg across the lesion is considered significant.

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Question 18 of 30

First-degree relatives of patients with which of the following conditions should be screened by
transthoracic echocardiography?

A. Ostium secundum atrial septal defect.

B. Mitral valve prolapse with severe mitral regurgitation.

C. Bicuspid aortic valve.

D. Tetralogy of Fallot.

E. Anomalous left coronary artery from the pulmonary artery.

Per the 2014 American College of Cardiology/American Heart Association (ACC/AHA) Valvular Heart
Disease Guideline, 20% to 30% of patients with a bicuspid valves have other family members with
bicuspid aortic valve (BAV) or aortopathy. Patterns of inheritance are variable, so it is important to take
a family history and inform patients that other family members may be affected. Imaging of first-degree
relatives is clearly appropriate if the patient has an associated aortopathy or a family history of valvular
heart disease or aortopathy. Many valve experts also recommend screening all first-degree relatives of
patients with BAV, although there are not yet data addressing the possible impact of screening on
outcomes or the cost-effectiveness of this approach. The 2010 ACC/AHA Guideline on Thoracic Aortic
Disease give a Class I recommendation (Level of Evidence C) for the evaluation of first-degree relatives
of patients with a BAV, premature onset of thoracic aortic disease with minimal risk factors, and/or a
familial form of thoracic aortic aneurysm and dissection for the presence of a BAV and asymptomatic
thoracic aortic disease. According to the 2018 ACC/AHA Guideline for Adults With Congenital Heart
Disease, because of the possibility of familial occurrence, a careful family history should be taken in
patients with atrial septal defect (ASD), and parents and offspring should be evaluated clinically for
possible septal defect, conduction disturbances, and skeletal anomalies. However, there is no
recommendation for the use of echocardiography for routine screening of first-degree relatives of
patients with ASD, mitral valve prolapse, Tetralogy of Fallot, or anomalous left coronary artery.

Answer : c
Key Point
Patent foramen ovale occurs in approximately 25% of the population, does not result in left-to-right

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shunt-related complications, and is only rarely associated with any pathologic sequelae (stroke,
migraine, platypnea-orthodeoxia, or provoked exercise desaturation).

Question 19 of 30

A 34-year-old man presents to your office for evaluation. He is referred by his internist for evaluation of
a murmur. He has no chest pain, shortness of breath, or palpitations. He takes no medications.

On examination he has a heart rate of 64 bpm and blood pressure of 106/60 mm Hg. His jugular venous
pulse is 6 cm H2O. There is a palpable thrill at the fourth left intercostal space. His heart is regular with a
normally split S2. There is a loud, high-frequency holosystolic murmur at the left lower sternal border.
The murmur does not change with Valsalva. His lungs are clear to auscultation and his extremities are
warm without edema.

What is this patient's most likely diagnosis?

A. Mitral valve prolapse.

B. Bicuspid aortic valve.

C. Hypertrophic cardiomyopathy.

D. Pulmonic stenosis.

E. Ventricular septal defect.

This patient has a small ventricular septal defect (VSD). A VSD should be suspected in a patient who
presents with a holosystolic murmur. There are generally no associated symptoms in patients with small
VSDs, while patients with moderate or large VSDs develop symptoms such as dyspnea and fatigue. The
smaller the VSD the louder the associated murmur, often accompanied by a palpable thrill. For large
VSDs with increased right ventricular (RV) and pulmonary artery pressure the murmur is instead in early
systole and physical findings of pulmonary arterial hypertension and RV hypertrophy are present. When
ventricular pressures are equal as in Eisenmenger syndrome there is no murmur across the VSD, instead
there is a mid-systolic murmur due to dilation of the pulmonary trunk or a holosystolic murmur related
to tricuspid valve regurgitation, and S2 is markedly accentuated and single.

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Mitral valve prolapse is a late systolic murmur preceded by single or multiple clicks. Valsalva decreases
left ventricular volume and will cause an earlier onset of the click and murmur.

A bicuspid aortic valve will result in a mid-systolic murmur, often with an ejection sound at the onset of
the murmur. The systolic murmur originating from left ventricular outflow obstruction (LVOTO) from
hypertrophic cardiomyopathy can be harsh, simulating a VSD murmur, but an LV outflow murmur is
typically an ejection type and can also be distinguished from a VSD murmur by dynamic auscultation
maneuvers. As this murmur does not increase with Valsalva, it is not consistent with a LVOTO murmur.

A wide splitting of S2 with reduced intensity of P2 is present in pulmonary stenosis; as S2 is normal here,
this favors a VSD. It would be important to evaluate for evidence of a double chamber right ventricle on
echocardiogram simultaneous with the assessment of the VSD.

Answer: E
Key Point
Though large ventricular septal defects (VSDs) have quieter murmurs than small VSDs, they can
result in ventricular volume overload and pulmonary hypertension; and remain the most common
cause of Eisenmenger syndrome.

Question 20 of 30

A 19-year-old woman presents to the emergency department. She has noticed several minutes of
palpitations over the last several weeks. Today she felt lightheaded and dizzy with the palpitations. She
denies syncope. She takes no medications. She drinks two or three cups of coffee daily. On examination
her blood pressure is 105/70 mm Hg and heart rate is 63 bpm. Her lungs are clear. Her cardiac
examination reveals a normal jugular venous pressure, but a subtle CV wave is noted. On auscultation
there is a loud click heard after S1 and a very soft systolic murmur. No gallops are evident. There is no
edema and the remainder of the examination is normal.

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An electrocardiogram (ECG) is obtained (Figure 1).

Which of the following is this patient's most likely diagnosis?

A. Ebstein anomaly.

B. Bicuspid aortic valve.

C. Mitral valve prolapse.

D. Pulmonary valve stenosis.

E. Subaortic membrane.

The correct answer is Ebstein anomaly. The ECG reveals classic right-sided (Type B) Wolff-Parkinson-
White syndrome (WPW). Twenty percent of patients with Ebstein anomaly exhibit variable degrees of
pre-excitation as is shown on the ECG (seen best in lead avL). A third of Ebstein anomaly patients with
WPW have multiple pathways and, should atrial fibrillation (AF) ensue and the antegrade pathway has a
short effective refractory period, then ventricular tachycardia and ventricular fibrillation may occur,
especially if the shortest R-R interval in AF is <220 msec. Some patients only have retrograde pathways,
but are susceptible to a reciprocating tachycardia.

Ebstein anomaly results in apical displacement of the posterior and/or septal tricuspid valve leaflets, but
not the anterior leaflet. The echocardiographic diagnosis requires a leaflet origin displacement into the
right ventricle (RV; relative to the mitral annulus) of >20 mm or >8 mm/m2. The loud click heard on
auscultation is related to the "sail sound" emanating from the large anterior tricuspid valve leaflet as it
billows backward in systole. About half of patients with Ebstein anomaly have either an atrial septal

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defect or a patent foramen ovale, and evaluation of exercise oxygen saturations is recommended. The
tricuspid valve displacement results in an atrialized RV. The more severe the displacement the less
functional the RV. Tricuspid regurgitation (TR) of varying degrees is usually evident. Magnetic resonance
imaging (MRI) is important to assess the size of the residual RV. An MRI on this patient (Video 1) reveals
mild Ebstein anatomy with mild TR. An Ebstein-like valve may also be seen in congenitally corrected
transposition of the great arteries and pre-excitation in this situation may appear to be a left-sided
WPW pathway on the surface ECG. This patient needs an electrophysiology evaluation and ablation of
the accessory pathway.

The other answers are incorrect, as none are associated with WPW. The systolic click heard could be
heard in pulmonary stenosis, a bicuspid aortic valve, or mitral prolapse, but none are associated with
WPW. A subaortic membrane would present with a crescendo-decrescendo systolic murmur and is also
not associated with WPW.

Answer : A
Key Point
Ebstein anomaly is a rare congenital heart defect and the age at presentation is related to the
severity of the disease, with more severe disease presenting earlier in life. Greater than or equal to
20% of Ebstein patients have evidence for Wolff-Parkinson-White syndrome. An examination,
electrocardiogram, and chest X-ray may provide clues to the diagnosis. The echocardiogram is more
definitive and requires the apical displacement of the septal leaflet >20 mm (>8 mm/m 2), and the
presence of a redundant elongated anterior tricuspid valve leaflet.

Question 21 of 30

A 46-year-old woman is seen in clinic due to a family history of bicuspid aortic valve (BAV). She denies
any cardiovascular symptoms and runs on the treadmill three times a week. She is on no medications.
Her father had a BAV and underwent an aortic valve replacement at the age of 64. Her blood pressure is
110/64 mm Hg and her heart rate is 72 bpm. Her cardiac examination reveals no murmurs. An
echocardiogram shows normal left ventricular systolic function. There is a BAV without stenosis or
regurgitation. Her ascending aorta measures 4.2 cm.

Which of the following would be the next best step in the management of this patient?

A. Losartan 25 mg daily.

B. Metoprolol 12.5 mg twice daily.

C. No additional therapy.

D. Amlodipine 5 mg daily.

E. Disopyramide 200 mg twice daily.

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The patient has a BAV without any valvular dysfunction, but there is a mildly dilated ascending aorta. Per
the American College Cardiology valvular heart disease guideline, there are no proven drug therapies
that have been shown to reduce the rate of progression of aortic dilation in patients with aortopathy
associated with BAV. In patients with hypertension, control of blood pressure with any effective
antihypertensive medication is warranted. Beta-blockers and angiotensin-receptor blockers have
conceptual advantages to reduce the rate of progression, but have not been shown to be beneficial in
clinical studies. As this patient does not meet the criteria for treatment of hypertension, there would be
no indication to start an empiric antihypertensive medications.

Answer :c

Question 22 of 30

An obese 35-year-old man is seen in the office for chronic, progressive dyspnea during physical exertion
over the past 2 years. He also mentions occasional lightheadedness precipitated by physical exertion. His
medical history is otherwise notable for hypertension which is well controlled with lisinopril 10 mg daily.
He takes no other medications. On examination his heart rate is 84 bpm, blood pressure is 92/60 mm
Hg, and jugular venous pressure is elevated at 10 cm H20. Cardiac auscultation reveals a fixed split S2
with a prominent pulmonic component. His lungs are clear bilaterally. There is 1+ bilateral pedal edema.

An echocardiogram is performed and reveals normal left ventricular (LV) size and function. The right
ventricle (RV) is moderately dilated with reduced systolic function. Estimated pulmonary artery systolic
pressure is 54 mm Hg.

Which of the following is the most appropriate next step in the management of this patient?

A. Right heart catherization.

B. Cardiac magnetic resonance imaging.

C. Overnight oximetry.

D. Pulmonary function testing.

E. V/Q scan.

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The patient described has normal LV function, but evidence of RV dysfunction with elevated pulmonary
pressures. The best next step is an anatomic assessment given the high likelihood of intracardiac
shunting due to the fixed split S2 on physical examination. Cardiac magnetic resonance imaging would
afford both the anatomic and physiologic assessment necessary for both diagnosis and management
decisions. Atrial septal defects (ASD) are a relatively common congenital heart defect, which can be
asymptomatic until adulthood. Early in life there is left-to-right shunting (from the left atrium to the
right atrium) placing a volume load on the right-sided cardiac structures and pulmonary circulation. This
is typically well-tolerated for many years, but may eventually result in symptoms, pulmonary
hypertension, RV failure, and Eisenmenger syndrome.

Right heart catherization is indicated for assessment of the hemodynamic consequences, but after
defining the anatomy. If there is an ASD along with partial anomalous pulmonary venous return, then
surgical repair would be indicated; however if only a central secundum ASD was found, percutaneous
closure would be considered. The diagnosis of ASD can be made using noninvasive tests (transthoracic
echocardiography, transesophageal echocardiography, cardiac magnetic resonance imaging, or
computed tomography).

Given the physical examination suggestive of an underlying ASD, pulmonary function tests, overnight
oximetry, and V/Q scan are not first-line assessments as they would be for an undifferentiated
pulmonary hypertension workup.

Answer : B
Key Point
Severe pulmonary arterial hypertension (pulmonary artery systolic pressure >2/3 systemic pressure
or pulmonary vascular resistance >2/3 systemic vascular resistance) is a contraindication to atrial
septal defect closure.

Question 23 of 30

A 60-year-old woman is referred to you for atypical chest pain. The pain occurs both at rest and with
exercise and is located in the mid-sternum. It can at times be elicited by pressure on the sternum. She
has family history of coronary artery disease, is hypertensive, and has a low-density lipoprotein of 149
mg/dl. Her daily medications include metoprolol succinate 50 mg, amlodipine 10 mg, atorvastatin 40
mg, chlorthalidone 25 mg, and potassium chloride 20 mEq. She has had several emergency department
visits for recurrent chest pain with normal troponins. A coronary computed tomography angiography
was then ordered (Figure 1).

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What is the next best step in her care?

A. An exercise stress echocardiogram.

B. Implantable loop recorder placement.

C. Surgical referral.

D. The addition of isosorbide mononitrate.

E. Percutaneous coronary intervention.

The correct answer is ischemic evaluation. In the evaluation of patients with anomalous coronaries the
first step is to evaluate for ischemia. Subsequently, arrhythmia monitoring (implantable loop recorder,
holter/event monitor) may be considered for evaluation of ventricular arrhythmias. Anomalous
coronaries that travel between the pulmonary artery and aorta (interarterial) are at the highest risk. The
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anatomy shown (anomalous right from the left sinus) has an interarterial course. In an autopsy series of
athletes and military recruits, there was an over representation of anomalous left coronary arteries from
the right sinus suggesting a higher risk of sudden cardiac death. In general, anomalous right coronary
arteries are considered lower risk in the absence of ischemia.

Figure 2 outlines how these should be handled. The left from the opposite sinus should undergo surgical
intervention whether there is ischemia (Class I) or not (Class IIa). The right from the opposite sinus
should undergo surgical intervention if there is ischemia (Class I) or ventricular arrhythmias (Class IIa).
Otherwise one can choose to observe (Class IIb) or intervene (Class IIb) depending on the clinical
situation.

Answer : A
Key Point
The most important coronary anomaly of potential clinical significance is anomalous coronary artery
(RCA) from the opposite sinus of Valsalva. It is rare, being observed in 0.1% of cardiac
catheterizations. This anomaly may be the RCA from the left sinus of Valsalva or the left main or left
anterior descending (LAD) artery from the right sinus of Valsalva. Studies of sudden death in college
athletes have reported that 4-14% of events are attributable to an anomalous coronary artery from
the opposite sinus of Valsalva. The mechanism of sudden death seems to be related to decreased
blood flow, leading to myocardial ischemia from an abnormal slit-like orifice of the anomalous
coronary ostium. Surgical unroofing is recommended in cases of the left main or LAD from the right
sinus of Valsalva with or without documented ischemia or the RCA from the left sinus of Valsalva
with symptoms or documented ischemia. Computed tomography angiography (CTA) is the best test
to demonstrate the anatomy and magnetic resonance imaging (MRI) can be used as well. The role
of stress testing in coronary anomalies is controversial.

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Question 24 of 30

A 37-year-old man who has a history of coarctation of the aorta that was surgically repaired as a child
presents to establish care. He does not have a bicuspid aortic valve. He feels well. He plays soccer
regularly without exertional symptoms. You review the computed tomography (CT) imaging of his aorta
and a transthoracic echocardiogram that was performed 4 years ago. A minimal gradient was reported
across the coarctation from his last echocardiogram. He takes no medications.

On examination his blood pressure on the left is 112/78 mm Hg and it is 108/74 mm Hg on the right. He
has robust pulses in both feet and right leg blood pressure is 122/84 mm Hg. There is no radiofemoral
delay. There is a soft bruit heard anteriorly beneath his left clavicle.

What is the best next step in his management?

A. Cardiac magnetic resonance imaging or CT angiography within the next year.

B. Cardiac magnetic resonance imaging or CT angiography in 3 years.

C. Cardiac echocardiogram in 3 years.

D. No further imaging.

E. Cardiac magnetic resonance imaging or CT in 5 years.

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The correct answer is cardiac magnetic resonance imaging (MRI) or CT angiography of the aorta within
the next year. Patients with coarctation need to be monitored continuously after intervention for
evidence of aneurysmal formation or re-coarctation. The current guideline (Figure 1) suggests that in the
asymptomatic patient this should be done at least every 36-60 months with either MRI or CT
angiography of the aorta. An accompanying video reveals a typical re-coarctation image by MRI (Video
1). Eleven percent of repaired coarctation patients will develop re-coarctation or aneurysm formation.
Aneurysms are not well seen by echocardiography and aortic cross-sectional imaging should be done.

Restenosis clinically can be suggested by an increase in upper body blood pressure or evidence of a
gradient across the coarctation by echocardiogram. In younger individuals, the lower body systolic blood
pressure is higher than the upper body blood pressure due to reflected waves from the periphery.
Clinically, restenosis of the coarctation should be considered if the upper to lower extremity blood
pressure difference is >20 mm Hg, there is significant radiofemoral delay, and/or claudication. Of note,
significant collateral flow can decrease the sensitivity of echocardiographically assessed gradient due to
decreased blood flow through the native aorta.

Answer : A

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Question 25 of 30

A 23-year-old woman with Marfan syndrome (MFS) presents for preconception counseling.

On examination her height is 5' 8" and weight is 115 lbs. Her temperature is 98.4 degrees Fahrenheit,
heart rate is 70 bpm, and blood pressure is 100/60 mm Hg. Her lungs are clear, heart sounds are regular
with a mid-systolic click and II/VI systolic murmur, the abdomen is soft, and extremities are warm
without edema.

Her echocardiogram showed an ejection fraction of 65%, mitral valve prolapse with moderate mitral
regurgitation, and an aortic root diameter of 3.6 cm. On magnetic resonance imaging, the aortic root
dimension was 3.7 cm and the rest of the aorta had a normal dimension.

With shared decision making, the patient elects to proceed with pregnancy.

During pregnancy, for which of the following complications is the patient at greatest risk?

A. Fetal complete heart block.

B. Mitral valve chordal rupture.

C. Pulmonary embolism.

D. Aortic dissection.

E. Fetal Ebstein anomaly.

Aortic dissection is a rare, but often catastrophic event and occurs in patients with disorders of
connective tissue, including those caused by fibrillin mutations such as MFS. Pregnancy is associated
with a substantially increased risk of aortic dissection, probably caused by a maternal increase in blood
volume, heart rate, and stroke volume, and by hormonally-mediated changes in the diseased aortic wall.
The expected rate of aortic dissection may reach approximately 3% on average, ranging from 1% in
women with an aortic diameter <40 mm to as much as 10% in high-risk patients (those with an aortic
root diameter >40 mm, rapid dilation, or previous dissection of the ascending aorta). Despite the rare
occurrence of aortic dissection in women with MFS and a normal-sized aorta, an event-free pregnancy
cannot be guaranteed in these women. In patients with aortic dimensions >40 mm, it is reasonable to
consider prophylactically replacing the aorta prior to pregnancy. In most women, aortic dissection
occurs during the third trimester or postpartum, but it may occur at any time of gestation. According to
the World Health Organization criteria, pregnancy is contraindicated in MFS patients with a dilated aorta
>45 mm.

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While patients with MFS may have mitral valve prolapse or chordal rupture, severe mitral regurgitation
is not commonly observed in pregnancy. Ebstein anomaly can be a teratogenic effect of maternal lithium
ingestion. The congenital heart block associated with neonatal lupus is considered a form of passively
acquired autoimmune disease in which maternal autoantibodies to the intracellular ribonucleoproteins
Ro (SS-A) and La (SS-B), cross the placenta and injure the previously normal fetal heart. While pulmonary
embolism can occur during and after pregnancy, it is not associated with MFS.

Answer : D
Key Point
Pregnancy is a risk factor for aortic dissection in females with aortopathy. Evaluation of the aorta is
necessary prior to pregnancy to determine risk. Aortic root diameter >40 mm is associated with an
even greater risk of dissection in Marfan syndrome.

Question 26 of 30

A patient with Trisomy 21 is noted to have a murmur and is referred for an echocardiogram (Videos 1
and 2).

Video 1

Video 2

Which of the following is the correct diagnosis?

A. Secundum atrial septal defect.

B. Atrioventricular canal defect.

C. Ebstein anomaly.

D. Truncus arteriosus.

E. Tetralogy of Fallot.

The correct answer is atrioventricular (AV) canal defect. There is a strong association between Trisomy
21 and AV canal defects. The echocardiogram shows that the AV valves are at the same levels and there
is shunting across a primum atrial septal defect (ASD) and a small inlet ventricular septal defect (VSD).
Secundum ASD is due to an isolated defect in the fossa ovalis and the echocardiogram shows a defect in
the ventricular septum as well as the atrial septum. Ebstein anomaly results from abnormally developed
and positioned tricuspid valve leaflets with a downward displacement of the tricuspid valve and an

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extremely large right atrium. It is frequently associated with an atrial communication and often presents
with cyanosis. Tetralogy of Fallot (TOF) is characterized by VSD, overriding aorta, right ventricular
hypertrophy, and outlet obstruction. Though TOF is associated with Trisomy 21, AV canal defects are
found much more commonly. Truncus arteriosus is the incomplete division of the aortic and pulmonary
vessels and can be seen more frequently in patients with DiGeorge syndrome.

Answer : B
Key Point
There are “classic” phenotypes that have become identified with certain congenital lesions. Among
them are Down syndrome (atrioventricular septal defects), Holt-Oram syndrome (silundum atrial
septal defects), Noonan syndrome (dysplastic pulmonary valve), and Marfan syndrome (mitral valve
prolapse, aortic regurgitation, ascending aortic aneurysm, and dilated pulmonary arteries).

Question 27 of 30

A 44-year-old woman is evaluated for progressive dyspnea on exertion over the past year. She has been
previously told she has a murmur. The remainder of her medical history is unremarkable. Her father
underwent aortic valve replacement (AVR) with aortic root repair when he was 50 years old. She is a
non-smoker.

Her physical examination is notable for an S1 and soft S2 with a 3/6 crescendo-decrescendo systolic
ejection murmur heard at the right upper sternal border. The murmur decreases in intensity with
Valsalva and increases in intensity with passive leg raise. There is a separate soft diastolic murmur at the
left upper sternal border without radiation.

An echocardiogram reveals a dilated, mildly hypertrophic left ventricle with an estimated ejection
fraction of 50%. There is a bicuspid aortic valve (BAV) with moderate calcification of the leaflets. The
mean aortic valve (AV) gradient is 50 mm Hg. There is moderate aortic regurgitation. The ascending
aortic diameter is 47 mm.

Which of the following is the most appropriate treatment recommendation for this patient at this time?

A. Transcatheter AVR.

B. AVR with ascending aorta replacement.

C. Surgical AVR.

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D. AV repair.

E. Septal myomectomy.

This patient has a congenital bicuspid aortic valve (BAV), as evidenced on her echocardiogram and by
her family history. She has developed severe aortic stenosis in her 5 th decade of life, far earlier than seen
with calcific aortic stenosis. She is symptomatic with a mean AV gradient in the severe range. AVR is
indicated at this time. However, she also has a dilated ascending aorta of 47 mm. According to the 2014
Valvular Heart Disease guideline, it is a class IIa indication to consider replacement of the aorta for
patients with congenital BAV who are undergoing AVR for either aortic stenosis or aortic regurgitation if
the diameter of the ascending aorta is >4.5 cm in diameter. This represents a lower diameter threshold
than for patients without BAVs. Therefore, the correct treatment recommendation is AVR with
ascending aorta replacement.

AV repair is not appropriate for this calcified stenotic BAV. A septal myomectomy is appropriate for a
patient with hypertrophic obstructive cardiomyopathy (HOCM), but this patient's murmur and
echocardiogram findings are consistent with aortic stenosis not HOCM. Transcatheter AVR would not be
appropriate given the need for ascending aortic intervention.

Answer :B
Key Point
Surgery to resect the aortic root and/or ascending aorta for bicuspid aortic valve disease is
recommended when the aneurysm diameter ≥5.5 cm. Surgery may be considered when the aorta
reaches 5.0 cm if there are risk factors for aortic dissection such as a family history of dissection,
rapid aortic growth (3-5 mm/year), aortic coarctation, or small stature, or if surgery can be performed
at a low risk (<4%) by a surgeon with expertise at a center with experience.

Question 28 of 30

What other cardiovascular abnormality is associated with the finding seen in Video 1?

Video

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A. Partial anomalous pulmonary venous return.

B. Apical displacement of the tricuspid valve.

C. Right-sided aortic arch.

D. Ventricular septal defect.

E. Atrioventricular accessory pathway.

A sinus venosus defect typically results from the absence of the dividing wall between the vena cava as
it enters the right atrium and the adjacent pulmonary vein. It occurs most commonly at the origin of the
superior vena cava. Sinus venosus defects are almost always associated with partial anomalous return of
the pulmonary veins (either right superior or both right-sided pulmonary veins). Because of the location
of this type of atrial septal defect (ASD), traditional transthoracic echocardiograms often miss the
defect, so transesophageal echocardiography, cardiac computed tomography, or cardiac magnetic
resonance imaging are often required to make the diagnosis. Apical displacement of the tricuspid valve,
or Ebstein anomaly is associated with secundum ASDs and atrioventricular accessory pathways. A right-
sided aortic arch is a rare anatomical variant and is associated with tetralogy of Fallot. There is no
association of ventricular septal defects with sinus venous defects.

Answer : A
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Key Point
Partial anomalous pulmonary venous return is almost always present in patients with sinus venosus
atrial septal defect.

Question 29 of 30

A 42-year-old woman was admitted to the hospital with right-sided facial weakness and difficulty
speaking. She has no past medical history. Computed tomography of the brain was negative for
intracranial hemorrhage. Magnetic resonance imaging of the brain showed a large acute stroke in the
territory of the left middle cerebral artery as well as a small old stroke in the territory of the right middle
cerebral artery. She was successfully treated with endovascular thrombectomy. Her current vital signs
are stable and her cardiac examination is unremarkable.

A transthoracic echocardiogram (TTE) showed normal left ventricular size and function, normal right
ventricular size and function, and trace mitral and tricuspid regurgitation. The visualized portion of the
aortic root was normal. No intracardiac masses were seen. A saline bubble study was negative at rest ,
with Valsalva, and with cough.

Which of the following is the most appropriate next step in the management of this patient?

A. Transcranial Doppler.

B. Cardiac computed tomography.

C. Right heart catheterization.

D. Transesophageal echocardiography.

E. Cardiac magnetic resonance imaging.

Transesophageal echocardiography (TEE) with agitated saline contrast, with provocative maneuvers
such as coughing, Valsalva, or abdominal pressure (in sedated patients) is more sensitive for diagnosing
intermittent interatrial shunting from a patent foramen ovale (PFO) and therefore the best answer. The
finding of ischemic strokes of different ages in the territories of the carotid and middle cerebral arteries
suggests cardioembolism as a cause of strokes, as opposed to lacunar strokes from small vessel disease
or watershed territory infarcts from other causes. The sensitivity of TEE for diagnosing the cause of
cardioembolic strokes is higher than TTE and is likely to be helpful if TTE is of poor quality in patient
patients with stroke, in patients with stroke of unknown or unclear etiology, and in those with non-
lacunar strokes. Additionally in this young patient, suspicion for paradoxical embolus via an atrial septal
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defect or PFO is high; therefore further investigation with a TTE is warranted. PFO is present in
approximately 25% of the general population and up to 40% of younger patients with strokes.

Transcranial Doppler has high (>90%) sensitivity and specificity for right-to-left shunting, but is limited in
many adults due to the absence of a suitable temporal bone window and also by the inability of
transcranial Doppler to localize the anatomic origin of the shunt. Cardiac computed tomography (CT)
can identify aortic atherosclerosis or intracardiac thrombus. The main limitation of CT is the lack of
inherent soft-tissue contrast, which limits its assessment of the myocardium and identification of small
thrombi. Intermittent shunting or opening of a PFO may not be detected using single-beat CT
acquisition.

Cardiovascular magnetic resonance may have a role as an adjunct to echocardiography in selected

patients with stroke, such as tissue characterization of cardiac tumors or masses seen with
echocardiography or intracardiac thrombus evaluation, but does not currently have an evidence-based
role in the routine evaluation of stroke.

Right heart catheterization can diagnose intracardiac shunts, particularly left-to-right shunt lesions, but
is not sensitive for small intermittent shunting from a PFO. Additionally, right heart catheterization does
not reveal structural details of the shunt lesion and is more invasive than the other options listed.

Answer : D
Key Points

1. Patent foramen ovale occurs in approximately 25% of the population, does not result in left-
to-right shunt-related complications, and is only rarely associated with any pathologic
sequelae (stroke, migraine, platypnea-orthodeoxia, or provoked exercise desaturation).
2. Patent foramen ovale (PFO) is more common in patients with cryptogenic stroke. In patients
with cryptogenic stroke, randomized trials suggest that PFO closure, when added to medical
therapy, reduces the risk of subsequent stroke.

Question 30 of 30

A 20-year-old college sophomore presents to your office for evaluation of aortic root dilation. He had an
echocardiogram after his internist heard a murmur, which revealed normal biventricular function with
aortic root dilation of 5.0 cm (Z score 6.74). He denies chest pain, shortness of breath, palpitations, or
syncope. He has a history of lens dislocation at age 17. He takes no medications. He is adopted and
unaware of his family history.
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What is the most likely diagnosis?

A. Marfan syndrome.

B. Loeys-Dietz syndrome.

C. Williams syndrome.

D. Noonan syndrome.

E. Ehlers-Danlos syndrome.

Marfan syndrome (MFS) is an autosomal dominant condition with mutations most commonly involving
the gene (FBN1) encoding the connective tissue protein fibrillin-1. Classically, patients have ocular,
cardiovascular, and musculoskeletal manifestations. Stringent criteria for the diagnosis of MFS (Ghent
nosology) were proposed in 1996 and revised in 2010. The 2010 revised Ghent nosology puts greater
weight on aortic root dilatation/dissection and ectopia lentis as the cardinal clinical features of MFS and
on testing for mutations in FBN1 (Figure 1). This patient meets the criteria based on aortic root dilation
(Z score ≥2; aortic root indexed to body surface area) along with lens dislocation. Alternatively, if there is
no lens dislocation, a calculation of the systemic score would need to be calculated. A score over 7 with
aortic dilation would also meet the criteria (www.marfan.org/dx/score).

The discriminating features of Shprintzen-Goldberg syndrome (mental retardation), Loeys-Dietz

syndrome (bifid uvula, thin and velvety skin, easy bruising), and vascular Ehlers-Danlos syndrome
(translucent skin, dystrophic scars, intestinal rupture) are not present. Loeys-Dietz syndrome is ruled out
by the absence of other signs, including bifid uvula/cleft palate, arterial tortuosity, hypertelorism,
diffuse aortic and arterial aneurysms, craniosynostosis, clubfoot, cervical spine instability, thin and
velvety skin, and easy bruising. Williams syndrome is characterized by unusual facial features,
intellectual disability, and hypercalcemia, not consistent with this patient's presentation. Noonan
syndrome manifests as short stature and congenital heart disease, most often pulmonic stenosis.

Answer : A
Key Point
Marfan syndrome is an autosomal-dominant connective tissue disorder that is diagnosed based on
the revised Ghent criteria, which include the aortic dimension, family history, genetic testing, ectopia
lentis, and a systemic score of phenotypic features. Marfan syndrome diagnosis requires a
multidisciplinary evaluation including physical examination, slit-lamp eye examination,

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Pericardial disease

Question 1
1-A 35-year-old man who recently immigrated from Africa presented with
progressive chest pain of two weeks' duration. He described a sharp pain
that increased with inspiration and was relieved by sitting forward. He had
no past medical history and took no medications.

His vital signs were temperature 100.5°F, pulse 100 bpm, blood pressure
118/68 mm Hg, and respirations 20 per minute. A physical examination
revealed a diaphoretic man in moderate distress. A cardiac examination
showed normal S1 and S2 with a pericardial friction rub. The lungs were
clear. There was no peripheral edema.

Laboratory evaluation was remarkable for a white blood cell count of

17,000 per mm3 and C-reactive protein 100 mg/L.

Which of the following is the most likely cause of his symptoms?

A. Echinococcus granulosus.
B. Histoplasma capsulatum.
C. Borrelia burgdorferi.
D. Staphylococcus aureus.
E. Mycobacterium tuberculosis.

This patient has acute pericarditis with typical chest pain and a pericardial
friction rub. A range of infectious agents may cause pericarditis (Figure 1).

In developing countries where tuberculosis is prevalent, tuberculosis

accounts for about 70% of pericarditis diagnoses and has a high mortality:
about 25% at 6 months in the absence of HIV infection and approximately
40% in those with associated HIV infection. Tuberculous pericarditis is
much less common in developed countries, accounting for <5% of all
cases. Immigration may increase these cases in developed countries, such
as in this patient.
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S. aureus infections should be considered in patients with recent

instrumentation of the pericardium or cardiac surgery.
B. burgdorferi, the causative agent of Lyme disease, is a rare cause of
pericarditis that is endemic to the northeastern United States.
H. capsulatum, endemic to the Ohio River Valley, rarely causes pericarditis
but is nevertheless the most common cause of fungal pericarditis in
immunocompetent patients.
Echinococcus, a parasite, is a very rare cause of pericarditis. Answer E

Key Point
Pericarditis may be infectious (most often viral or tuberculous) or
noninfectious (especially autoimmune, cancer related, and as part of post–
cardiac injury syndrome), but most cases remain idiopathic (without a
known cause).
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Question 2

2-A 36-year-old woman presented to the emergency department with

worsening chest pain. Her past medical history was significant for
pericarditis in the past year. She was not receiving any medication. Review
of symptoms was unremarkable apart for facial flushing and myalgia. On
examination, she was afebrile with blood pressure of 100/50 mm Hg and
heart rate of 100 bpm. Cardiac examination revealed a pericardial rub. An
electrocardiogram (ECG) was obtained (Figure I).

Echocardiogram revealed a small pericardial effusion without tamponade.

What is the most appropriate next test ?

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A. Antinuclear antibody.
B. Tuberculin skin test.
C. Thyroid stimulating hormone.
D. Viral serology.
E. Cardiac magnetic resonance imaging.

Pericardial involvement in systemic autoimmune diseases may be

symptomatic (pericarditis and symptomatic pericardial effusion) or
asymptomatic (usually pericardial effusion); it generally reflects the degree
of activity of the underlying disease. Approximately 5-15% of patients with
acute or recurrent pericarditis may have a systemic autoimmune disease,
either overt or underlying. Pericardial involvement is common in systemic
lupus erythematosus (SLE), Sjögren syndrome, rheumatoid arthritis, and
scleroderma, but may also be present in systemic vasculitides, Behçet
syndrome, sarcoidosis, and inflammatory bowel diseases.

Pericardial involvement rarely occurs as the first manifestation of these

diseases. Antinuclear antibody (ANA) titer should be obtained in selected
cases (e.g., young women, especially those in whom the history suggests a
rheumatologic disorder). It is important to recognize that a positive ANA is a
nonspecific test.

Testing for tuberculosis with either a tuberculin skin test or interferon-

gamma release assay is most helpful in patients who are
immunocompromised or HIV positive and in regions where tuberculosis is
endemic.

Pericardial effusion may occur in approximately 5-30% of patients with

hypothyroidism. It is diagnosed by a high thyroid stimulating hormone level,
and clinically is characterized by relative bradycardia and low QRS voltage
in the ECG, neither of which are present in this case.

Viral serology has low yield and would not provide a likely diagnosis.

A recent study shows that cardiac involvement as observed by cardiac

magnetic resonance (CMR) is frequent in SLE and not necessarily
associated with typical symptoms. CMR may help to detect subclinical
cardiac involvement, which could lead to earlier treatment. Imaging
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techniques for the detection of pericardial inflammation (e.g., CMR) may

identify forms of initial reversible constrictive pericarditis, allowing a trial of
medical anti-inflammatory therapy that may reduce the need for surgery.
However, this would not be the most appropriate next test. Answer A

Key Point
Any disease process that affects the pericardium may result in a pericardial
effusion. The diagnosis is most often established through
echocardiography.

Question 3
3-A 68-year-old woman with a history of osteoarthritis, hypertension, and
chronic lower extremity edema presented with 2 days of unremitting chest
pain. Home medications included lasix 40 mg daily, as well as glucosamine
chondroitin and celecoxib 200 mg daily. A physical examination was
notable for temperature 37°F, heart rate 90 bpm, blood pressure 160/100
mm Hg, and oxygen saturation of 98% on room air. Heart sounds were
normal without murmurs. Lung examination was clear. Extremities were
with 1+ bilateral ankle edema and normal pulses.

An electrocardiogram (ECG) was obtained (Figure 1). Laboratory values

included white blood cell count of 10/mcL, hematocrit of 40%, and creatine
of 1.0 mg/dL.

Which of the following is the next best step in her care?

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(Figure 1)
A. Prednisone 40 mg daily.
B. Nitroglycerin 0.4 mg sublingual.
C. Colchicine 0.6 mg twice daily.
D. Therapeutic heparin drip.
E. Increase lasix to 40 mg twice daily.

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The ECG shows diffuse ST elevation and P-R depression that, in

combination with her 2 days of unremitting chest pain, is most consistent
with acute pericarditis. Cochicine 0.6 mg daily (<70 kg) or BID (= 70 kg) for
3 months is recommended as first-line therapy in addition to anti-
inflammatory medications (typically high-dose aspirin three times a day or
ibuprofen). She is already on a nonsteroidal anti-inflammatory drug
(celecoxib) as an outpatient.

Acute pericarditis can present with pleuritic chest pain, a pericardial friction
rub, diffuse ST elevations on ECG, and pericardial effusion.

The ECG and presentation are not consistent with acute coronary
syndrome; therefore, nitroglycerin and heparin are not indicated.

Steroids should be avoided in the initial treatment of acute pericarditis due

to the increased risk of recurrence.
Pericardial effusions commonly accompany acute pericarditis and can lead
to cardiac tamponade. Therefore, diuretics should be used judiciously.
Answer C
Key Point
Acute pericarditis should be treated with a nonsteroidal anti-inflammatory
drug (NSAID), typically with a 2-week to 4-week taper after resolution of
symptoms, along with colchicine (3 months); this combination reduces the
risk for recurrent pericarditis compared with an NSAID alone. Because of
the risk for significant side-effects and increased risk of recurrent
pericarditis, glucocorticoid therapy should be reserved for patients unable
to take NSAID therapy or for those with specific indications (e.g.,
autoimmune disease, renal failure, pregnancy, concomitant anticoagulant
therapy).

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Question 4

4- A 43-year-old woman was seen in clinic for a recent cough, sore throat,
and mild shortness of breath. She had no past medical history and was not
on any medications. Her vitals and examination were unremarkable. She
was sent for a chest X-ray (Figure 1). Based on its abnormal appearance,
she subsequently underwent a computed tomography (CT) chest scan
(Figure 2).

What is the most likely diagnosis of the finding indicated by the arrow in
Figure 2?

(Figure 1)
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(Figure 2)
A. Teratoma.
B. Pericardial effusion.
C. Pericardial cyst.
D. Hiatal hernia.
E. Lymphoma.

The chest X-ray shows an echodensity obscuring the right diaphragm and
cardiac border. The CT scan reveals a thin-walled fluid-containing structure
abutting the pericardium near the right atrium. This is most consistent with
a pericardial cyst. A pericardial effusion would be contained within the
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normal pericardium and not extend so far away from the heart in one
direction. A hiatal hernia is usually a retrocardiac mass (near the left
atrium) that often contains an air-fluid level. A teratoma is composed of
heterogenous tissue and is thus less likely to have such a homogeneous
appearance. Lymphoma would more commonly appear as a poorly defined
mass and would be unlikely without constitutional symptoms.

In general, pericardial cysts are incidental findings and are not intervened
upon unless they are believed to be causing significant symptoms (usually
shortness of breath or chest pain). Answer C
Key Point
Congenital pericardial abnormalities such as absence of the pericardium
and pericardial cysts are rare. Detection, when it occurs, is typically
incidental. Occasionally, however, these abnormalities may cause
symptoms or life-threatening consequences such that intervention is
necessary.

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Question 5

5-A 38-year-old woman presented for follow-up of pericarditis and

complained of chest discomfort, which was worse with deep inspiration and
when in a recumbent position. She was previously treated with a
combination of ibuprofen 400 mg BID for 2 weeks and colchicine 0.6 mg
BID for 3 months. She completed this treatment 2 months prior. At the time
of initial diagnosis, she had a pericardial friction rub and an
electrocardiogram (ECG) demonstrating diffuse 1 mm ST elevations.
Echocardiogram revealed normal ventricular function, no valvular disease,
and normal-appearing pericardium without a pericardial effusion.

ECG performed upon presentation demonstrated normal sinus rhythm

without ST segment or T-wave abnormalities.

What is the most appropriate next step?

A. Colchicine and ibuprofen.
B. Cardiac magnetic resonance imaging.
C. Azathioprine.
D. Prednisone.
E. High-sensitivity C-reactive protein.

This patient presents with a clinical syndrome consistent with recurrent

pericarditis. Recurrent pericarditis can occur in 15-30% of cases, but may
be as high as 50% in patients not treated with colchicine initially. This
remains a clinical diagnosis, so recurrence of typical symptoms within this
time frame strongly supports the diagnosis of recurrent pericarditis.
Although there were ST elevations when she was initially diagnosed with
pericarditis 5 months prior, the ECG in recurrent pericarditis is often less
specific.

Medical therapy for recurrent pericarditis is similar to the therapy for acute
pericarditis, which is colchicine BID plus a nonsteroidal anti-inflammatory
drug (NSAID), usually ibuprofen or aspirin. Since she responded favorably
to colchicine and ibuprofen initially, resuming these medications would be
the most reasonable next step.
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Glucocorticoid use in recurrent pericarditis is generally reserved for those

with a contraindication to NSAID therapy or those who have failed NSAID
therapy because glucocorticoid therapy has been shown to increase the
likelihood of further recurrences.

In patients with refractory pericarditis or in an attempt to minimize the use

of long-term corticosteroids, azathioprine and intravenous immunoglobulin
have been used. This would not be appropriate in this case.

Cardiac magnetic resonance imaging or computed tomography can be

helpful to evaluate for pericardial abnormalities or inflammation. However,
this is generally reserved for atypical cases or where the diagnosis is in
doubt.

A high-sensitivity C-reactive protein is unlikely to alter medical therapy at

this time. Answer A
Key Point
Recurrent pericarditis, which may occur in =30% of patients after an initial
episode of acute pericarditis, should be treated with a nonsteroidal anti-
inflammatory drug (NSAID), typically with a 2-week to 4-week taper after
resolution of symptoms, along with colchicine (6-12 months). Glucocorticoid
therapy should be reserved for those who have failed multiple attempts at
therapy with an NSAID plus colchicine. Few patients will need more
advanced therapies, such as azathioprine, biological agents (especially
anti–interleukin-1 agents), human intravenous immunoglobulins, or
pericardiectomy.

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Question 6

6-A 67-year-old man presented to the emergency department with

lightheadedness and exertional dyspnea. He had a history of malignant
pericardial effusion with tamponade, treated with pericardiocentesis 2
months prior, and stage IV non–small cell lung cancer on chemotherapy.
His heart rate was 94 bpm, blood pressure 104/78 mm Hg, respiratory rate
16 breaths per minute, and oxygen saturation 93% on ambient air.
Examination revealed a 6 mm Hg drop in systolic blood pressure with
inspiration and was otherwise unremarkable. Echocardiography confirmed
a recurrent large pericardial effusion.

Which of the following is the best treatment?

A. Prednisone.
B. Ibuprofen and colchicine.
C. Pericardial window.
D. Pericardiectomy.
E. Pericardiocentesis.

This patient has recurrent malignant pericardial effusion. A pericardial

window allows drainage of pericardial fluid into the pleural space and is
used as a palliative strategy in this scenario. Intrapericardial
sclerosing/cytotoxic agents (e.g., cisplatin) may help reduce recurrent
malignant pericardial effusion and could also be considered.

Pericardiectomy is used primarily for constrictive pericarditis.

Pericardiocentesis would remove the fluid but given the likelihood of

recurrence pericardial window would be the preferred option.

Anti-inflammatory treatments (prednisone, NSAIDs and colchicine) are

therapies for acute pericarditis and are not appropriate here. Answer C
Key Point
Urgent pericardiocentesis is the only definitive therapy for cardiac
tamponade. This is most often accomplished percutaneously, rather than

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surgically. Intravenous fluid administration may improve cardiac output, but

is only a temporizing measure while facilitating drainage of the fluid.

Question 7

7-Which of the following factors is most important in predicting

hemodynamic consequences of a pericardial effusion?
A. Volume of fluid.
B. Composition of fluid.
C. Rate of accumulation.
D. Pericardial thickness.
E. Patient age.

The acuity with which pericardial effusion accumulates determines whether

the features of cardiac tamponade become manifest. This is shown in
Figure 1, where the diastolic pressure-volume relationship of the pericardial
space is shown for someone with a normal pericardium (in red) and for
someone with chronic pericardial disease (in blue) wherein the pericardium
has stretched over time.

The normal pericardium contains about 50 cc of fluid. Acute accumulation

of about 250 cc of fluid can increase the pericardial fluid pressure enough
to elicit tamponade physiology. In the chronic setting, tamponade may not
become evident even after over a liter of fluid has accumulated.
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Pericardial thickness, composition of pericardial fluid or etiology of the

effusion, and patient age are not as important for the development of
hemodynamic consequences.

Location of the pericardial effusion may also play a factor, in that loculated
effusions near the thin-walled atria may be more likely to produce chamber
collapse than similar-sized effusions near the thick-walled ventricles.
Answer C

Key Point
Cardiac tamponade is due to a hemodynamically unstable pericardial
effusion, the presentation of which largely depends on the rate of fluid
accumulation and the clinical situation. Tamponade is a life-threatening
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cause of cardiogenic shock that may develop acutely or chronically, and

should be suspected in patients with unexplained sinus tachycardia,
hypotension, elevated jugular venous pressure, or pulseless electrical
activity.

Question 8

8-A 56-year-old man presented to the emergency department with sharp,

stabbing retrosternal chest pain. The pain was worse with inspiration. He
had no medical history and took no medications. His vital signs were blood
pressure 138/82 mm Hg, pulse 90 bpm, and oxygen saturation 98% on
room air. On examination, he appeared uncomfortable and was sitting up
on the stretcher. A scratchy sound was heard along the left sternal border.

His electrocardiogram (ECG) showed sinus tachycardia at 104 bpm but

was otherwise unremarkable.

An echocardiogram would be expected to demonstrate which of the

following?
A. Small pericardial effusion.
B. Dilated, hypokinetic right ventricle.
C. Apical and anteroseptal left ventricular hypokinesis.
D. Dissection flap in the aortic root.
E. Midsystolic billowing of the mitral valve into the left atrium.

The patient's presentation is most consistent with acute pericarditis. The

clinical diagnosis can be made with two of the following four criteria: 1)
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typical chest pain (sharp, pleuritic, improved by sitting up and leaning

forward); 2) pericardial friction rub (present in less than one-third of
patients); 3) ECG changes (classically diffuse ST elevations and/or P-R
depressions, except in aVR, where the findings are reversed); and 4)
pericardial effusion. Thus, a small pericardial effusion might be seen on
echocardiography.

A dilated, hypokinetic right ventricle may be seen in acute pulmonary

embolism.

Apical and anteroseptal hypokinesis may be seen in acute myocardial

ischemia/infarction and would not be expected without ischemic ECG
changes.

Midsystolic billowing of the mitral valve >2 mm above the annular plane is
characteristic of mitral valve prolapse.

The pleurtic pain that improves with sitting up is not typical for acute aortic
syndrome, nor would aortic dissection explain the friction rub, so this is not
the preferred choice. Answer A
Key Point
Acute pericarditis is diagnosed when at least two of the following criteria
are met: 1) characteristic chest discomfort (persistent, pleuritic, and
positional); 2) suggestive electrocardiographic changes (typically, diffuse
ST elevation); 3) pericardial friction rub; and 4) new or worsening
pericardial effusion.

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Question 9

9-A 75-year-old man presented to the emergency department with fatigue,

dyspnea, chest pain, and lightheadedness. His symptoms started
approximately 1 week prior and have progressively worsened. His
lightheadedness occured with standing and resulted in near-syncope.

On examination, heart rate was 110 bpm, blood pressure 80/46 mm Hg,
respiratory rate 20/min, and temperature 98.6°F. His heart sounds were
distant but with a normal S1 and S2 without murmur. Jugular venous
distention was present. His remaining examination was normal.

Which of the following is the most appropriate diagnostic study?

A. Right heart catheterization.
B. Transthoracic echocardiogram.
C. Transesophageal echocardiography.
D. Cardiac computerized tomography.
E. Cardiac magnetic resonance imaging.

The patient has cardiac tamponade. On examination, the patient has

Beck's triad, which includes hypotension, jugular venous distention, and
decreased heart sounds. The most appropriate diagnostic study is a
transthoracic echocardiogram (TTE), which should be performed
immediately if tamponade is suspected.

On TTE, typical findings include:

• Right atrial notching in atrial diastole;

• Right ventricular collapse in early diastole (the most specific finding
for cardiac tamponade);
• Abnormal ventricular septal motion;
• Dilated, noncompressible inferior vena cava with blunted respiratory
changes; and
• Excessive respiratory variation of tricuspid and mitral valve inflow
velocities.

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Transesophageal echocardiography can also be used to diagnose

tamponade but is often not needed unless TTE is nondiagnostic.

Computerized tomography and magnetic resonance imaging can assess

the presence and size of a pericardial effusion; however, echocardiography
is the first-line diagnostic modality.

Right heart catheterization would confirm equalization of diastolic pressures

throughout the heart but is not the first-line test. Answer B
Key Point
Any disease process that affects the pericardium may result in a pericardial
effusion. The diagnosis is most often established through
echocardiography.

Question 10

10-A 55-year-old man with a history of idiopathic pericarditis 1 year prior

was admitted with progressive abdominal fullness, edema, and fatigue. He
had no history of radiation exposure or prior cardiac surgery. His
medications at the time of presentation included torsemide 40 mg BID,
metoprolol succinate 12.5 daily, lisinopril 2.5 mg daily, and spironolactone
25 mg daily. His vital signs were blood pressure 98/68 mm Hg, heart rate
90 bpm, and respirations 12 per minute. On examination, he had jugular
venous distension without inspiratory decline. No murmurs were heard. His
liver was mildly distended and pulsatile. He had pitting bilateral lower
extremity edema.

Laboratory studies included creatinine 1.3 mg/dL, sodium 134 mg/dL,

serum albumin 3.8 md/dL, and B-type natriuretic peptide 300 pg/mL.
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A transthoracic echocardiogram showed normal left ventricular (LV) wall

thickness, LV ejection fraction 50%, prominent septal bounce, mitral E'
septal velocity 13 cm/sec and mitral E' lateral velocity 6 cm/sec, and
diastolic hepatic venous flow reversal increased with expiration. Cardiac
magnetic resonance imaging (MRI) showed ventricular interdependence
with 4 mm unenhancing pericardium and no evidence of late gadolinium
enhancement of the myocardium.

What is the most appropriate next step in management?

A. Surgical pericardiectomy.
B. Azathioprine.
C. Left ventricular assist device.
D. Milrinone.
E. Digoxin.

The history of prior pericarditis and the presence of examination findings of

elevated right heart filling pressures with Kussmaul's sign (jugular venous
distension without inspiratory decline) is suggestive of constrictive
pericarditis. Echocardiographic and MRI findings are diagnostic for
pericardial constriction. Surgical pericardiectomy is the only definitive
therapy for constrictive pericarditis. The patient appears to be symptomatic
and a reasonable candidate for cardiac surgery. The long-term outcomes
for pericardiectomy are best for patients with idiopathic constrictive
pericarditis as opposed to postsurgical or radiation induced.

The absence of gadolinium enhancement by MRI suggests there is no

active pericardial inflammation; therefore, the addition of anti-inflammatory
therapies is of no benefit.

Inotropic therapy or digoxin may be appropriate for palliation of heart failure

but would not be the best option for this patient with constrictive
pericarditis.

The LV assist device is not indicated for treatment of constrictive

pericarditis. Answer A
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Key Point
Oftentimes, constrictive pathophysiology is chronic and will cause
progressive heart failure. Surgical pericardiectomy is the only curative
therapy in these cases.

Question 11

11- A 45-year-old man came to clinic with lower extremity swelling. Six
months prior, he was treated for acute pericarditis with colchicine and
ibuprofen with resolution of his chest pain. Since then, however, his pants
had fit tighter and he could not wear his socks and shoes because of ankle
swelling. His medications at the time of presentation were furosemide 40
mg daily.

His vital signs were pulse 78 bpm, respirations 14 per minute, and blood
pressure 104/64 mm Hg. An examination revealed mild jugular venous
distension that increased with inspiration. The lungs were clear. The
cardiac examination revealed soft S1 and S2 with a diastolic gallop. The
abdomen was distended and nontender. There was 3+ bilateral lower
extremity edema to the waist. Laboratory values include CRP 2.0 mg/L and
ESR 15 mm/hr.

Representative tissue Doppler tracings from his echocardiogram are shown

in Figure 1.

Which of the following would be the most appropriate next step in his
treatment?

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(Figure 1)
A. Pericardiectomy.
B. Pericardiocentesis.
C. Colchicine.
D. Lisinopril.
E. Pericardial window.

This patient has signs and symptoms of constrictive pericarditis (CP) with
lower extremity edema, Kussmaul's sign, and a pericardial knock. The
tissue Doppler on echocardiogram shows a lateral mitral annular velocity
less than the medial mitral annular velocity, which is the reverse of the
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normal pattern, in which the lateral annulus has greater freedom of

movement than the septal annulus. This is the so-called annulus reversus;
it represents constraint of mitral annular motion by the abnormal
pericardium with a compensatory increase in the septal motion. Definitive
treatment consists of removal of the diseased visceral pericardium.

Pericardial window or pericardiocentesis is not indicated; these address

pericardial fluid but do not address the constraint imposed by the diseased
pericardium.

Medical heart failure treatment is not likely to be effective.

Colchicine has been shown to reduce the likelihood of CP following acute

pericarditis but does not have a role in managing CP. Answer A

Key Point
A comprehensive echocardiogram with expanded Doppler can reveal
constrictive physiology. Key features include septal bounce, ventricular
interdependence (including respiration-related ventricular septal motion and
expiratory end-diastolic hepatic vein flow reversal), restrictive LV filling, IVC
plethora and relatively increased LV medial wall tissue early diastolic
velocity (e’).

Question 12
12-A 44-year-old woman with exertional fatigue, weakness, and lower
extremity edema for the past year came to your office for evaluation. She
had no past medical history. Her medications at the time of presentation
included furosemide 80 mg daily.

Vital signs were blood pressure 98/68 mm Hg, heart rate 78 bpm, and
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respirations 14 per minute. An examination revealed elevated jugular

venous pressure and bilateral lower extremity edema.

Hemodynamic tracings are shown in Figure 1.

Which of the following is the most likely diagnosis?

(Figure 1)
Exp = expiration; Insp = inspiration; LV = left ventricular; RV = right ventricular.

Reproduced with permission from Geske JB, Anavekar NS, Nishimura RA, Oh
JK, Gersh BJ. Differentiation of constriction and restriction: complex
cardiovascular hemodynamics. J Am Coll Cardiol2016;68:2319-47.
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A. Constrictive pericarditis.
B. Restrictive cardiomyopathy.
C. Severe mitral stenosis.
D. Severe mitral regurgitation.
E. Cardiac tamponade.

The hemodynamic tracing should be recognized as simultaneous left

ventricular (LV) and right ventricular (RV) measurements. Simultaneous
right- and left-sided hemodynamic measurements can help differentiate
constrictive from restrictive physiology. The dip-and-plateau (i.e., square
root sign) during diastole occurs due to rapid early diastolic filling, which is
abruptly halted due to constricted volume. The square root sign is a classic
finding with constrictive pericarditis but can also occur with restrictive
cardiomyopathy. However, the most specific finding is the presence of
ventricular interdependence.

In constrictive pericarditis, the stiff pericardium restricts total ventricular

filling in the two chambers with a shared interventricular septum, resulting
in exaggerated ventricular interdependence: during inspiration, right atrial
pressures decrease transiently, resulting in increased venous return and
RV filling and decreased LV filling, which leads to bowing of the
interventricular septum towards the LV. During expiration, the reverse
occurs.

Ventricular concordance, with simultaneous drop in both RV and LV filling

during inspiration, is seen with restrictive cardiomyopathy.

Mitral stenosis can be assessed by cardiac catheterization using

simultaneous LV and left atrial (LA) pressures (or pulmonary capillary
wedge pressure [PCWP] pressures) indicating a significant diastolic
gradient between the LA (or PCWP) and LV.

Severe mitral regurgitation will typically demonstrate tall V waves on LA

pressure tracing.

Cardiac catheterization is not typically performed to assess cardiac

tamponade but would reveal equalization of the diastolic pressures
between all four cardiac chambers due to the elevated pericardial
pressures. Answer:A
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Key Point
Constrictive pathophysiology is a hemodynamic diagnosis. An invasive
hemodynamic assessment with simultaneous recordings of right and left
ventricular pressures remains the gold standard for diagnosis.
Echocardiography provides extensive hemodynamic information and is
particularly sensitive for early or mild constrictive pathophysiology. Cross-
sectional imaging with magnetic resonance imaging and computed
tomography may also be helpful in securing the diagnosis and planning
treatment.

Question 13
13-A 32-year-old woman with no significant past medical history presented
to the emergency department with 3 days of progressive shortness of
breath and pleuritic chest pain. On examination, her heart rate was 110
bpm, blood pressure 115/78 mm Hg, and oxygen saturation of 98% on
room air. She was unable to lay flat due to shortness of breath. Jugular
veins were distended sitting upright. Heart sounds were tachycardic and
distant without murmurs or rubs. Lungs were clear to auscultation.
Extremities showed no edema. An electrocardiogram demonstrated 1 mm
of diffuse ST elevations with P-R segment depressions.

A stat bedside echocardiogram was performed, showing a moderate-sized

pericardial effusion with right ventricular diastolic collapse.

She was taken for right heart catheterization and pericardiocentesis was
performed; 350 mL of serosanguenous fluid was drained.

Following pericardiocentesis, which of the following findings is consistent

with effusive constrictive pericarditis?
A. Pulmonary artery systolic pressure >50 mm Hg.
B. Pulmonary vascular resistance of >4 Woods units.

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C. Intrapericardial pressure 1 mm Hg.

D. Right atrial pressure 12 mm Hg.
E. Pulmonary capillary wedge pressure 9 mm Hg.

Failure of the right atrial pressure to fall by 50% or to a level <10 mm Hg

after pericardiocentesis is consistent with effusive-constrictive pericarditis.
Pulmonary artery systolic pressure of >50 mm Hg is consistent with
pulmonary hypertension and not diagnostic of effusive constrictive
pericarditis.
Elevated pulmonary vascular resistance is seen in primary pulmonary
arterial hypertension.
Intrapericardial pressure of 1 mm Hg is normal and is expected after
pericardiocentesis for tamponade. Effusive constrictive pericarditis would
have an elevated intrapericardial pressure.
Pulmonary capilliary wedge pressure (PCWP) of 9 mm Hg is normal. In
effusive constrictive pericarditis, the PCWP is typically elevated. Answer D
Key Point
Occasionally, constrictive pathophysiology is subacute and potentially
transient due to pericardial inflammation (transient constrictive pericarditis).
In addition, constrictive pathophysiology may occur after pericardiocentesis
when intracardiac pressures fail to decline (effusive constrictive
pericarditis).

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Question 14

14-A 45-year-old woman was referred to your office for evaluation of a

newly diagnosed pericardial effusion, found after her internist ordered an
echocardiogram to evaluate a systolic murmur.

The patient denied chest pain, shortness of breath, palpitations, or

lightheadedness. She exercised by power walking 3 miles daily without
limitations. She had no past medical history and was on no medications.

On examination, temperature was 36.4°C, heart rate 64 bpm, and blood

pressure 110/70 mm Hg. Jugular venous pressure was flat, lungs were
clear to auscultation, heart was regular with a I/VI systolic murmur at the
left upper sternal border without radiation, and extremities were warm
without edema.

The echocardiogram showed normal ventricular and valvular function; the

pericardial effusion is shown in Figure 1.

What is the most appropriate next step?

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(Figure 1)
Reproduced with permission from HeartViews.org. 2017. Available at
http://www.heartviews.org/viewimage.asp?img=HeartViews_2017_18_4_145_
221228_f1.jpg. Accessed 03/06/2019.
A. C-reactive protein.
B. Percutaneous pericardiocentesis.
C. Pericardial biopsy.
D. Cardiac magnetic resonance imaging.
E. Troponin.

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The patient had an innocent flow murmur that prompted an

echocardiogram, which incidentally diagnosed a pericardial effusion. This is
a moderate effusion shown in Figure 1, as defined by 10-20 mm in greatest
diameter in diastole. The 2015 European Society of Cardiology guidelines
outline management of a pericardial effusion (Figure 2).

The first step is determine whether there is evidence of tamponade, or

hemodynamic significance caused by elevated intrapericardial pressure
impairing filling of the cardiac chambers. Symptoms and signs of
tamponade include shortness of breath, tachycardia, and elevated jugular
venous pressure. Echocardiographic evidence of hemodynamic
compromise includes right-sided diastolic chamber collapse and increased
variation in mitral and tricuspid inflow velocities. As this patient has no
clinical evidence of tamponade, repeat echocardiogram for further
assessment of hemodynamic compromise is not warranted.

Additional imaging of the pericardium with computed tomography or

magnetic resonance is reserved for patients with refractory pericardial
effusion or clinical signs of hemodynamic involvement (i.e., constrictive
pericarditis).

The next step in the algorithm is assessment of elevated inflammatory

markers, as this would mandate empiric anti-inflammatory therapy for an
associated pericarditis. This is because, in the absence of hemodynamic
compromise, the therapy for a pericardial effusion should be targeted at the
etiology. Thus, the correct answer in this case is to check a C-reactive
protein.

Pericardiocentesis is only indicated in cases of tamponade or symptomatic

moderate-to-large effusions not responsive to medical therapy.
Pericardiocentesis or pericardial biopsy would be indicated if there were a
suspicion for an unknown bacterial or neoplastic etiology. This patient is
otherwise healthy and asymptomatic, making a bacterial or neoplastic
source unlikely.

It is recommended that a troponin be checked in cases of pericarditis to

exclude myopericarditis. However, there is no indication to routinely check
troponin in an asymptomatic patient with an incidentally diagnosed
pericardial effusion.
Answer A
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(Figure 2)
Empiric Anti-inflammatory therapies should be considered if a missed
diagnosis of pericarditis is presumed.
Key Point
The hemodynamic effect of the effusion is typically assessed through a
combination of clinical and echocardiographic criteria. Hemodynamically
stable effusions may improve with treatment of the underlying disease
process; some will require drainage for diagnostic purposes.

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Question 15

15-A 66-year-old man was seen in clinic for ongoing dyspnea on exertion.
He had recurrent pericarditis after treatment with colchicine. A follow-up
echocardiogram revealed a hemodynamically significant pericardial
effusion 4 months prior, which was treated with a pericardial window.

Representative frames from his current echocardiogram are shown in

Video 1 and Video 2.

After reviewing the current echocardiographic videos, what additional

findings would you expect?

Vedios consistent with CP ( septal bounce)

A 30% drop in peak mitral inflow velocity during inspiration.

B. Mitral E wave velocity 130 cm/sec with deceleration time 240 msec.
C. Septal tissue Doppler annulus velocity 7 cm/sec.
D. Lateral tissue Doppler annulus velocity greater than septal annulus velocity.
E. Inspiratory reversal of systolic and diastolic flow velocities in the hepatic veins.

The correct answer is an inspiratory drop in the mitral inflow velocities

because the patient appears to have evidence for constrictive pericarditis.
This is strongly suggested by the interventricular septal "bounce" seen on
the videos. He likely had both pericardial tamponade and constriction when
he presented earlier and underwent a pericardial window procedure to
remove the pericardial fluid. He is now left with constrictive pericarditis and
that is likely why he remains symptomatic. In patients with constrictive
pericarditis, there are a number of hemodynamic events that occur, and
these events can be observed on the physical examination, during cardiac
catheterization, and with Doppler echocardiography. When constrictive
pericarditis is present, as one inspires and pulls blood through the right
heart, the right heart pressures rise rather than fall. This creates the
Kussmaul's sign and the ventricular "interdependence" observed at cardiac
catheterization.

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The Doppler echocardiogram can reflect these changes indirectly: Doppler

flow into the left ventricle is reduced with inspiration due to the right
ventricle (RV) filling and interventricular septal shift. This can be seen as a
drop in the pulmonary venous flow as well. This can also be shown by
noting an increase in the tricuspid E and A waves, with inspiration
accompanying the fall in the mitral E and A waves. When the hepatic veins
are interrogated, blood flow is pulled forward in both systole and diastole
during inspiration; however, during expiration, flow only goes forward in the
hepatic veins during systole (as the tricuspid ring is pulled into the RV) but
flow is reversed in diastole due to the high right-sided pressures. There is
thus a diastolic reversal of flow in the hepatic veins.

Because the ventricle can only fill in the first third of diastole and then filling
stops (the hemodynamic square root sign), the E wave velocity across the
mitral valve is very high (due to the high left atrial pressure), but the
deceleration time is very short and not blunted or slow. In general, the
tissue Doppler annular velocity reflects myocardial relaxation and it is
normally 7 cm/sec). Normally, as well, tissue Dopper is higher at the medial
mitral annulus than the lateral. In severe constriction, there is tethering of
the lateral mitral annulus to the constricted pericardium and the lateral
motion becomes greater than the medial motion. This latter is sometimes
called "annulus reversus." Of the echocardiographic options provided,
therefore, only the mitral inflow changes with inspiration are consistent with
the diagnosis of constrictive pericarditis. Answer A
Key Point
Occasionally, constrictive pathophysiology is subacute and potentially
transient due to pericardial inflammation (transient constrictive pericarditis).
In addition, constrictive pathophysiology may occur after pericardiocentesis
when intracardiac pressures fail to decline (effusive constrictive
pericarditis).

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Question 16

16-A 38-year-old man presented to the emergency department with 2 days

of sharp chest pain. The pain was worse when lying down and improved
slightly if he sat up straight. The week prior, he had a sore throat, cough,
and runny nose. He had no other medical problems and took no
medications. His vital signs were temperature 99.5°F (37.5°C), pulse 74
bpm, respirations 12 per minute, and blood pressure 110/70 mm Hg. He
appeared uncomfortable and was breathing shallowly. A cardiac
examination disclosed normally split S1 and S2; there was a pericardial
friction rub. The examination was otherwise normal. An electrocardiogram
was obtained (Figure 1).

In addition to an echocardiogram, which of the following tests is most

appropriate at this point?

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(Figure 1)
A. C-reactive protein.
B. Tuberculin skin test.
C. HIV viral load.
D. Antinuclear antibody.
E. Influenza RNA swab.

This patient has acute pericarditis. The diagnosis is made using clinical
criteria and may be confirmed by the presence of inflammatory markers
such as elevated C-reactive protein or demonstration of pericardial edema
on contrast computed tomography (CT) or magnetic resonance imaging
(MRI) (Figure 2). The presence of high-risk features such as fever, large
pericardial effusion or tamponade, and failure of symptoms to respond to
nonsteroidal anti-inflammatory therapy can identify patients who require
hospital admission for treatment. Testing for inflammatory markers may
also be useful to monitor response to therapy. Guidelines from the
American Society of Echocardiography and the European Society of
Cardiology recommend a step-wise approach to testing, with
echocardiography and laboratory evaluation for all cases of suspected
pericarditis, to assess for effusion and/or tamponade physiology (Figure 3)
and evaluate for myocardial involvement (myopericarditis). Advanced
imaging (CT and/or MRI) should be considered in cases with evidence of
myocardial involvement, hemodynamic compromise, ongoing fever or lack
of response to therapy, associated trauma, malignancy, tuberculosis, or
pancreatitis.

In North America and Western Europe, 80-90% of cases of acute

pericarditis are idiopathic and presumed viral in etiology (Figure 4). HIV and
influenza are not commonly associated with acute pericarditis, so testing
for these viruses is not warranted without other signs or symptoms to
suggest these infections. In general, virologic testing does not change
management.

In countries where tuberculosis is prevalent and among recent immigrants

in those areas, tuberculous pericarditis may be much more common (up to
70% of pericarditis cases) and carries a much higher mortality than
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idiopathic or viral causes. This patient does not have any risk factors for
tuberculosis.

Autoimmune and inflammatory disorders such as systemic lupus

erythematosus may affect the pericardium. Serologic testing for these
should be considered when other organ systems are involved, such as skin
and joints, but this patient has no clinical features to suggest this. Answer A

(Figure 2)
Reproduced with permission from Imazio M, Gaita F, LeWinter M. Evaluation
and treatment of pericarditis: a systematic review. JAMA 2015;314:1498-506.

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(Figure 3)
Transthoracic echocardiograms of pericarditis (panel A) without and (panel B)
with pericardial effusion.

(A) Parasternal long axis view showing “dry” (without pericardial effusion) acute
pericarditis characterized by increased brightness of the pericardial layers,
a nonspecific echocardiographic sign associated with fibrinous pericarditis.

(B) Parasternal long axis view showing a moderate (10-20 mm of telediastolic

echo-free space) pericardial effusion. Semiquantitative assessment of
pericardial
effusion is performed measuring the largest telediastolic echo-free space in
different echocardiographic views. A mild effusion is defined as <10 mm;
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moderate, between 10 and 20 mm; and large, >20 mm.5,19A large effusion is
associated with
an increased risk of complications and with specific etiologies (nonidiopathic
pericarditis; nonviral pericarditis). About 60% of patients with acute pericarditis
will have a pericardial effusion, generally mild.5,19 The absence of a
pericardial effusion does not exclude pericarditis. LA indicates left atrium; LV,
left ventricle

Reproduced with permission from Imazio M, Gaita F, LeWinter M. Evaluation

and treatment of pericarditis: a systematic review. JAMA 2015;314:1498-506.

Key Point
The initial diagnostic evaluation for all patients suspected to have acute
pericarditis includes blood work (including assessment for inflammation and

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myocardial damage), a chest radiograph, a 12-lead electrocardiogram, and

an echocardiogram.

Question 17

17-A 56-year-old woman with metastatic breast cancer presented to the

emergency department with progressive dyspnea and inability to lay flat.
Her vital signs were heart rate 120 bpm, blood pressure 100/60 mm Hg,
respiration rate 24 breaths/minute, and temperature 39°C. She was visibly
distressed.

Which echocardiographic finding would most likely rule out pericardial

tamponade?
A. Late diastolic collapse of the right atrium.
B. Inferior vena cava diameter 1.5 cm with inspiratory collapse.
C. Mitral valve respiratory flow variation 30%.
D. Tricuspid valve flow variation 60%.
E. Reduced lateral mitral annular velocity.

In addition to the confirmation of pericardial fluid, echocardiographic

findings suggestive of pericardial tamponade include: inferior vena cava
(IVC) plethora, diastolic blunting of hepatic vein forward flow, respiratory
inflow variation across the triscupsid valve of >60%, respiratory inflow
variation across the mitral valve of >30%, diastolic collapse of the right
atrium, diastolic collapse of the right ventricle, and left-sided chamber
collapse. IVC plethora (dilation and inspiratory reduction in diameter
<50%), indicative of elevated right atrial pressure, is highly sensitive for
pericardial tamponade, but not specific.
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The other answer options listed are less sensitive but more specific for
pericardial tamponade. In this case, normal IVC diameter and respiratory
variation suggest the need to explore an alternative diagnosis, such as an
infectious process given the elevated temperature.

The velocity of the mitral annulus may be reduced in cases of lateral wall
myocardial infarction or pericardial constriction, but is neither sensitive nor
specific for pericardial tamponade. Answer C
Key Point
Cardiac tamponade is a clinical diagnosis with key physical (elevated
venous pressure, pulsus paradoxus, hypotension) and echocardiographic
(chamber collapses, abnormal venous flows, exaggerated respiratory
variation in venous and cardiac flows, and vena caval plethora) findings.

Question 18

18-A 42-year-old woman with obesity presented to urgent care with 2

weeks of worsening dyspnea on exertion, fatigue, and occasional
palpitations. She reported a flu-like illness 1 month prior that resolved with
conservative management after several days. She denied any recent
cough, fever, or chills. Her only medication was an oral contraceptive. Her
vital signs were heart rate 108, blood pressure 102/76 mm Hg, respirations
16 per minute, and peripheral capillary oxygen saturation 97% RA. On
physical examination, she was in no apparent distress. Jugular venous
pressure was 10 cm H20. There were no murmurs and the lungs were
clear. The extremities were warm with trace pedal edema. An
electrocardiogram (ECG) revealed sinus tachycardia and low precordial
voltage.
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An echocardiogram would most likely demonstrate which of the following

findings?
A. Inferior vena cava plethora.
B. Left ventricular hypertrophy.
C. Severe mitral regurgitation.
D. McConnell's sign.
E. Increased left ventricular end-diastolic diameter.

This patient presented with a constellation of findings most consistent with

subacute pericardial tamponade that developed likely as a sequela of her
recent viral illness. Other tamponade signs and symptoms can include
chest pain, syncope/presyncope, tachypnea, hypotension, and a pericardial
rub. Measurement of an elevated pulsus paradoxus (inspiratory decrease
in systolic blood pressure >10 mm Hg) further suggests tamponade.
Pericardial tamponade develops when pressure due to fluid accumulation
within the pericardial space impedes cardiac filling. Tamponade can occur
acutely in the setting of trauma or chamber rupture, or subacutely if the rate
of pericardial fluid accumulation is more gradual.

The classic findings of low arterial blood pressure, dilated neck veins, and
muffled heart sounds (known as Beck's triad) are present in relatively few
patients with acute tamponade. ECG findings in tamponade may include
tachycardia, low voltages, and electrical alternans (beat-to-beat variation in
QRS complex). Echocardiography is the mainstay for rapid noninvasive
diagnosis, where the observation of pericardial effusion with chamber
collapse, increased mitral/tricuspid flow variation, and dilatation of the
inferior vena cava with <50% decrease in diameter on deep inspiration
(plethora) are hallmarks.

Severe mitral regurgitation and left ventricular hypertrophy or dilatation

would not be expected in an otherwise healthy patient with tamponade.
McConnell's sign is a finding of acute pulmonary embolism characterized
by abnormal right ventricular free wall contraction with sparing of the apex.
Answer A

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Key Point
Cardiac tamponade is due to a hemodynamically unstable pericardial
effusion, the presentation of which largely depends on the rate of fluid
accumulation and the clinical situation. Tamponade is a life-threatening
cause of cardiogenic shock that may develop acutely or chronically, and
should be suspected in patients with unexplained sinus tachycardia,
hypotension, elevated jugular venous pressure, or pulseless electrical
activity.

Question 19

19-You were consulting on a 53-year-old woman in the emergency

department on a Friday night who presented with complaints of shortness
of breath for the prior 2 days. She also described chills and nasal
congestion for the last week. She was taking no medications. Her blood
pressure was 86/64 mm Hg with a heart rate of 132 bpm. Her temperature
was 38.1°C. An examination revealed a mildly uncomfortable woman sitting
up in bed. Her jugular venous pressure was 13 cm of water. Her lungs were
clear. She was tachycardic without murmurs. An electrocardiogram showed
sinus tachycardia. A chest x-ray revealed an enlarged cardiac silhouette.

An echocardiogram revealed grossly normal left ventricular function with no

significant valvular disease. The right ventricle was not well visualized.
There was a moderate-sized circumferential pericardial effusion. The mitral
inflow velocity was 80 cm/sec with inspiration and 121 cm/sec with
expiration. The inferior vena cava (IVC) measured 2.9 cm.

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She was given 2 L of normal saline. Her blood pressure then became
100/78 mm Hg with a heart rate of 114 bpm.

Which of the following would be the best next step in management?

A. Urgent pericardiocentesis.
B. Repeat echocardiogram.
C. Computed tomography chest/abdomen/pelvis.
D. Intravenous dobutamine.
E. Pericardial window.

This patient's clinical presentation is consistent with cardiac tamponade.

She likely has a viral/idiopathic pericardial effusion that has enlarged to the
point of exerting hemodynamic effects. Her tachycardia and hypotension on
presentation are very concerning. Venous return is impaired due to
increased pericardial pressure, resulting in the elevation of her jugular
venous pressure despite hypotension. The echocardiogram shows >30%
variation in mitral inflow with respiration and a dilated IVC, both concerning
for tamponade physiology. Despite fluid resuscitation, the patient remains
tachycardic with tenuous hemodynamic status. As such, the best choice
would be urgent pericardiocentesis.

The effusion is circumferential and there would be no indication to delay for

consideration of surgical drainage. Waiting for a repeat echocardiogram is
not advisable in a clinical presentation consistent with tamponade.
Inotropes or vasopressor support may be helpful if she decompensates
while preparing for pericardiocentesis but should not be used instead of
definitive therapy. A computed tomography scan is also not a priority in
management and risks a decompensation in the radiology suite. Answer A
Key Point
Urgent pericardiocentesis is the only definitive therapy for cardiac
tamponade. This is most often accomplished percutaneously, rather than
surgically. Intravenous fluid administration may improve cardiac output, but
is only a temporizing measure while facilitating drainage of the fluid.

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Question 20

20-A 75-year-old man with a history of morbid obesity and atrial fibrillation
on warfarin presented with shortness of breath, lightheadedness, and new
lower extremity edema over the prior 3 days. In the emergency department,
he was in respiratory distress and unable to lie flat for the examination.
Heart rate was 120 bpm and blood pressure 100/75 mm Hg. Jugular
venous distension to the mandible was noted. Lung sounds were
diminished but clear. Cardiac examination revealed distant heart sounds.
Extremities were notable for 3+ bilateral edema.

Laboratory data were notable for white blood cell count 15, hemoglobin 10,
creatinine 1.6 mg/dL, troponin 0.12, and international normalized ratio
(INR) 1.2.

A chest X-ray showed cardiomegaly. A stat echocardiogram was performed

and an image was obtained (Figure 1).

What is the best next step?

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(Figure 1)
A. Pericardiocentesis.
B. Lasix 80 mg intravenous.
C. Heparin infusion.
D. Coronary angiogram.
E. Vitamin K 10 mg intravenous.

This patient has symptoms and findings consistent with cardiac tamponade
(elevated jugular venous pressure, tachycardia, with a large pericardial
effusion on echocardiogram and exaggerated respirophasic in-flow
variation across the tricuspid valve). Even in the absence of hypotension,
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the tachycardia and echocardiogram findings suggest hemodynamic

compromise; therefore, pericardiocentesis should be performed.

Lasix should not be given to a patient with hemodynamically significant

pericardial effusion because it can precipitate hemodynamic collapse.

In this patient, who may have a hemorrhagic effusion, heparin infusion

should be avoided.

The mild troponin elevation was likely due to demand ischemia from
cardiac tamponade; coronary angiogram is therefore not warranted.

Vitamin K is not indicated in this patient because his INR is subtherapeutic

at 1.2. Answer A
Key Point
The hemodynamic effect of the effusion is typically assessed through a
combination of clinical and echocardiographic criteria. Hemodynamically
stable effusions may improve with treatment of the underlying disease
process; some will require drainage for diagnostic purposes.

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Hypertension & Hypotension

Question 1 of 35

A 67-year-old woman with a history of ischemic cardiomyopathy and three-vessel coronary

artery bypass graft presents for routine follow-up. She has had worsening dyspnea with exertion
over the prior 3 months. She also notes higher blood pressure readings over the prior several
months.

On examination, she is in no distress and vital signs reveal a regular heart rate of 55 bpm, blood
pressure of 163/88 mm Hg, and oxygen saturation of 96% on room air. Her lungs are clear. She
has mild jugular venous distension to just above the clavicle and a positive hepatojugular reflux.
Her cardiac point of maximal impulse is diffusely enlarged with a palpable A wave. An S4 is
present but no S3. She has no edema and peripheral pulses are normal.

Her medications include carvedilol 12.5 mg BID, furosemide 20 mg daily, aspirin 81 mg daily,
atorvastatin 40 mg daily, metformin 500 mg twice daily, and lisinopril 40 mg once daily.

Pertinent laboratory values include sodium 140 mEq/L, potassium 4.0 mEq/L, creatinine 1.4
mg/dL, blood urea nitrogen 24 mg/dL, and hemoglobin 10.8 gm/dL. Her echocardiogram reveals
a left ventricular ejection fraction (LVEF) of 45% with no segmental wall motion abnormality,
moderate left ventricular hypertrophy, no mitral regurgitation, and no evidence for pulmonary
hypertension.

In addition to increasing her furosemide dose, the addition of which of the following is
recommended?

A. Carvedilol 25 mg twice daily.

B. Sacubitril/valsartan 24 mg/26 mg twice daily.

C. Spironolactone 12.5 mg daily.

D. Chlorthalidone 25 mg once a day.

E. Doxazosin 4 mg once a day.

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This patient has uncontrolled hypertension in addition to congestive heart failure and signs of
vascular congestion on physical examination. In addition to augmenting her furosemide dose for
volume removal, she should be placed on a mineralocorticoid receptor antagonist, such as
spironolactone.

Sacubitril/valsartan is a combination of a neprilysin inhibitor and an angiotensin II receptor

blocker; it is appropriate therapy for those with New York Heart Asociation (NYHA) functional
class II-IV and an LVEF ≤40%.

Carvedilol provides cardioprotection and is an excellent antihypertensive; however, her current

bradycardia precludes increasing her dosage.

Using a thiazide diuretic in combination with a loop diuretic (i.e., furosemide) has a higher risk of
renal toxicity and electrolyte abnormalities.

Doxazosin could be considered for additional afterload reduction; however, it has been shown
to be inferior to spironolactone in the treatment of resistant hypertension when added to a
three-drug regimen.

Answer : C
Key Point
The 2015 American Heart Association/American College of Cardiology (AHA/ACC) Scientific
Statement on Treatment of Hypertension in Patients with Coronary Artery Disease has
emphasized beta-blockers, angiotensin-converting enzyme inhibitors, angiotensin-receptor
blockers, calcium channel blockers, and diuretics as the most appropriate agents.

Question 2 of 35

A 52-year-old man is referred to you for management of hypertension. His medications include
hydrochlorothiazide 25 mg, lisinopril 40 mg, and amlodipine 10 mg, which he takes regularly. He
denies ethyl alcohol use. On examination, his body mass index is 40 kg/m2, heart rate is 50 bpm,
and blood pressure is 150/80 mm Hg on his right arm and 154/86 mm Hg on his left arm. You
review prior laboratory work obtained at the time of his initial diagnosis of hypertension. His
laboratory values at that time are significant for sodium 138 mg/dL, potassium 3.2 mmol/L,
blood urea nitrogen 14 mg/dL, creatinine 0.8 mg/dL, and thyroid stimulating hormone 3 uIU/mL.

Which of the following is most likely to identify the cause of his hypertension?

A. Random cortisol level.

B. Aldosterone/renin activity.

C. 24-hour urine for metanephrines.

D. Renal duplex ultrasound.

E. Abdominal computed tomography.

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The presence of hypokalemia at the time of diagnosis and difficult-to-control hypertension

should raise the suspicion of primary hyperaldosteronism, a common cause of secondary
hypertension. Therefore, he should undergo screening for hyperaldosteronism and the
recommended test is plasma aldosterone/renin ratio (Class I). Although all additional choices
may be appropriate in screening for secondary hypertension, the presence of hypokalemia
should focus the initial evaluation.

The diagnosis of primary aldosteronism generally requires a confirmatory test unless the plasma
aldosterone concentration is significantly elevated. If the patient undergoes surgery, then renal
sampling to localize origin is appropriate.

Answer :B
Key Point
Primary aldosteronism (PA), due to an aldosterone-secreting adrenal adenoma or adrenal
hyperplasia, presents with metabolic alkalosis, a potassium-losing diathesis (sometimes
leading to hypokalemia; ≤3.5 mEq/L) and hypertension. A plasma aldosterone
concentration/plasma renin activity (PAC/PRA) ratio ≥20 with a PAC of ≥12 ng/dL is
suggestive of PA, and a ratio ≥70 with a PAC of ≥15 ng/dL and a PRA ≤1 ng/mL/hour is
virtually diagnostic of PA. Treatment of PA is with a mineralocorticoid receptor antagonist
(spironolactone or epleronone). Surgery is rarely necessary.

Question 3 of 35

A 59-year-old woman with a history of hypertension and hyperlipidemia comes in for follow-up.
Her medications include losartan 100 mg daily, hydrochlorothiazide 25 mg daily, and carvedilol
25 mg twice daily, which she states she takes routinely. She denies any nonsteroidal anti-
inflammatory drug use. On examination, her height is 66 inches, weight is 150 lbs (body mass
index 24 kg/m2), blood pressure (BP) is 152/96 mm Hg, heart rate is 59 bpm, and physical
examination is unremarkable. Her 24-hour BP monitor mean is 142/88 mm Hg. Transthoracic
echocardiogram shows preserved biventricular function, mild left ventricular hypertrophy, and
no significant valvular abnormalities. Basic metabolic panel, thyroid stimulating hormone, and
urinalysis are unremarkable.

Her hypertension is classified as which of the following?

A. Refractory.

B. Essential.

C. Pseudoresistant.

D. Masked.

E. Resistant

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Resistant hypertension is defined as persistent hypertension despite the use of three

antihypertensive agents at or near maximal dose, of different classes including a diuretic if
possible. This patient has hypertension above goal (130/80 mm Hg) despite the maximal dose of
three antihypertensive drugs. Pseudoresistant is excluded because the 24-hour ambulatory
monitor verifies the elevated BPs. Nonadherence to medical therapy needs to be excluded. This
patient is likely compliant with her medications, as evidenced by her resting heart rate of 59
bpm. Masked hypertension is characterized by office readings suggesting normal BP but out-of-
office readings that are consistently above normal. This patient has elevated BPs in both
settings. Refractory hypertension is defined as hypertension not adequately controlled with five
antihypertensive medications, including chlorthalidone and a mineralocorticoid receptor
antagonist.

Answer :E
Key Point
Resistant hypertension is defined as a blood pressure of ≥130/80 mm Hg despite three drugs
of different classes at maximum approved doses, given ≥1 month to take effect.

Question 4 of 35

A 60-year-old white man presents to the hospital with recent complaints of dizziness and near-
syncope. He has a history of type 2 diabetes mellitus treated with insulin for the prior 20 years.

Orthostatic vital signs are performed. Supine vital signs after 5 minutes of rest are blood
pressure of 130/80 mm Hg and heart rate of 80 bpm. The patient stands and vital signs are
performed after 3 minutes of standing.

Which of the following standing vital signs are most indicative of a diagnosis of autonomic
neuropathy as a mechanism for this patient's near-syncope?

A. Standing vital signs: blood pressure 125/85 mm Hg, heart rate 100 bpm.

B. Standing vital signs: blood pressure 140/90 mm Hg, heart rate 110 bpm.

C. Standing vital signs: blood pressure 95/60 mm Hg, heart rate 80 bpm.

D. Standing vital signs: blood pressure 110/70 mm Hg, heart rate 40 bpm.

E. Standing vital signs: blood pressure 115/75 mm Hg, heart rate 130 bpm.

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Normal orthostatic vital sign changes in response to moving from a supine to standing position
include a minor drop in systolic blood pressure (5-10 mm Hg), increased diastolic blood pressure
(5-10 mm Hg), and increased heart rate (10-25 bpm). The hallmark of autonomic dysfunction is a
brief initial rise in heart rate with standing followed by a sustained fall in systolic (>20 mm Hg)
and diastolic (>10 mm Hg) blood pressure without a compensatory rise in heart rate. In the case
of a modest drop in blood pressure accompanied by an extreme rise in heart rate, the diagnosis
may be postural tachycardia syndrome. In the case of a marked drop in heart rate followed by a
drop in blood pressure, the diagnosis is cardiodepressor syncope.

Answer :C
Key Point
Significant orthostatic hypotension (OH) rarely occurs in patients with intact autonomic
reflexes. Vasovagal syncope (an autonomic reflex) may be triggered before OH ensues.

Question 5 of 35

A 60-year-old African American man presents to clinic to discuss cardiovascular risk reduction.
He has no significant past medical history and does not take any medications. He exercises
regularly and is a lifelong nonsmoker. He tries to minimize salt intake and eats a balanced diet.
He denies any exertional chest discomfort or shortness of breath. On examination, the average
of two blood pressure (BP) readings is 136/84 mm Hg. His BP was 133/85 mm Hg at his primary
care visit 2 months prior. His body mass index is 23 kg/m2. The remainder of his examination is
normal. Laboratory tests reveal total cholesterol 220 mg/dL and high-density lipoprotein 38
mg/dL. Renal function is normal. A urinalysis shows no evidence of proteinuria. His calculated
10-year atherosclerotic cardiovascular disease (ASCVD) risk score is 10.1%.

Which of the following medications would you recommend in this patient?

A. Irbesartan.

B. Carvedilol.

C. Chlorthalidone.

D. Spironolactone.

E. Hydralazine.

Per the 2017 multisociety high BP clinical practice guidelines, this man has stage 1 hypertension
(HTN; systolic BP 130-139 mm Hg or diastolic BP 80-89 mm Hg). For patients with stage 1 HTN
and clinical ASCVD or an estimated 10-year ASCVD risk score ≥10%, initiation of antihypertensive
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therapy along with nonpharmacological therapy is recommended. This patient adheres to a

healthy lifestyle. He may benefit from initiation of an antihypertensive agent along with a statin
for primary prevention of ASCVD.

With respect to choice of antihypertensive agent, in African Americans with HTN but without
heart failure or chronic kidney disease, including those with diabetes mellitus, initial
antihypertensive treatment should include a thiazide-type diuretic or calcium channel blocker.
The patient has a normal creatinine and no evidence of proteinuria on urinalysis to suggest
kidney disease. Chlorthalidone would be the best agent among the choices given.

Answer :C

Key Point

Most patients with hypertension require two or more antihypertensive medications to achieve
goal blood pressure (BP), namely <130/80 mm Hg in most; but <120/80 mm Hg is reasonable in
some individuals with coronary artery disease (CAD); with previous myocardial infarction,
stroke, or transient ischemic attack; or with CAD risk equivalents (carotid artery disease,
peripheral arterial disease, abdominal aortic aneurysm). Initiation of therapy with two agents (in
individual tablets or fixed-dose combination) is a reasonable strategy. In general, the two agents
should be any two of angiotensin-converting enzyme inhibitors or angiotensin-receptor
blockers, calcium channel blockers, and diuretics (especially chlorthalidone), with doses
uptitrated against the BP; the third agent can be added if necessary for BP control.

Question 6 of 35

A 54-year-old man with no known past medical history presents to the emergency department
with 30 minutes of crushing substernal chest discomfort. He does not seek regular medical care
and is on no medications. He smokes two packs of cigarettes daily.

On examination, he is pale and diaphoretic. Temperature is 99.4°F, heart rate 120 bpm, and
blood pressure (BP) is 80/60 mm Hg. He is intubated and mechanically ventilated. Jugular
venous pressure is 8 cm water, lungs have crackles half way up both lung fields, heart is regular
with an S3 gallop and a soft holosystolic murmur, and extremities are cool without edema.

An electrocardiogram is shown in Figure 1.

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In the emergency department, a pulmonary artery catheter is placed. Right atrial pressure is 12
mm Hg, pulmonary artery pressure 40/28 mm Hg, pulmonary capillary wedge pressure 32 mm
Hg, and cardiac index (CI) 1.5 L/min/m2. He is started on dopamine 5 mcg/kg/min and
norepinephrine 5 mcg/kg/min, resulting in a BP of 100/70 mm Hg and his CI is 2.1 L/min/m2.

An echocardiogram shows a nondilated left ventricle with anterior akinesis and ejection fraction
25%, as well as moderate mitral regurgitation.

Coronary angiography shows 100% stenosis of the proximal left anterior descending (LAD)
artery, 70% mid left circumflex artery stenosis, and 80% proximal right coronary artery stenosis.

What is the best next step?

A. Percutaneous coronary intervention to the left anterior descending artery.

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B. Tenecteplase 40 mg over 10 seconds.

C. Intra-aortic balloon pump placement.

D. Percutaneous transvalvular left ventricular assist device.

E. Percutaneous coronary intervention of all three coronary arteries.

This patient has an anterior ST-segment elevation myocardial infarction (STEMI) resulting in
cardiogenic shock. The treatment of choice in this situation is revascularization. Emergency
revascularization with either percutaneous coronary intervention (PCI) or coronary artery
bypass graft (CABG) is recommended in suitable patients with cardiogenic shock due to pump
failure after STEMI regardless of the time delay from myocardial infarction (MI) onset. This
patient has an acute proximal LAD occlusion that should be addressed with PCI. Thus, LAD PCI is
the correct answer.

In the setting of cardiogenic shock, only the culprit lesion should be addressed with PCI. The
CULPRIT-SHOCK (Culprit Lesion Only PCI Versus Multivessel PCI in Cardiogenic Shock) trial
demonstrated that, among patients with acute MI, cardiogenic shock, and multivessel coronary
artery disease, the risk of a composite of death from any cause or severe renal failure leading to
renal-replacement therapy at 30 days was found to be lower with PCI of the culprit lesion only
than with immediate multivessel PCI, and mortality did not differ significantly between the two
groups at 1 year of follow-up. Thus, only the LAD should be addressed and treatment of the
other diseased vessels delayed until the patient has stabilized.

Fibrinolytic therapy with tenectelplase may be considered for patients who are not candidates
for revascularization with PCI or CABG. Although there is some benefit of fibrinolysis compared
with placebo, PCI or CABG is superior compared with fibrinolysis.

Intra-aortic balloon pumps (IABPs) may be useful in patients with STEMI and cardiogenic shock,
but only in patients who do not respond to pharmacologic therapy. This patient has
improvement in hemodynamics with inotropic and vasopressor support, so IABP is not
indicated. Similarly, percutaneous left ventricular assist devices may be used but would not be
indicated in this setting, in which the hemodynamics responded to pharmacologic support.

Answer :A

Key Point
Patients with cardiogenic shock have hypotension, tachycardia, a low cardiac output, high

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systemic vascular resistance, and poor organ perfusion. Cardiogenic shock is usually caused
by a large myocardial infarction, acute valvular insufficiency, or an acute intracardiac shunt.

Question 7 of 35

A 45-year-old man without significant past medical history presents to the office to establish
care. He exercises regularly and takes his blood pressure using a machine located at the gym. His
blood pressure readings had been elevated over the prior 6 months. His family history is notable
for his father, who had a myocardial infarction at the age of 55. On examination, his blood
pressure is 150/96 mm Hg and heart rate is 58 bpm. His body mass index is 22 kg/m2. His
physical examination is otherwise normal.

Which of the following is the best next step in his care?

A. Electrocardiogram.

B. Renal duplex ultrasound.

C. Coronary artery calcium score.

D. Echocardiogram.

E. Polysomnogram.

The patient has newly diagnosed hypertension, which is likely essential hypertension. Secondary
hypertension is less likely based on his clinical history and normal physical examination. An
electrocardiogram is recommended in patients with primary hypertension to assess for other
conditions such as left ventricular hypertrophy, rhythm disturbances, and myocardial infarction.
Other additional basic tests are outlined in Figure 1.

Coronary artery calcium scores can be helpful in risk stratification for lipid management, but is
not indicated as routine screening in patients with hypertension.

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Without evidence of hypertensive heart disease, routine echocardiography in patients with

hypertension is not indicated.

Renal duplex Doppler ultrasound should only be considered in patients with suspected
renovascular disease (e.g., resistant hypertension, hypertension of abrupt onset or worsening or
increasingly difficult to control, flash pulmonary edema, early onset hypertension, abdominal
systolic-diastolic bruit).

Polysomnogram is indicated for patients with difficult-to-control hypertension, but would not be
first-line testing for the new diagnosis of hypertension.

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Answer :A
Key Point
Laboratory evaluation should be tailored to the same objectives, and should include at least
serum creatinine, blood urea nitrogen, estimated glomerular filtration rate, potassium,
sodium, fasting glucose and lipid profile, urinalysis, and measurement of the
albumin/creatinine ratio in a spot urine sample.

Question 8 of 35

A 40-year-old woman without previous cardiovascular disease presents to urgent care with a 2-
month history of intermittent pulsating headaches. She denies associated symptoms such as
visual changes or focal neurological deficits. Since the birth of her children, she has not had
routine health care and currently takes no medications. She denies tobacco or illicit drug use. On
examination, her heart rate is 62 bpm and blood pressure is 170/92 mm Hg; her physical
examination is otherwise normal. Laboratory studies are significant for normal creatinine and
potassium levels. A renal artery ultrasound demonstrates increased velocity on the right with a
slight reduction in the size of the right kidney. An abdominal computed tomography (CT)
angiogram is performed (Figure 1).

Which of the following is the best next step in this patient's management?

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Abdominal computed tomography angiogram.

Reproduced with permission from Vascular: Case Study 6,915 (CTisus website). 2016. Available
at: http://www.ctisus.com/redesign/teachingfiles/vascular/368580. Accessed 04/03/2019.

A. Renal angiography.

B. Renal artery stenting.

C. Renal scintigraphy.

D. Catheter renal denervation.

E. Antihypertensive therapy.

This patient has secondary hypertension due to renal artery stenosis from fibromuscular
dysplasia (FMD). Figure 1 shows an abdominal CT angiogram with the characteristic “string of
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beads” sign associated with media fibroplasia, the most common form of FMD (80-90% of
cases). FMD is a noninflammatory vascular disease typically discovered in younger women that
predominately affects the renal arteries, but can also involve other vascular beds such as the
carotids. Although the prevalence of FMD is not known, screening of kidney donors suggests it is
found in <3% in the general population. Most cases of renal artery stenosis are secondary to
atherosclerosis, and renal artery stenosis secondary to FMD accounts for <10% of cases. First-
line therapy for patients with FMD-related renal artery stenosis is antihypertensive medical
therapy, typically requiring multiple agents that include an angiotensin-converting enzyme
inhibitor or angiotensin-receptor blocker. For cases of resistant hypertension despite optimal
medical treatment, percutaneous balloon angioplasty can be considered. The role of renal artery
stenting is less well defined in this population. Additional testing with renal scintigraphy or X-ray
angiography, the gold standard for detecting renal artery stenosis, would not be indicated at
present. A role for catheter-based renal denervation in FMD has not been established.

Answer :E
Key Point
Renal artery stenosis (RAS) causes hypertension and progressive renal decompensation. It
can be atherosclerotic (the common form in elderly individuals) or due to fibromuscular
dysplasia (in younger persons, particularly women). Treatment of fibromuscular dysplasia is
renal angioplasty and stenting. Treatment of atherosclerotic RAS is more complex;
aggressive medical management of the hypertension is usually the treatment of choice,
since revascularization procedures often fail to control blood pressure (BP), because the
hypertension is sustained by renal parenchymal damage. If there is rapidly worsening renal
function in spite of good BP control, then revascularization is an option.

Question 9 of 35

A 35-year-old woman presents to your office for management of heart failure (HF). She had
been diagnosed with an idiopathic dilated cardiomyopathy 2 years prior. She had been admitted
once in the prior year for decompensated HF and, at the time of presentation, has shortness of
breath with walking up half a flight of stairs, two-pillow orthopnea, and occasional paroxysmal
nocturnal dyspnea. Her medications include sacubitril/valsartan 24/26 mg twice daily,
metoprolol XL 25 mg daily, spironolactone 12.5 mg daily, and furosemide 80 mg daily.

On examination, she is 5'2" and 130 lbs. Her heart rate is 108 bpm and blood pressure is 86/68
mm Hg. Jugular venous pressure is 14 cm water. She has a regular rhythm with S3 gallop. Her
abdomen is soft and nontender. Her extremities are cool with 1+ edema.

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Laboratories reveal sodium 130 mEq/L, potassium 5.1 mEq/L, and creatinine 1.6 mg/dL (baseline
1.1 mg/dL).

What is the best next step in her management?

A. Add metolazone.

B. Stop spironolactone.

C. Start intravenous furosemide.

D. Stop sacubitril/valsartan.

E. Start midodrine.

This patient has evidence of decompensated HF with worsening symptoms and evidence of
volume overload on examination. The concerning prognostic findings are low blood pressure
along with renal dysfunction, evidence of poor perfusion related to the hypotension. This
constellation of findings indicates "wet and cold" HF and merits inpatient admission.

The American College of Cardiology/American Heart Association (ACC/AHA) guidelines

recommend that patients with heart failure with reduced ejection fraction (HFrEF) experiencing
a symptomatic exacerbation of HF requiring hospitalization during chronic maintenance receive
treatment with guideline-directed medical therapy (GDMT); it is recommended that GDMT be
continued in the absence of hemodynamic instability or contraindications. Whereas one may
consider stopping sacubitril/valsartan and spironolactone due to renal dysfunction, these alone
are not the best answer because the most important intervention would be admission for
inpatient diuresis and monitoring of renal function.

The combination of hypotension and worsening renal function suggests that an increase in oral
diuretics alone will be inadequate to treat decompensated HF in this setting and that this is not
the best approach.

Midodrine, an alpha-1 agonist to increase blood pressure, is not recommended in HF because an

increase in systemic vascular resistance can worsen cardiac output in HFrEF patients. As the
blood pressure is low because of a reduced cardiac output, measures to increase cardiac output
(indirectly, via diuresis) will be more effective and measures to increase systemic vascular
resistance ultimately maladaptive.

Answer :C
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Key Point
Heart failure is a common comorbidity with orthostatic hypotension. Low cardiac output is an
important cause of hypotension in patients with heart failure.

Question 10 of 35

A 53-year-old woman with a history of hypertension, osteoporosis, and obesity presents for a
follow-up appointment. She complains of fatigue and occasional ankle swelling during hot
weather. She denies headaches, visual changes, shortness of breath, or chest pain. Her daily
medications include amlodipine 10 mg, hydrochlorothiazide 25 mg, lisinopril 40 mg, pravastatin
20 mg, and calcium carbonate 600 mg. On examination, her heart rate is 68 bpm, blood
pressure is 149/93 mm Hg, and body mass index is 34.5 kg/m2. She is well appearing. A cardiac
examination reveals normal S1 and S2 without murmurs. Lungs are clear. Abdomen is obese
without bruits. Extremities have 2+ posterior tibial pulses and are without edema. Laboratory
values include normal renal function and electrolytes.

Which of the following is the best next step in her care?

A. Add spironolactone 12.5 mg daily.

B. Increase hydrochlorothiazide to 50 mg daily.

C. Change lisinopril to losartan 100 mg daily.

D. Add metoprolol succinate 50 mg daily.

E. Stop supplemental calcium.

The addition of a mineralocorticoid receptor antagonist (MRA) has been shown to be very
effective in lowering blood pressure. In this patient, who is on adequate doses of three different
agents including a diuretic, the addition of spironolactone or eplerenone as add-on therapy is
appropriate. Studies have shown that the addition of an MRA reduces systolic blood pressure by
24 mm Hg at 6 months. Beta-blockers (metoprolol succinate) are not effective antihypertensive
medications and therefore would not be expected to have a significant impact on her resistant
hypertension. Changing lisinopril to losartan is indicated for patients with a cough attributed to
angiotensin-converting enzyme inhibitors, but would not necessarily provide better blood
pressure control. In patients with normal renal function, there is little added benefit in

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increasing hydrochlorothiazide beyond 25 mg daily. Calcium supplements do not cause

secondary hypertension and, given her osteoporosis, she should continue this therapy.

Answer :A
Key Point
Most patients with hypertension require two or more antihypertensive medications to achieve
goal blood pressure (BP), namely <130/80 mm Hg in most; but <120/80 mm Hg is
reasonable in some individuals with coronary artery disease (CAD); with previous myocardial
infarction, stroke, or transient ischemic attack; or with CAD risk equivalents (carotid artery
disease, peripheral arterial disease, abdominal aortic aneurysm). Initiation of therapy with
two agents (in individual tablets or fixed-dose combination) is a reasonable strategy. In
general, the two agents should be any two of angiotensin-converting enzyme inhibitors or
angiotensin-receptor blockers, calcium channel blockers, and diuretics (especially
chlorthalidone), with doses uptitrated against the BP; the third agent can be added if
necessary for BP control.

Question 11 of 35

A 50-year-old man presents to your office for a second opinion regarding management of
hypertension. He had seen his internist for a routine physical 1 month prior and reports that his
blood pressure (BP) was "high" and his internist recommended that he "start some pills," which
the patient prefers to avoid. He denies any symptoms. He has no medical problems and takes
only a daily multivitamin. He does not exercise regularly. He does not smoke cigarettes.

On examination, he is 5'10" tall and weighs 200 lbs with body mass index 28.7 kg/m2. His heart
rate is 88 bpm and BP is 154/92 mm Hg. Lungs are clear and heart is regular with no murmurs or
gallops. Abdomen is soft without bruits. Extremities are warm without edema.

Laboratories show sodium 140 mmol/L, potassium 4.0 mEq/L, and creatinine 0.8 mg/dL. A
fasting lipid profile shows cholesterol 200, high-density lipoprotein 50, low-density lipoprotein
130, and triglycerides 100. His 10-year risk of atherosclerotic cardiovascular disease (ASCVD)
calculated by the ASCVD pooled cohort risk assessment equations is 4.8%.

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In addition to recommending the DASH (Dietary Approaches to Stop Hypertension) diet and
daily exercise, what is the best next step?

A. Start lisinopril 10 mg daily and chlorthalidone 12.5 mg daily.

B. Start metoprolol XL 50 mg daily and chlorthalidone 12.5 mg daily.

C. Start chlorthalidone 25 mg daily.

D. Reassess blood pressure in 3 months.

E. Start amlodipine 10 mg daily.

The 2017 multisociety guidelines on BP management define normal BP as <120/80 mm Hg,

elevated BP as 120-129/<80 mm Hg, stage 1 hypertension as 130-139/80-89 mm Hg, and stage 2
hypertension as ≥140/90 mm Hg. Stage 2 hypertension merits nonpharmacological therapy
(DASH diet, sodium restriction, weight loss, exercise, avoidance of alcohol) along with BP-
lowering medication (Class I recommendation) with reassessment in 1 month to determine
whether the BP goal has been met.

Initiation of antihypertensive drug therapy with two first-line agents of different classes, either
as separate agents or in a fixed-dose combination, is recommended in adults with stage 2
hypertension or an average BP >20/10 mm Hg above their BP target (Class I). Drug regimens
with complementary activity (in which a second antihypertensive agent is used to block
compensatory responses to the initial agent or affect a different pressor mechanism) can result
in additive lowering of BP. For example, thiazide diuretics may stimulate the renin-angiotensin-
aldosterone system. By adding an angiotensin-converting enzyme inhibitor or angiotensin-
receptor blocker to the thiazide, an additive BP-lowering effect may be obtained.

Initiation of lisinopril and hydrochlorothiazide is the correct answer. Initiation of

antihypertensive drug therapy with a single antihypertensive drug is reasonable in adults with
stage 1 hypertension and BP goal <130/80 mm Hg with dosage titration and sequential addition
of other agents to achieve the BP target (Class IIa). This patient has stage 2 hypertension, so
initiation of a single agent (chlorthalidone or amlodipine) is not the best answer.

In addition, beta-blockers are not recommended as first-line agents for BP management unless
the patient has ischemic heart disease or heart failure.

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Patients with normal BP, <120/80 mm Hg, require only promotion of optimal lifestyle habits and
can be re-evaluated in 1 year. Re-evaluation in 3 months is not correct for this man with stage 2
hypertension.

Answer :A
Key Point
Most patients with hypertension require two or more antihypertensive medications to achieve
goal blood pressure (BP), namely <130/80 mm Hg in most; but <120/80 mm Hg is
reasonable in some individuals with coronary artery disease (CAD); with previous myocardial
infarction, stroke, or transient ischemic attack; or with CAD risk equivalents (carotid artery
disease, peripheral arterial disease, abdominal aortic aneurysm). Initiation of therapy with
two agents (in individual tablets or fixed-dose combination) is a reasonable strategy. In
general, the two agents should be any two of angiotensin-converting enzyme inhibitors or
angiotensin-receptor blockers, calcium channel blockers, and diuretics (especially
chlorthalidone), with doses uptitrated against the BP; the third agent can be added if
necessary for BP control.

Question 12 of 35

A 58-year-old man is referred to your clinic for treatment of hypertension. He has no complaints
and no other chronic medical problems. He takes no medications and has no known drug
allergies. His vital signs are blood pressure 138/90 mm Hg, pulse 88 bpm, and respirations 12
per minute. A physical examination includes normal heart sounds, no carotid or abdominal
bruits, and normal peripheral pulses. There is no lower extremity edema.

Laboratory evaluations include sodium 139 mEq/L, potassium 3.7 mEq/L, creatinine 1.2 mg/dL,
blood urea nitrogen 20 mg/dL, and random glucose 111 mg/dL. His total cholesterol is 218
mg/dL, triglycerides 189 mg/dL, high-density lipoprotein cholesterol 33 mg/dL, and low-density
lipoprotein cholesterol 147 mg/dL. A spot albumin:creatinine ratio is 350 mg/g.

Which of the following is the most appropriate antihypertensive drug to start?

A. Diltiazem.

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B. Spironolactone.

C. Lisinopril.

D. Chlorthalidone.

E. Labetalol.

Proteinuria, both microalbuminuria (spot urine albumin:creatinine ratio 30-300 mg/g) and
macroalbuminuria (spot urine albumin:creatinine ratio >300 mg/g), is associated with both
chronic kidney disease and increased cardiovascular risk in patients with and without diabetes
mellitus.

An angiotensin-converting enzyme (ACE) inhibitor (or an angiotensin-receptor blocker [ARB] in

case of ACE inhibitor intolerance) is a preferred drug for treatment of hypertension if
albuminuria (≥300 mg/day or ≥300 mg/g creatinine by first morning void) is present, although
the evidence is mixed (Figure 1).

If this patient with hypertension had normal renal function without proteinuria, then calcium
channel blockers, diuretics, ACE inhibitors, or ARBs would all be reasonable first-line choices for
treatment of hypertension.

In the course of reducing intraglomerular pressure and thereby reducing albuminuria, ACE
inhibitors or ARBs may cause serum creatinine to increase ≤30% because of concurrent
reduction in glomerular filtration rate (GFR). Further GFR decline should be investigated and
may be related to other factors, including volume contraction, use of nephrotoxic agents, or
renovascular disease.

Beta-blockers should not be used as monotherapy for hypertension.

Answer :C

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Key Point
In diabetes mellitus and chronic kidney disease, angiotensin-converting enzyme inhibitors
and angiotensin-receptor blockers are essential components of the management of
hypertension because they slow the rate of decline of renal function in diabetic nephropathy,
and may do so in hypertensive nephropathy.

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Question 13 of 35

A 58-year-old man with paroxysmal atrial fibrillation and hyperlipidemia presents to the
cardiology clinic to establish care. He needs refills on his medications but denies any acute
issues or concerns. He exercises regularly by walking his dogs 2 miles per day and has no
difficulties walking at a "brisk" pace.

His medications include aspirin 81 mg daily, atorvastatin 80 mg daily, and metoprolol succinate
25 mg daily.

On examination, he is tall and fit appearing (body mass index: 24 kg/m2). Heart rate is 72 bpm
and blood pressure (BP) is 142/92 mm Hg. He states that his BP is "always high at the doctor's
office" but is generally well controlled outside of the office. He had recently checked his BP at
his local pharmacy and his BP was 120/78 mm Hg.

What is the best next step?

A. Metoprolol 50 mg daily.

B. Nocturnal polysomnography.

C. Amlodipine 5 mg daily.

D. Continue current management.

E. Ambulatory blood pressure monitor.

The 2017 multisociety guidelines on the evaluation and management of high BP recommend
either ambulatory home blood pressure monitoring (ABPM) or home blood pressure monitoring
(HBPM) to screen for the presence of "white-coat hypertension." White-coat hypertension is
defined as elevated office readings but normal BP readings outside of the office with either
ABPM or HBPM.

The prevalence of white-coat hypertension is estimated to be between 13-35%. This condition is

relevant because it confers an elevated risk for cardiovascular disease and all-cause mortality, so
the diagnostic evaluation should be pursued and the patient should receive extensive
counselling on lifestyle modifications.

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For patients not on drug therapy with an office BP >130/80 mm Hg but <160/100 mm Hg after 3
months of lifestyle modifications, it is recommended to proceed with ABPM or HPBM to
establish a diagnosis of either white-coat hypertension or hypertension. ABPM is preferred, but
when this is not available, HBPM is a reasonable alternative.

Starting an antihypertensive medication or increasing his beta-blocker are not correct at this
stage of the evaluation. If the APBM supports a diagnosis of hypertension, then medical therapy
would be appropriate.

Sleep apnea is an important secondary cause of hypertension, which should be considered in

the appropriate clinical context. However, this patient does not yet have a diagnosis of
hypertension or any symptoms of sleep apnea.

Answer :E
Key Point
White coat effect is the situation where the clinic BP is higher than the BP at home in a
patient with hypertension. White coat hypertension is where the clinic BP is elevated but is
normal at home in a patient without prior diagnosis of hypertension.

Question 14 of 35

A 46-year-old man is referred to your clinic for management of his resistant hypertension. He
feels well overall, but does note occasional lightheadedness. He leads an active lifestyle and
denies chest pain or shortness of breath. His medications at the time of presentation include
amlodipine 10 mg daily, carvedilol 12.5 mg twice a day, chlorthalidone 12.5 mg daily, and
lisinopril 40 mg daily.

A physical examination is notable for pulse 58 bpm and blood pressure (BP) 156/94 mm Hg on
the right and 152/90 mm Hg on the left. Body mass index is 27 kg/m2. A cardiac examination
reveals normal S1 and a prominent S2 sound with no murmurs. Lungs are clear. Abdomen is soft
without organomegaly or bruits. There are 2+ pulses in the upper and lower extremities.

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An electrocardiogram (ECG) reveals sinus bradycardia. An echocardiogram reveals normal

biventricular size and function. There is no ventricular hypertrophy or hemodynamically
significant valve disease. Basic metabolic panel is normal.

In addition to reinforcing lifestyle modifications, which of the following is the best next step in
the management of this patient?

A. Exercise treadmill stress test.

B. Carotid duplex.

C. Chest computed tomography angiography.

D. Low-potassium diet.

E. Home blood pressure monitoring.

Resistant hypertension is defined as persistently elevated BPs despite a three-drug regimen of

different classes, one of which should be a diuretic. Given this patient's lightheadedness and
lack of left ventricular hypertrophy (LVH) on echocardiogram or ECG, it is unclear whether his
elevated office BPs are reflective of his home BPs. Therefore, additional information such as 24-
hour BP monitoring to exclude pseudoresistant hypertension is indicated. An ambulatory BP
monitor will yield a more accurate representation of his BP (Figure 1).

Computed tomography angiography can be used to evaluate for aortic coarctation, which is a
rare cause of secondary hypertension. In this patient, his symmetric BP, lack of LVH, and lack of
a pressure gradient in the descending aorta on echocardiogram make this an unlikely diagnosis.

Nonpharmacologic interventions to reduce BP include a diet rich in high-potassium foods;

therefore, a low-potassium diet is incorrect.

An exercise treadmill stress test is not indicated in this asymptomatic patient.

The patient has no transient ischemic attack or stroke-like symptoms to suggest cerebral
vascular disease; therefore, a carotid duplex is not indicated.

Answer :E
Key Point
Resistant hypertension is defined as a blood pressure of ≥130/80 mm Hg despite three drugs
of different classes at maximum approved doses, given ≥1 month to take effect.
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Question 15 of 35

You are seeing a 78-year-old man for follow-up of New York Heart Association (NYHA) class III,
American College of Cardiology/American Heart Association (ACC/AHA) stage D heart failure. He
has no dyspnea, orthopnea, or chest pain. However, he says that every time he stands up from a
sitting position, he feels "woozy." His past medical history includes coronary disease status/post
myocardial infarction, systolic heart failure with left ventricular ejection fraction 35%,
hypertension, and depression. His current medications include aspirin 81 mg daily, atorvastatin
80 mg daily, ramipril 10 mg daily, furosemide 40 mg daily, spironolactone 25 mg daily,
metoprolol 100 mg extended release daily, and venlafaxine 75 mg extended release daily.

His vital signs are blood pressure 100/60 mm Hg sitting and 82/50 mm Hg standing, pulse 80
bpm sitting and 92 bpm standing, and respirations 14 per minute. A physical examination
reveals flat neck veins. Cardiac auscultation reveals normal S1 and S2 with no extra heart
sounds. The lungs are clear. The extremities are warm with no edema.

What is the best next step in his management?

A. Stop venlafaxine.

B. Add midodrine.

C. Add fludorcortisone.

D. Add droxidopa.

E. Decrease furosemide dose.

This patient with systolic heart failure has orthostatic hypotension, likely due to side effects of
heart failure medications, including a diuretic. The first step in treatment of orthostatic
hypotension in this setting is to reduce the dose of offending agents, which often involves
trading off appropriate doses of heart failure medications versus quality of life. Diuretics in
particular may need to be used judiciously, with some degree of permissive hypervolemia to
avoid falls from orthostasis.

Both midodrine and droxidopa are alpha agonists that can mitigate symptomatic orthostasis,
but because of their potential side effect of supine hypertension that can exacerbate heart
failure, they should only be added after reducing doses of drugs known to cause
hypotension.

Fludrocortisone can cause sodium retention and long-term adverse effects in patients with
heart failure and should therefore be avoided.
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Selective serotonin-norepinephrine reuptake inhibitors such as venlafaxine have been

reported to cause hypertension, not hypotension, so stopping venlafaxine is not the preferred
answer.

Answer :E
Key Point
Droxidopa, which has both alpha and beta agonism, appears to cause less heart failure
exacerbations than the pure alpha agonist midodrine.

Question 16 of 35

A 39-year-old African American man is referred to your clinic for poorly controlled hypertension.
His blood pressure (BP) on ambulatory readings is 150/90 mm Hg. He takes hydrochlorothiazide
25 mg once a day. His vital signs show temperature 98.9°F, BP 149/95 mm Hg, and heart rate 90
bpm. A cardiac examination reveals normal S1 and S2 with soft systolic murmur.

His laboratory data show sodium 135 mg/dL, potassium 3.9 mg/dL, and creatinine 1.1.

In addition to discussion about lifestyle modification, what is your best next step?

A. Switch to spironolactone 25 mg daily.

B. Add amlodipine 10 mg daily.

C. Increase hydrochlorothiazide to 50 mg daily.

D. Start lisinopril 20 mg daily.

E. Start isosorbide dinitrate 20 mg with hydralazine 37.5 mg three times daily.

The patient has BP above goal and requires additional antihypertensive therapy.

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In African Americans, thiazide diuretics or calcium channel blockers are more effective in
lowering BP than are renin-angiotensin system (RAS) inhibitors or beta-blockers, as well as more
effective in reducing cardiovascular events than are RAS inhibitors or alpha-blockers.

Optimal treatment of hypertension often requires multiple drugs. Combination therapy is often
associated with fewer side effects compared with uptitration of a single agent. Increasing
hydrochlorothiazide to 50 mg daily would likely not be the best option. Isosorbide/hydralazine
combination therapy is adjunctive therapy for heart failure treatment in African Americans
(which this patient does not have).

Answer :B
Key Point
Most patients with hypertension require two or more antihypertensive medications to achieve
goal blood pressure (BP), namely <130/80 mm Hg in most; but <120/80 mm Hg is
reasonable in some individuals with coronary artery disease (CAD); with previous myocardial
infarction, stroke, or transient ischemic attack; or with CAD risk equivalents (carotid artery
disease, peripheral arterial disease, abdominal aortic aneurysm). Initiation of therapy with
two agents (in individual tablets or fixed-dose combination) is a reasonable strategy. In
general, the two agents should be any two of angiotensin-converting enzyme inhibitors or
angiotensin-receptor blockers, calcium channel blockers, and diuretics (especially
chlorthalidone), with doses uptitrated against the BP; the third agent can be added if
necessary for BP control.

Question 17 of 35

A 74-year-old man is seen in the cardiology clinic for a follow-up visit. He denies any chest pain
or shortness of breath. However, he does note several months of significant lightheadedness
when standing up and has almost fallen twice. He has a past medical history of coronary artery
disease with a stent placed 6 years prior, hypertension, hyperlipidemia, benign prostatic
hyperplasia, and gout. His medications are aspirin 81 mg, atorvastatin 80 mg, lisinopril 10 mg,
metoprolol succinate 50 mg, terazosin 2 mg, and allopurinol 100 mg; he takes all these
medications daily in the morning. His blood pressure is 126/76 mm Hg with heart rate of 62
bpm. Upon standing, his blood pressure is 101/68 mm Hg. The remainder of his examination is
unremarkable.

Which of the following medications is most likely contributing to his symptoms?

A. Atorvastatin.

B. Lisinopril.
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C. Allopurinol.

D. Metoprolol.

E. Terazosin.

The patient is presenting with lightheadedness when standing, which is concerning for
orthostatic (or postural) hypotension. His systolic blood pressure drops >20 mm Hg with
standing, which is consistent with orthostatic hypotension. Whereas orthostatic hypotension
becomes more common as people age, it is important to evaluate for any medications with
sympatholytic side effects that can contribute to symptoms. Alpha-blocker medications used
to treat prostate conditions, especially terazosin specifically, can contribute to orthostatic
hypotension.

Scheduling alpha-blocking medications at bedtime may mitigate this side effect. Other
medications include tizanidine (muscle relaxant), trazodone (and other
antipsychotic/antidepressant medications), and carvedilol (with its alpha-blocking properties).

In general, beta-blockers and angiotensin-converting enzyme inhibitors are not as frequently

associated with orthostatic hypotension in patients with normal left ventricular function.
Statins and allopurinol are not associated with orthostatic hypotension.

Answer :E
Key Point
Commonly used medications (hidden sympatholytics) can worsen orthostatic hypotension
(tizanidine, trazodone, tamsulosin). Carvedilol should be used with caution in patients with
orthostatic hypotension because of its alpha-blocking actions.

Question 18 of 35

Higher systolic blood pressure (SBP) and diastolic blood pressure (DBP) are associated with
increased risk for angina, myocardial infarction, heart failure, stroke, peripheral arterial disease,
and abdominal aortic aneurysm.

Which of the following best describes the statistical model for the relationship between
hypertension and cardiovascular risk?

A. Inverse linear.

B. Exponential.

C. Bimodal.
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D. Deterministic.

E. Log-linear.

There is a log-linear relationship between hypertension and cardiovascular disease risk. A 20

mm Hg higher SBP and 10 mm Hg higher DBP are each associated with a doubling in the
risk of death from stroke, heart disease, or other vascular disease. "Log-linear" describes the
finding that incremental or categorical increases in blood pressure (BP) are associated with
doubling of risk. There is no apparent threshold for this effect.

An exponential relationship would mean that cardiovascular risk doubles with every single-
point increase in BP, which is not true.

Inverse linear relationships exist when increase in one variable is associated with decrease
in the other variable, which is not true of hypertension and cardiovascular risk.

A deterministic relationship would imply that everyone with hypertension, regardless of

severity, is certain to have myocardial infarction, stroke, and heart failure.

A bimodal relationship implies that risk increases or decreases in a different manner within
two categories of BPs, which is not true.

Answer :E
Key Point
The risk of dying from stroke or ischemic heart disease at all ages doubles for each
increment of 20/10 mm Hg of blood pressure, starting as low as 115/75 mm Hg.

Question 19 of 35

An 82-year-old woman is evaluated in clinic for generalized fatigue and malaise after returning
from a cruise. She denies headache, chest pain, shortness of breath, or ankle swelling. Her past
medical history is significant for hypertension, polymyalgia rheumatica, and dyslipidemia. Her
medications include aspirin 81 mg, losartan 50 mg, amlodipine 5 mg, chlorthalidone 25 mg, and
prednisone 2 mg per day.

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Physical examination reveals an 82-year-old woman in mild distress. Her heart rate is 82 bpm
and blood pressure is 182/125 mm Hg on the right and 180/122 mm Hg on the left. Repeat
blood pressures in 5 minutes are unchanged. Respiratory rate is 18 breaths per minute and
oxygen saturation is 90% on room air. A cardiovascular examination reveals normal S1 and S2, as
well as S4 with a soft ejection systolic murmur. Jugular venous pressure is not visible due to
body habitus. There is 1+ lower extremity edema with palpable pedal pulses. Twelve-lead
electrocardiogram reveals sinus rhythm with left ventricular hypertrophy.

What is the best next step in this patient's management?

A. Increase amlodipine dose to 5 mg per day, re-evaluate in 2 days.

B. Increase chlorthalidone to 50 mg per day and amlodipine to 10 mg per day, re-evaluate

in 2 days.

C. Outpatient basic metabolic profile then start furosemide 40 mg daily, re-evaluate in 2

days.

D. Transfer to the emergency department for further evaluation and treatment.

E. Obtain outpatient basic metabolic profile, add metoprolol succinate 50 mg daily.

This patient's presentation is consistent with hypertensive crisis with a diastolic blood pressure
>120 mm Hg. Hypertensive emergency is defined when there is evidence of end-organ damage
such as encephalopathy, pulmonary edema, or renal dysfunction. This patient has evidence of
heart failure with decreased oxygen saturation and volume overload. She should be admitted
for acute blood pressure lowering, so transfer to the emergency department is appropriate.

Advancing her oral antihypertensive regimen is not adequate in management of hypertensive

emergency. Therefore, each of the outpatient medication regimens is incorrect in the setting of
hypertensive emergency.

Answer :D
Key Point
A patient with hypertensive emergency should be admitted to the hospital for immediate
antihypertensive therapy, often parenteral, with continuous blood pressure monitoring. In
contrast, a patient with hypertensive urgency should be managed aggressively with oral
antihypertensive medications in the outpatient environment.

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Question 20 of 35

A 77-year-old woman presents to the emergency department with dyspnea. She had had acute
onset of shortness of breath earlier in the day with a cough productive of scant clear sputum.
Her past medical history includes chronic obstructive pulmonary disease, paroxysmal
supraventricular tachycardia, nonobstructive coronary artery disease, and hypertension. She is
compliant with her medications, which include aspirin 81 mg daily, atorvastatin 40 mg daily,
lisinopril 20 mg daily, metoprolol tartrate 50 mg twice daily, and several inhalers.

On physical examination, her heart rate is 90 bpm and regular, blood pressure 166/94 mm Hg,
respiratory rate 24 breaths/min, oxygen saturation 92% on 6 L nasal cannula, and temperature
99.5°F. Her body mass index is 38.2 kg/m2. Her jugular venous pressure is difficult to assess due
to body habitus, lung sounds are diminished with mild wheezing, cardiac examination is regular
with soft systolic murmur at the apex, and extremities are warm with trace bilateral pretibial
edema.

An electrocardiogram reveals sinus tachycardia and left ventricular hypertrophy, with

nonspecific ST-T changes in the inferolateral leads. Laboratories return with a normal white
blood cell count and creatinine, troponin T 0.05 ng/mL, and NT-proBNP 185 ng/mL. A chest X-
ray demonstrates hyperexpanded lung fields with bilateral alveolar infiltrates. An
echocardiogram demonstrates a left ventricular ejection fraction of 55%.

Which of the following is the most likely cause of her acute shortness of breath?

A. Pulmonary embolism.

B. Heart failure with preserved ejection fraction.

C. Pneumonia.

D. Supraventricular tachycardia.

E. Vocal cord dysfunction.

This patient has developed acute pulmonary edema related to decompensated heart failure
with preserved ejection fraction, likely secondary to uncontrolled hypertension. A pulmonary
embolism would likely present with a clear chest X-ray and hypoxemia. Pneumonia would
typically have a fever and elevated white blood cell count, as well as a subacute
development of symptoms. There is no evidence of supraventricular tachycardia at the time

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of presentation. Vocal cord dysfunction is most commonly seen in patients with recent
intubation and may present as stridor without chest X-ray abnormalities.

Answer :B
Key Point
Hypertension is a major cause of coronary artery disease, myocardial ischemia, left
ventricular hypertrophy, atrial fibrillation, acute coronary syndrome, and heart failure.

Question 21 of 35

A 60-year-old man presents to the emergency department with shortness of breath and altered
mental status for the prior week. His past medical history includes coronary artery disease,
status/post coronary artery bypass grafting, active tobacco use, and diabetes mellitus.

Home medications include metoprolol succinate 25 mg daily, lisinopril 10 mg daily, aspirin 81

mg daily, and atorvastatin 40 mg at bedtime.

On examination, he appears lethargic. His vital signs are temperature 99°F, blood pressure
100/70 mm Hg with heart rate 130 bpm in atrial fibrillation, and oxygen saturation 88% on room
air. Jugular venous pressure is found to be elevated with bilateral crackles on chest examination
and cold edematous extremities.

White blood cell count, troponin, and liver function tests are elevated. An echocardiogram
reveals a severely depressed left ventricular systolic function with an ejection fraction of 20%.
There are no valvular vegetations.

Which of the following is the most likely finding on right heart catheterization?

A. Right atrium 5 mm Hg, pulmonary artery 26/11 mm Hg, pulmonary capillary wedge
pressure 10 mm Hg.

B. Right atrium 14 mm Hg, pulmonary artery 67/32 mm Hg, pulmonary capillary wedge
pressure 14 mm Hg.

C. Right atrium 15 mm Hg, pulmonary artery 40/15 mm Hg, pulmonary capillary wedge
pressure 15 mm Hg.

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D. Right atrium 12 mm Hg, pulmonary artery 50/20 mm Hg, pulmonary capillary wedge
pressure 21 mm Hg.

E. Right atrium 9 mm Hg, pulmonary artery 30/10 mm Hg, pulmonary capillary wedge
pressure 27 mm Hg.

Cardiogenic shock is a high-acuity, potentially complex, and hemodynamically diverse state

of end-organ hypoperfusion that is frequently associated with multisystem organ failure.
Despite improving survival in recent years, patient morbidity and mortality remain high, and
there are few evidence-based therapeutic interventions known to clearly improve patient
outcomes.

The hallmark of cardiogenic shock due to left-sided failure is elevated pulmonary capillary
wedge pressure (PCWP) in association with low cardiac output and right-sided congestion.
The corresponding findings on examination of peripheral and pulmonary edema reflect
congestion and altered mental status; cold extremities reflect hypoperfusion.

Right atrium (RA) 12 mm Hg (elevated), pulmonary artery (PA) 50/20 mm Hg (elevated),

PCWP 21 mm Hg (elevated): This is the correct answer.

RA 5 mm Hg (normal), PA 26/11 mm Hg (normal), PCWP 10 mm Hg (normal): These are

normal values.

RA 14 mm Hg (elevated), PA 67/32 mm Hg (elevated), PCWP 14 mm Hg (normal): These

are findings suggestive of pulmonary hypertension.

RA 15 mm Hg, PA 40/15 mm Hg, PCWP 15 mm Hg: This pattern of equalization of diastolic

pressure is consistent with cardiac tamponade physiology.

RA 9 mm Hg (normal), PA 30/10 mm Hg (borderline normal), PCWP 27 mm Hg (markedly

elevated): This is not physiologic and suggests an error in measurement.

Answer :D
Key Point
Patients with cardiogenic shock have hypotension, tachycardia, a low cardiac output, high
systemic vascular resistance, and poor organ perfusion. Cardiogenic shock is usually caused
by a large myocardial infarction, acute valvular insufficiency, or an acute intracardiac shunt.

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Question 22 of 35

A 35-year-old woman is 24 weeks pregnant and has a history of hypertension. Her current
medications include labetalol 200 mg twice daily and prenatal vitamins. She is asymptomatic
and has had an uncomplicated pregnancy. On examination, her blood pressure is 166/102 mm
Hg and pulse is 66 bpm. Her physical examination is unremarkable. After 5 minutes of rest in a
seated position, her repeat blood pressure is 162/100 mm Hg.

Which of the following is the most appropriate next step in management?

A. Hydrochlorothiazide 25 mg daily, reassess in 2 days.

B. Lisinopril 10 mg daily, reassess in 2 days.

C. Complete blood count.

D. Urinalysis.

E. Ambulatory blood pressure monitoring.

Given the significant morbidity and mortality associated with pre-eclampsia, it is essential to
diagnose this condition rapidly. The first step in managing this patient is to assess for
proteinuria. Thus, urinalysis is the correct answer. High-risk features of pre-eclampsia are
shown in Figure 1.

Laboratory evaluation for end-organ damage in pre-eclampsia should include a complete

blood cell count to evaluate for thrombocytopenia. However, the initial diagnosis of pre-
eclampsia should be made first with a urinalysis.

Angiotensin-converting enzyme inhibitors and angiotensin-receptor blockers are

contraindicated during pregnancy because of potential harm to the fetus. Thus, adding
lisinopril is contraindicated and is a Class III recommendation in the American College of
Cardiology (ACC) guidelines. Although hydrochlorothiazide may be continued in patients
already taking it prior to pregnancy, it should not be added during pregnancy unless there
are signs or symptoms of heart failure.

Ambulatory blood pressure monitoring can be used in certain clinical settings (e.g., white-
coat hypertension) to understand the temporal nature of a patient's blood pressure

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throughout the day. However, this would not be an appropriate test in this patient given the
clinical scenario.

Answer :D

Key Point
A hypertensive crisis is a diastolic blood pressure (BP) >120 mm Hg. If there is no evidence
of acute or rapidly worsening target organ damage, it is referred to as a hypertensive
urgency. A hypertensive emergency is a situation in which the BP is >180/120 mm Hg and
there is evidence of new or worsening target organ damage. A more useful definition is the
situation in a hypertensive patient with elevated BP in which there is rapidly worsening target
organ damage irrespective of the BP level.

Question 23 of 35

A 64-year-old man presents to the emergency department with shortness of breath. He has a
past history of hypertension, hypercholesterolemia, and erectile dysfunction. His outpatient
medications include aspirin 81 mg daily, atorvastatin 40 mg daily, amlodipine 10 mg daily,
lisinopril 40 mg daily, hydrochlorothiazide 25 mg daily, and sildenafil 100 mg as needed,
although he reports that some of his prescriptions have run out and he has not taken any blood
pressure (BP) medications in about a week.
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On examination, he appears short of breath. His temperature is 98.4°F, heart rate 104 bpm, BP
220/125 mm Hg, respiratory rate 20 breaths per minute, and oxygen saturation 88% on room
air. Jugular venous pressure is 10 cm water. There are crackles up to the mid lung fields
bilaterally. Heart is regular with an S4 gallop. Extremities are warm without edema.

Laboratories show creatinine 2.1 mg/dL (1.5 mg/dL 1 month prior). A chest X-ray is shown in
Figure 1.

Which of the following is the best next step?

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Reproduced with permission from Pulmonary Edema (University of Virginia School of Medicine
website). 2013. Available at: https://www.med-
ed.virginia.edu/courses/rad/cxr/pathology2chest.html. Accessed 04/02/2019.

A. Hydralazine 10 mg intravenous.

B. Nicardipine 5 mg/hour.

C. Esmolol 50 mcg/kg/min.

D. Resume outpatient antihypertensive medications.

E. Nitroglycerin 100 mcg/min.

"Hypertensive urgency" describes the situation in which a patient's systolic BP is >180 mm Hg

and/or diastolic BP is >120 mm Hg. If there is elevated BP and acute or rapidly worsening target
organ damage, then the term used is "hypertensive emergency." The therapeutic goal in
hypertensive emergency is to minimize target organ damage safely by rapid recognition of the
problem and early initiation of an appropriate antihypertensive treatment (Figure 2).

This patient has hypertensive emergency with diastolic BP >120 mm Hg along with acute renal
failure and pulmonary edema. The selection of an antihypertensive agent should be based on
the drug's pharmacology, pathophysiological factors underlying the patient's hypertension,
degree of progression of target organ damage, the desirable rate of BP decline, and the
presence of comorbidities.

There is no high-quality randomized controlled trial evidence to inform clinicians as to which

first-line antihypertensive drug class provides more benefit than harm in hypertensive
emergencies. This lack of evidence is related to the small size of trials, the lack of long-term
follow-up, and failure to report outcomes. However, two trials have demonstrated that
nicardipine may be better than labetalol in achieving the short-term BP target (Farias et al.,
Peacock et al.). Because autoregulation of tissue perfusion is disturbed in hypertensive
emergencies, continuous infusion of short-acting titratable antihypertensive agents is often
preferable to prevent further target organ damage. In this patient, nicardipine is the best choice.

Oral therapy is not appropriate in this case of hypertensive emergency with end-organ
dysfunction.

Unpredictability of response and prolonged duration of action do not make hydralazine a

desirable first-line agent for acute treatment in most patients.
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Beta-blockers are contraindicated in patients with bradycardia or decompensated heart failure.

As this patient has pulmonary edema, esmolol is not the preferred answer.

Nitrates such as nitroprusside or nitroglycerin are useful for most patients with hypertensive
emergency with pulmonary edema, but are contraindicated in this patient with recent sildenafil
use (Figure 3).

Answer :B

Key Point
A patient with hypertensive emergency should be admitted to the hospital for immediate
antihypertensive therapy, often parenteral, with continuous blood pressure monitoring. In
contrast, a patient with hypertensive urgency should be managed aggressively with oral
antihypertensive medications in the outpatient environment.

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Question 24 of 35

A 73-year-old woman is brought to the emergency department from her nursing home for
evaluation of lethargy. She has a history of dementia and type 2 diabetes mellitus. Medications
include aspirin 325 mg daily, donapezil 10 mg daily, glipizide 10 mg daily, and quetiapine 50 mg
daily. On examination, her blood pressure (BP) is 85/69 mm Hg, heart rate 113 bpm, respiratory
rate 18 breaths/min, and oxygen (O2) saturation 94% on room air. Her jugular venous pressure
is 13 cm of water. Her extremities are cool and mottled.

Laboratory data show hemoglobin 12 g/dL and mixed central venous O2 saturation 54%.

Which one of the following medical conditions is most consistent with the clinical presentation?

A. Decreased oral intake.

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B. Acute gastrointestinal bleed.

C. Aspiration pneumonia.

D. Hypoglycemic coma.

E. Myocardial infarction.

This patient had evidence of shock given her hypotension (systolic BP <90 mm Hg) and
evidence of end-organ hypoperfusion manifested by lethargy. Using the Fick equation, her
cardiac index is given by O2consumption / arteriovenous O2 difference = (125 mL O2/min/m2) /
[13.6 x hemoglobin x (O2 saturation – O2 mixed venous saturation)] = 125 / (13.6 x 12 x 0.4)
= 1.9 L/min/m2, which is lower than normal (2.5-4.5 L/min/m2). This finding of decreased
cardiac output along with elevated central venous pressures and increased systemic
vascular resistance (note the cool extremities) is characteristic of cardiogenic shock. Only
acute myocardial infarction fits this profile among the answer choices. An acute
gastrointestinal bleed and decreased oral intake would be associated with reduced central
venous pressures. Aspiration pneumonia may be associated with distributive shock, which
would manifest with reduced systemic vascular resistance (warm extremities) and likely
compensatory increased cardiac output. A hypoglycemic coma would not be expected to
increase central venous pressure or cause decreased cardiac output.

Answer :E
Key Point
Patients with cardiogenic shock have hypotension, tachycardia, a low cardiac output, high
systemic vascular resistance, and poor organ perfusion. Cardiogenic shock is usually caused
by a large myocardial infarction, acute valvular insufficiency, or an acute intracardiac shunt.

Question 25 of 35

You are seeing a 59-year-old patient with difficult-to-control hypertension in clinic. Her past
medical history is significant for coronary artery disease, osteoarthritis, asthma, hyperlipidemia,
fibromyalgia, and migraine headaches. Her daily medications include aspirin 81 mg, amlodipine
10 mg, chlorthalidone 25 mg QD, potassium chloride 20 mEq QD, losartan 100 mg QD, naproxen
500 mg BID, atorvastatin 40 mg QD, montelukast 10 mg QD, albuterol inhaled 2.5 mg NEB TID
PRN, and gabapentin 300 mg TID. On examination, her blood pressure (BP) is 140/80 mm Hg,
heart rate is 70 bpm, and oxygen saturation is 98%. Her lungs are clear and heart is without
murmurs, rubs, or gallops. Pertinent laboratory studies are notable for creatinine of 1.0 mg/dL
and normal electrolytes.

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Which of the following may be contributing to her systemic hypertension?

A. Atorvastatin.

B. Gabapentin.

C. Montelukast.

D. Albuterol.

E. Naproxen.

The correct answer is naproxen. Nonsteroidal anti-inflammatory drugs (NSAIDs) are known to
increase BP in both normotensive and hypertensive patients. In addition, they may reduce the
effect of all antihypertensives except for calcium channel blockers. The cause is believed to be
the NSAIDs' effect on inhibition of cyclooxygenase 2 in the kidney, which results in reduced
sodium excretion and an increase in intravascular volume.

NSAIDs can also mildly reduce the glomerular filtration rate. In many series, the mean effect of
NSAIDs on BP is to increase systolic BP by 3 mm Hg and diastolic BP by 2 mm Hg.

Other drugs that have been reported to increase BP include oral contraceptives, stimulants
(such as cocaine or amphetamines), antidepressants, and glucocorticoids. None of the other
drugs listed result in an increase in the systemic BP.

Answer :E

Key Point
Inadequate therapy is a common cause of uncontrolled BP. Also, up to 50% of resistant
hypertension is caused by nonadherence to prescribed therapy. Various medications,
including decongestants, bronchodilators, corticosteroids, and NSAIDs, including aspirin,
contribute to refractory hypertension by directly raising the BP, interfering with the actions of
antihypertensive medications, or both.

Question 26 of 35

A 47-year-old woman is referred to your office for an abnormal electrocardiogram (ECG). She
has a history of obesity and pre-eclampsia when she was pregnant with twins. She is on no
medications. On examination, her body mass index is 31 kg/m2, heart rate 80 bpm, and blood
pressure (BP) 128/72 mm Hg. Her physical examination is otherwise unremarkable. An ECG is
recorded (Figure 1).

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In addition to lifestyle counseling, which of the following is the best next step in this patient's
care?

A. 24-hour Holter monitor.

B. 24-hour ambulatory blood pressure monitor.

C. Exercise treadmill stress testing.

D. Thyroid stimulating hormone level.

E. 24-hour urine protein collection.

The ECG provided shows left ventricular hypertrophy, but her office BP is not significantly
elevated, suggesting a diagnosis of masked hypertension. The incidence of masked
hypertension has been reported to be as high as 10-40% in the general population.
Therefore, a 24-hour ambulatory BP monitor is indicated to make the diagnosis of masked
hypertension. Her history of pre-eclampsia puts her at higher risk for the development of
hypertension later in life. She is asymptomatic from a cardiovascular standpoint; therefore, a
Holter monitor or exercise testing are not warranted. A thyroid stimulating hormone and
urinalysis may be helpful once the diagnosis has been established; however, the first step in
the patient is an ambulatory BP monitor. If the spot urinalysis is abnormal, 24-hour urine
collection may be indicated.

Answer :B
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Key Point
White coat hypertension describes patients whose blood pressure (BP) is elevated (>130/80
mm Hg) in an office or clinic setting, with a normal daytime home (<130/80 mm Hg) or mean
24-hour ambulatory BP (<125/75 mm Hg). Masked hypertension is where the clinic BP is
normal, but home or mean 24-hour ambulatory BP measurements are high.

Question 27 of 35

A healthy, postmenopausal 53-year-old woman presents for evaluation of elevated blood

pressure. At her most recent primary care visit, her blood pressure was 138/88 mm Hg. At the
time of presentation, her blood pressure is 132/80 mm Hg. The patient wants to know if she has
hypertension.

Which of the following best describes her blood pressure?

A. Stage II hypertension is present.

B. Prehypertension is present.

C. There are inadequate blood pressure readings for a diagnosis.

D. Blood pressure is normal for her age.

E. Stage 1 hypertension is present.

The diagnosis of hypertension requires two careful readings on two or more occasions. This
patient has blood pressure readings on two separate occasions that fall within the stage 1
hypertension category (Figure 1). Stage 1 hypertension should be managed with
nonpharmacologic interventions such as dietary sodium restriction, DASH (Dietary
Approaches to Stop Hypertension) diet, limiting alcohol intake, weight loss, regular aerobic
exercise, and reassessment of blood pressure in 3-6 months.

Answer :E
Key Point
Normal blood pressure is <120 and <80 mm Hg, elevated blood pressure is 120-129 and
<80 mm Hg, stage 1 hypertension is 130-139 or 80-89 mm Hg, and stage 2 hypertension is
=140 or =90 mm Hg.

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Question 28 of 35

A 78-year-old man with a medical history notable for tobacco abuse, hypertension, and insulin-
dependent diabetes mellitus presents to the emergency department with chest pain, dyspnea,
and hypotension. His heart rate is 118 bpm with a blood pressure of 88/70 mm Hg. An
electrocardiogram demonstrates 5 mm anterior ST elevation. He receives a heparin bolus and
infusion, aspirin 325 mg, and 180 mg of ticagrelor prior to being transferred emergently to the
cardiac catheterization laboratory. Angiography demonstrates a thrombus in the proximal left
anterior descending artery. This lesion is treated with one drug-eluting stent, achieving
Thrombolysis in Myocardial Infarction (TIMI) 3 flow.

Due to persistent hypotension during the procedure, a pulmonary artery catheter is inserted
and yields the following data:
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Pulmonary capillary wedge pressure: 26 mm Hg

Cardiac output: 3.6 L/min

Cardiac index: 1.6 L/min/m2

Systemic vascular resistance: 1600 dynes-sec/cm5

An intra-aortic balloon pump (IABP) is considered; however, severe peripheral arterial disease
precludes its use. The patient is then transferred to the cardiac care unit, where his heart rate is
112 bpm with a blood pressure of 80/60 mm Hg. On exam he has bilateral crackles and requires
4 L of supplemental oxygen via nasal cannula. Laboratories are pending.

Which medication should be given at this time?

A. Phenylepherine.

B. Norepinephrine.

C. Vasopressin.

D. Dobutamine.

E. Nicardipine.

This patient has sustained an anterior myocardial infarction (MI) complicated by cardiogenic
shock supported both by the clinical scenario and the data obtained from the pulmonary
artery catheter. The mortality of patients with MI complicated by cardiogenic shock is high,
approaching 50% in some studies. Prompt revascularization and medical therapies are
crucial to lowering mortality in this population. This patient has already undergone successful
revascularization. Now, efforts should be directed toward support with medical therapies and
supportive devices (IABPs, left ventricular assist devices). Unfortunately, due to severe
peripheral arterial disease, options for advanced support devices are limited in this case.

The most recent ST-segment elevation MI guidelines do not specify a first choice for
vasopressor/inotrope in the setting of cardiogenic shock, as there are insufficient data upon
which to base such a recommendation. Rather, the guidelines state that therapy should be
individualized and guided by invasive hemodynamic monitoring.

In terms of medical therapies, norepinepherine—a potent vasopressor that has some

inotropic effects—is the most reasonable initial vasopressor of the choices provided.

Phenylepherine and vasopressin are both vasoconstrictors that will increase afterload and
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may adversely decrease cardiac output.

Nicardipine is a potent afterload-reducing agent that would potentially be harmful in this

patient with significant hypotension.

Dobutamine is an inotrope that can be used in the setting of low cardiac output with
preserved blood pressure. In this case, however, dobutamine can worsen the hypotension by
dilating peripheral arteries and would not be an ideal first choice.

Answer :B
Key Point
Inotropic agents and vasopressors are the mainstays of acute therapy. If unsuccessful, intra-
aortic balloon counterpulsation or even a short-term or long-term ventricular assist device
may become necessary. Vasodilators can be started when ventricular performance has
improved.

Question 29 of 35

A 45-year-old man with no prior medical history presents for evaluation of elevated blood
pressure (BP) after elevated readings in his primary care's office of 146/84 mm Hg. His BP at the
time of presentation is 144/82 mm Hg and, on repeat measurement, is 135/78 mm Hg. Body
mass index is 27 kg/m2. A physical examination is otherwise normal. Low-density lipoprotein
cholesterol is 138 mg/dL. His atherosclerotic cardiovascular disease (ASCVD) risk score is
calculated at 2.5%.

What is the best next step in his care?

A. 10 lb weight loss.

B. Hydrochlorothiazide/lisinopril 12.5 mg/10 mg daily.

C. Atorvastatin 40 mg daily.

D. Repeat blood pressure measurement in 6 months.

E. Amlodipine 5 mg daily.

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This patient has stage 1 hypertension, classified as systolic BP 130-140 mm Hg or diastolic BP 80-
90 mm Hg.

In all patients with newly diagnosed hypertension, counseling regarding lifestyle—including

sodium restriction, DASH (Dietary Approaches to Stop Hypertension) diet, weight loss,
treatment of sleep apnea, exercise regimen, alcohol reduction, and avoidance of nonsteroidal
anti-inflammatory drugs—is fundamental. Patients with stage 1 hypertension and an ASCVD risk
score of <10% should be managed with lifestyle modifications alone. Therefore, initiation of
antihypertensive medications is not warranted at this point.

Although repeat BP measurements are indicated, the patient should be counseled on lifestyle
modification.

Based on his ASCVD risk score, he does not meet the criteria for high-intensity statin therapy
with atorvastatin.

Answer :A
Key Point
There is clear evidence from clinical trials that changes in lifestyle, including weight loss and
maintenance of normal body weight; increased physical activity; dietary modification to
include more fruits, vegetables, and low fat dairy products; moderation of alcohol intake; and
sodium reduction in salt-sensitive individuals, can lower blood pressure in both individuals
with hypertension and those who are prehypertensive, and can prevent hypertension in the
latter group.

Question 30 of 35

A 67-year-old woman presents with crushing chest pain of 2 hours' duration. Coronary
angiography reveals multivessel disease with a left circumflex coronary artery occlusion,
successfully treated with a drug-eluting stent. Four hours after the procedure, she develops
acute shortness of breath and requires intubation.

The patient is intubated and sedated. Pulse rate is 110 bpm, respirations are 14 per minute, and
blood pressure is 100/65 mm Hg. Heart sounds are distant and no murmurs are audible. There
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are coarse breath sounds throughout the right lung field. Abdominal examination is normal. The
extremities are cool with 1+ pulses. A chest radiograph shows right-sided fluffy opacities and a
normal heart size. An electrocardiogram shows lateral Q waves and flipped T waves in the
inferior leads.

The patient is receiving milrinone (0.375 mcg/kg/min) and heparin (900 units hourly). A
pulmonary artery (PA) catheter is placed (pressures in mm Hg):

Right atrial pressure (central venous pressure) 11

Right ventricular pressure 53/10

Pulmonary artery pressure 55/35

Pulmonary capillary wedge pressure 40; “difficult to wedge”

Cardiac index 1.85 L/min/m2

Cardiac output 3.9 L/min

Pulmonary artery saturation 45%

Which of the following is the most likely diagnosis?

A. Right ventricular failure.

B. Left ventricular free-wall rupture.

C. Papillary muscle rupture.

D. Acute stent thrombosis.

E. Ventricular septal rupture.

Cardiogenic shock in patients with ST-segment elevation myocardial infarction (STEMI) may be
caused by extensive left ventricular (LV) infarction or by mechanical complications, including
papillary muscle rupture, ventricular septal rupture, free-wall rupture with tamponade, and
right ventricular (RV) infarction.

This patient presents with pulmonary edema and cardiogenic shock. The absence of a murmur
does not rule out mitral regurgitation (MR) because acute severe MR may not produce turbulent
flow. The right-sided pulmonary edema may be seen with acute MR from papillary muscle
rupture because the regurgitant jet produced by lateral papillary muscle rupture is directed
toward the right-sided pulmonary veins. Rupture of the posteromedial papillary muscle, which
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only receives its blood supply from a single coronary artery, is more common than rupture of
the anterolateral papillary muscle. The pulmonary catheter tracing demonstrates a high V-wave
pressure consistent with severe MR.

Stent thrombosis is unlikely in the absence of recurrent or new ST elevations.

Free-wall rupture is characterized by recurrent chest pain and ST-T–wave changes, with rapid
progression to hemodynamic collapse, electromechanical dissociation, and death. With a
contained free-wall rupture, the patient may have evidence of cardiac tamponade. The
hemodynamics in this case do not demonstrate equalization of diastolic pressures or elevated
right atrial (RA) pressure.

Ventricular septal rupture usually is heralded by a loud systolic murmur and heart failure or
shock, depending on the size of the defect and the degree of RV and LV dysfunction. A
postinfarct ventricular septal defect would cause an elevated PA saturation due to the left-to-
right shunt at the RV level.

RV infarction complicates the course of approximately one-third of patients with inferior STEMI,
is most often due to proximal occlusion of the right coronary artery, and is associated with a
higher mortality risk. Evidence of RV involvement should be sought in all patients with inferior
STEMI. The clinical triad of hypotension, clear lung fields, and elevated jugular venous pressure
is characteristic. This patient has pulmonary edema and RV/PA pressures out of proportion to
RA hypertension.

Answer :C
Key Point
Patients with cardiogenic shock have hypotension, tachycardia, a low cardiac output, high
systemic vascular resistance, and poor organ perfusion. Cardiogenic shock is usually caused
by a large myocardial infarction, acute valvular insufficiency, or an acute intracardiac shunt.

Question 31 of 35

A 74-year-old man with a history of hypertension, sleep apnea, and diabetes mellitus presents
to the emergency department complaining of chest pressure for the prior 12 hours. This has
been accompanied by dyspnea with exertion, particularly when ascending the stairs in his home.
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His medications include metformin 1000 mg BID, amlodipine 5 mg daily, and lisinopril 20 mg
daily. He has already taken his medications that morning.

A physical examination is notable for an elderly man who is visibly dyspneic and uncomfortable
appearing at rest. His heart rate is 68 bpm with blood pressure (BP) of 186/120 mm Hg. Oxygen
saturation is 88% on room air. Radial pulses are 2+ and symmetric. There is no murmur on
cardiac auscultation. Jugular venous pressure is elevated to 12 cm water and there are bilateral
crackles to the mid lung fields. Neurologic examination is unremarkable.

Complete blood cell count and basic metabolic panel are within normal limits. D-dimer is
negative. Troponin T is negative.

An electrocardiogram shows sinus rhythm with left ventricular (LV) hypertrophy. A chest X-ray
demonstrates pulmonary edema. A bedside transthoracic echocardiogram demonstrates a
dilated LV with global hypokinesis and an estimated LV ejection fraction of 45%. There is no
hemodynamically significant valve disease.

In addition to starting intravenous (IV) diuretics, which is the best next step in his care?

A. Esmolol.

B. Spironolactone.

C. Intravenous nitroglycerin.

D. Intravenous hydralazine.

E. Intravenous labetalol.

This clinical scenario describes a patient with hypertensive emergency, defined as a systolic BP
>180 mm Hg and/or diastolic BP >120 mm Hg in addition to evidence of end-organ dysfunction
(Figure 1). In this case, the end-organ dysfunction is the presence of pulmonary edema. The goal
of treatment is to lower the systolic BP by no more than 25% in the first hour, then to
approximately 160/100 mm Hg over the next 2-6 hours, and then eventually to normal over the
next 24-48 hours. This is generally achieved with parenteral medications in the intensive care
unit setting (Figure 2a and b). Caveats to this management strategy include acute ischemic
stroke, acute aortic dissection, severe pre-eclampsia, or pheochromocytoma with hypertensive
crisis.

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Selection of a first-line medication should be based on the drug's pharmacology and the clinical
circumstances. In the setting of hypertensive emergency and acute heart failure, vasodilating
medications that can be titrated (i.e., nitroglycerin, nitroprusside) are ideal. Medicines that can
decrease contractility (i.e., labetalol or esmolol) should be avoided. Similarly, IV hydralazine can
result in rebound tachycardia and is therefore not the best choice.

Oral antihypertensive medications are not appropriate first steps in the management of
hypertensive emergency, so oral spironolactone is incorrect.

Answer :C
Key Point
A patient with hypertensive emergency should be admitted to the hospital for immediate
antihypertensive therapy, often parenteral, with continuous blood pressure monitoring. In
contrast, a patient with hypertensive urgency should be managed aggressively with oral
antihypertensive medications in the outpatient environment.

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Question 32 of 35

A 39-year-old woman gravida 2 (G2), parity 1 (P1) at 30 weeks of gestation presents to her
routine prenatal visit with complaints of headaches and blurry vision. Her past medical history is
unremarkable and her previous pregnancy and delivery were uneventful. Her medications
include prenatal vitamins and folate. On examination, her blood pressure (BP) is 180/110 mm
Hg, heart rate is 90 bpm, and oxygen saturation is 95% on room air. There are mild bilateral
basal crackles and bilateral lower extremity swelling.

Stat laboratory data show creatinine of 1.8 mg/dL. Urine analysis shows evidence of proteinuria.

In addition to admitting the patient to the intensive care unit, what is your best next step in
management?

A. Start enalaprilat 1.25 mg intravenous to reduce systolic blood pressure to <140 mm Hg

during the first hour.

B. Start nitroprusside intravenous to reduce systolic blood pressure to <140 mm Hg during

the first hour.

C. Start esmolol 80 mg intravenous to reduce systolic blood pressure to 120 mm Hg within

the first hour.

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D. Start labetalol 20 mg intravenous to reduce systolic blood pressure to <140 mm Hg

during the first hour.

E. Start nicardipine 20 mg PO to reduce systolic blood pressure to 120 mm Hg within the

first hour.

Hypertensive emergencies are defined as severe elevations in BP (>180/120 mm Hg)

associated with evidence of new or worsening target organ damage. Hypertensive
emergencies demand immediate reduction of BP
(not necessarily to normal) to prevent or limit further target organ damage. Examples of
target organ damage
include hypertensive encephalopathy, intracranial hemorrhage, acute ischemic stroke, acute
myocardial infarction, acute left ventricular failure with pulmonary edema, unstable angina
pectoris, dissecting aortic aneurysm, acute renal failure, and eclampsia.

In general, use of oral therapy is discouraged for hypertensive emergencies. Compelling

conditions requiring rapid lowering of systolic BP, usually to <140 mm Hg, in the first hour of
treatment include aortic dissection, severe pre-eclampsia or eclampsia, and
pheochromocytoma with hypertensive crisis. This patient has features of severe pre-
eclampsia based on clinical picture and laboratory findings.

Angiotensin-converting enzymes are contraindicated in pregnancy.

Nitroprusside has the risk of fetal toxicity and should be a last resort.

Esmolol is a poor antihypertensive agent and should be used for rate control.

Answer :D
Key Point
A patient with hypertensive emergency should be admitted to the hospital for immediate
antihypertensive therapy, often parenteral, with continuous blood pressure monitoring. In
contrast, a patient with hypertensive urgency should be managed aggressively with oral
antihypertensive medications in the outpatient environment.

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Question 33 of 35

What changes would you expect to see for the following parameters in a typical 80-year-old
patient compared with a 20-year-old?

Systolic Blood Pressure Diastolic Blood Pressure Aortic Pulse Wave Velocity
Endothelial Nitric Oxide Release

A Increase Decrease Increase Decrease

B Decrease Increase Decrease Decrease

C Increase Increase Decrease Increase

D Increase Decrease Decrease Increase

E Decrease Increase Increase Decrease

A. A.

B. B.

C. C.

D. D.

E. E.

Aging is typically associated with multiple hemodynamic changes, including increases in

systolic blood pressure and decreases in diastolic blood pressure due to decreased aortic
distensibility (and thus increased pulse pressure), increased pulse wave velocity due to
decreased aortic distensibility and increased arteriolar resistance, and endothelial
dysfunction (which includes reduced nitric oxide synthesis and/or release). These changes
can result in isolated systolic hypertension, which is more common among older adults.

Answer :A
Key Point
Primary hypertension represents a complex interplay of polygenic and acquired etiology.
Many pathophysiologic factors have been implicated in the genesis of essential
hypertension, including increased sympathetic nerve activity, increased concentrations of
vasoactive and salt-retaining hormones, endothelial dysfunction, increased vascular
reactivity, arterial stiffness, and remodeling.

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Question 34 of 35

A 56-year-old man with coronary artery disease status/post right coronary artery stenting 3
years prior, hypertension, type II diabetes mellitus, and hyperlipidemia presents to clinic for
annual follow-up evaluation. He reports that he is currently asymptomatic.

His blood pressure (BP) is 146/91 mm Hg with heart rate of 72 bpm. His height is 5 feet 8 inches
and his weight is 218 lbs. On examination, he is euvolemic with a soft S4 gallop and no murmurs.
There is no peripheral edema. His electrocardiogram demonstrates sinus rhythm with left
ventricular hypertrophy (LVH). An echocardiogram performed the year prior showed preserved
ejection fraction, LVH, and no significant valvular abnormalities. Laboratory values reveal
potassium 4.0 mmol/L, creatinine (Cr) 1.2 mg/dL, total cholesterol 170 mg/dL, low-density
lipoprotein 64 mg/dL, and hemoglobin A1c 6.2%. Urinalysis reveals microalbuminuria. His daily
medications include rosuvastatin 40 mg, aspirin 81 mg, and metoprolol succinate 50 mg.

Lisinopril 10 mg daily is started. He returns to clinic 1 week later and his BP is 122/78 mm Hg.
Repeat laboratory values reveal potassium 4.4 mmol/L and Cr 1.5 mg/dL.

What is the best next step in his care?

A. Reduce lisinopril to 5 mg daily.

B. Stop lisinopril, add amlodipine 5 mg daily.

C. Continue current therapies.

D. Add spironolactone 12.5 mg daily.

E. Add sacubitril/valsartan.

This patient has diabetes mellitus with renal insufficiency and microalbuniuria for which an
angiotensin-converting enzyme inhibitor (ACEI) or angiotensin II receptor blocker (ARB) is
indicated to treat the hypertension and slow the rate of renal dysfunction. An expected effect
of the addition of an ACEI or ARB is a rise in serum Cr of ≤30%, which was observed in this
patient. This is not an indication to change dose or stop lisinopril.

This patient does not have resistant hypertension or heart failure, and his BP is reasonably
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controlled on his current regimen. Therefore, addition of spironolactone is incorrect.

Similarly, sacubitril/valsartan is indicated in patients with reduced ejection fraction and heart
failure, which this patient does not have.

Answer :C
Key Point
In diabetes mellitus and chronic kidney disease, angiotensin-converting enzyme inhibitors
and angiotensin-receptor blockers are essential components of the management of
hypertension because they slow the rate of decline of renal function in diabetic nephropathy,
and may do so in hypertensive nephropathy.

Question 35 of 35

You are seeing a 56-year-old African American woman in clinic for a routine annual visit. She has
a history of type 2 diabetes mellitus, hypertension, and obstructive sleep apnea. Her
medications include metformin 500 mg twice daily, lisinopril 40 mg daily, amlodipine 5 mg daily,
and atorvastatin 40 mg daily. Her body mass index is 34.6 kg/m2. Her blood pressure is 134/82
mm Hg and heart rate is 72 bpm. Her examination is unremarkable.

From an epidemiologic perspective, which of the following risk factors present in this patient is
most responsible for an increased population burden of heart failure with preserved ejection
fraction (HFpEF)?

A. Diabetes mellitus.

B. Obesity.

C. Obstructive sleep apnea.

D. Female sex.

E. Hypertension.

Among the listed risk factors, hypertension is the risk factor with the highest population
attributable risk (PAR) for incident HFpEF. Women are more likely to be diagnosed with
HFpEF than men, but the PAR for female sex over male sex is less prominent than the PAR
for hypertension. Diabetes mellitus and obesity have been reported to confer higher risk of
incident HFpEF with less powerful effects than hypertension. This has been shown across
races. Not included in the options is history of coronary artery disease, which also confers
increased risk of HFpEF diagnosis.

Answer :E

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Key Point
The risk of dying from stroke or ischemic heart disease at all ages doubles for each
increment of 20/10 mm Hg of blood pressure, starting as low as 115/75 mm Hg.

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Vascular diseases

Question1:
A 72-year-old woman with a history of coronary artery disease status/post coronary artery bypass
grafting, hypertension, dyslipidemia, diabetes mellitus, and osteoarthritis presents with 6 months of
abdominal pain most notable after eating. She has experienced a 5 lb weight loss over the prior 2 months.
Her medications include aspirin 81 mg daily, metoprolol succinate 25 mg daily, lisinopril 10 mg daily,
rosuvastatin 40 mg daily, and metformin 500 mg twice daily. She smokes 1 pack of cigarettes daily.
On examination, her heart rate is 68 bpm and blood pressure is 138/90 mm Hg. Lungs are clear and heart
is regular with a II/VI systolic murmur at the base. Abdomen is soft and nontender to palpation with no
organomegaly or abdominal bruits. Extremities are warm with no edema.Duplex ultrasonography of the
mesenteric vessels reveals a 90% stenosis of the celiac artery and 70% stenosis of the superior mesenteric
artery.

Which of the following is most likely to alleviate her symptoms?

A. Percutaneous revascularization.
B. Cilostazol 100 mg twice daily taken before meals.
C. Smoking cessation.
D. Graduated refeeding program.
E. Clopidogrel 75 mg daily.

Chronic mesenteric ischemia, also called intestinal angina, refers to episodic or constant hypoperfusion of
the small intestine caused by atherosclerotic narrowing of the origins of the celiac or superior mesenteric
arteries. Patients are typically >60 years of age, female, and smokers, as well as have a history of other
atherosclerotic disease.
Unexplained chronic abdominal pain, weight loss, and food aversion in a patient with atherosclerotic
disease should suggest chronic mesenteric ischemia. The diagnosis is supported by the imaging that
demonstrates high-grade stenosis or occlusion of two or more mesenteric vessels.
The indication for revascularization (open or endovascular) is the presence of symptoms, including
abdominal pain and weight loss, in the setting of documented severe splanchnic artery stenoses. The aim
of intervention is to prevent future bowel infarction. Options for revascularization include open surgical
reconstruction and percutaneous transluminal angioplasty with or without placement of a stent. Provided
an endovascular approach is technically feasible, angioplasty with or without stenting is preferred over a
surgical approach.

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Although it is imperative that she stop smoking to minimize the progression of disease, it is not likely to
improve her symptoms of mesenteric ischemia.
Intensification of antiplatelet therapy does not relieve symptoms of chronic mesenteric ischemia.
Cilostazol, a phosphodiesterase inhibitor approved by the Food and Drug Administration for the treatment
of claudication, is not a first-line therapy for symptomatic mesenteric ischemia.
Although supervised exercise is a treatment for claudication, there is not an analogous approach for
mesenteric ischemia. Graduated refeeding is not an accepted intervention for this condition.
Answer: A
Key Point
For patients with symptoms of chronic mesenteric ischemia and weight loss, revascularization is
recommended when anatomically feasible.

Question2:
A 65-year-old woman with hyperlipidemia presents to the emergency department with a 2-week history
of left leg swelling. Her history is significant for overseas travel 3 weeks prior. She otherwise feels well
and denies dyspnea or chest discomfort.On examination, her heart rate is 68 bpm, blood pressure is
126/68 mm Hg, and oximetry is normal. Her left calf appears larger than the right, with associated pitting
edema and tenderness to palpation. Duplex ultrasound reveals an occlusive thrombus in the distal
popliteal vein.

Which one of the following is the most appropriate initial therapy?

A. Dabigatran 150 mg BID.
B. Rivaroxaban 20 mg BID for 7 days, then 20 mg daily.
C. Warfarin with international normalized ratio goal of 2-3.
D. Edoxaban 60 mg daily.
E. Apixaban 10 mg BID for 7 days, then 5 mg BID.

The patient has a provoked deep vein thrombosis (DVT). Direct oral anticoagulants (e.g., dabigatran,
rivaroxaban, apixaban, or edoxaban) are now recommended as first-line therapy for venous
thromboembolism over vitamin K antagonists (e.g., warfarin). Low molecular weight heparin (LMWH)
would be correct as a first-line therapy.

The dosing of rivaroxaban for the treatment of DVT is 15 mg BID for 21 days, then 20 mg daily.

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Edoxaban for treatment of a DVT needs to be preceded by LMWH for 5-10 days. Therefore, edoxaban 60
mg daily without LMWH is incorrect. Edoxaban requires dose adjustment for reduced creatinine
clearance (i.e., glomerular filtration rate: 30-50 mL/min) or concomitant use of potent proton pump
inhibitor.

Similar to edoxaban, dabigatran needs to be preceded by LMWH, making dabigatran alone incorrect.
Answer : E

Key Point
Direct oral anticoagulants are safe and effective agents for the prevention and treatment of venous
thromboembolism (VTE). They are recommended as first-line anticoagulants for most VTE patients.

Question3:
A 58-year-old woman presents to the emergency department with acute onset of left-sided weakness. She
has a history of hypertension and dyslipidemia. Her medications include aspirin 81 mg daily, atorvastatin
40 mg daily, chlorthalidone 25 mg daily, and amlodipine 10 mg daily.On examination, she is anxious
appearing. Her temperature is 98.6°F, heart rate 90 bpm, and blood pressure 150/80 mm Hg. Her heart is
regular, lungs are clear, abdomen is soft and nontender, and extremities are warm without edema.On
neurologic examination, she has left arm and leg hemiparesis and paresthesia with expressive
aphasia.Electrocardiogram shows normal sinus rhythm with nonspecific ST-T changes.Carotid ultrasound
shows a 40-69% right carotid occlusion and 10-39% left carotid occlusion.Echocardiogram shows normal
ventricular and valvular function.

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There is a patent foramen ovale (PFO) noted on color Doppler. This is confirmed on transesophageal
echocardiogram, which shows an atrial septal aneurysm with right-to-left shunting on injection of agitated
saline contrast.Brain magnetic resonance imaging shows infarction of the M1 branch of the right middle
cerebral artery (MCA).
What is the best next step?
A. Surgical closure of the patent foramen ovale.
B. Right carotid endarterectomy.
C. Intravenous unfractionated heparin and warfarin titrated to international normalized ratio 2.5.
D. Percutaneous device closure of the patent foramen ovale.
E. Prasugrel 10 mg daily.

This patient has a cryptogenic stroke, defined as stroke of undetermined origin after exclusion of treatable
causes such as cardioembolism from atrial fibrillation or large artery atherosclerosis from carotid disease.
In patients 60 years of age or younger with a cryptogenic stroke, patent PFO percutaneous device closure
is more effective than antiplatelet therapy alone for reducing the risk of recurrent stroke. The risk of
recurrent stroke with device closure is reduced by approximately 60% compared with medical therapy
(5% to 2% in 3- to 6-year follow-up).Notably, national and society guidelines regarding the management
of a PFO in patients with cryptogenic stroke (American College of Chest Physicians 2012, American
Heart Association/American Stroke Association 2014, American Academy of Neurology 2016) were
published prior to the 2017 publication of three randomized trials showing reductions in risk of recurrent
stroke with PFO closure compared with medical therapy (RESPECT [Randomized Evaluation of
Recurrent Stroke Comparing PFO Closure to Established Current Standard of Care Treatment] extended
follow-up, CLOSE [Patent Foramen Ovale Closure or Anticoagulants vs. Antiplatelet Therapy to Prevent
Stroke Recurrence], and REDUCE PFO [Patent Foramen Ovale Closure or Antiplatelet Therapy for
Cryptogenic Stroke]).
If cardiac surgery is required for another reason, surgical PFO closure is a reasonable alternative to
percutaneous closure, although there are no clinical trials comparing surgical PFO closure with
percutaneous closure or medical therapy. This patient has no indication for cardiac surgery, so surgical
PFO closure is not warranted.Carotid endarterectomy is recommended for significant symptomatic carotid
stenosis, defined as stenosis >70% by ultrasound. Although a right carotid artery stenosis could cause a
right M1 MCA stroke, the carotid stenosis is not significant in this case and thus carotid endarterectomy
is not indicated.
In the absence of atrial fibrillation or deep venous thrombosis, anticoagulation is not recommended
poststroke. Although antiplatelet therapy is warranted, percutaneous PFO closure is superior to
antiplatelet therapy alone. In patients with a history of TIA or stroke, prasugrel is contraindicated as a
higher rate of stroke (thrombotic and hemorrhagic) has been observed.
Answer: D
Key Point
Catheter closure of patent foramen ovale can be considered in the setting of embolic-appearing ischemic
stroke without other etiology despite adequate testing in patients 18-60 years of age.

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Question 4:
A 37-year-old woman presents to your office with a longstanding history of her fingers turning white and
becoming painful in cold weather. She has no medical problems. She is on no medications. She reports a
history of methamphetamine and heroin use in the past and, at the time of presentation, smokes one pack
of cigarettes daily and drinks four to six beers daily.On examination, she is thin and anxious appearing.
Her heart rate is 88 bpm and blood pressure is 100/60 mm Hg. Lungs are clear, heart is regular, and
abdomen is soft. Her hands are shown in Figure 1.

What is the most likely cause of her presentation?

A. Heroin.
B. Methamphetamines.
C. Hypothyroidism.
D. Carpal tunnel syndrome.
E. Cigarette smoking.
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The Raynaud phenomenon (RP) is an exaggerated vascular response to cold temperature or emotional
stress. The RP is manifested clinically by sharply demarcated color changes of the skin of the digits.
Abnormal vasoconstriction of digital arteries and cutaneous arterioles due to a local defect in normal
vascular responses is thought to underlie the disorder. In this case, Figure 1 is classic for RP, with the
sharply demarcated color changes of skin pallor due to constricted blood flow. She has a history of
methamphetamine use, which is the most likely cause of RP in this patient.

Primary RP or idiopathic Raynaud disease are terms to describe those patients without a definable cause
for their vascular events. In this setting, RP is considered to be an exaggeration of normal
vasoconstriction to cold exposure. Secondary RP refers to those patients with RP in whom an associated
disease or cause may underlie the attacks, and in this case is often referred to as Raynaud syndrome.

Since a variety of possible insults can disrupt the normal complex regulation of regional blood flow to the
digits and skin, the number of diseases and exposures associated with secondary RP is extensive. Diseases
commonly associated with RP include autoimmune rheumatic diseases such as systemic sclerosis,
systemic lupus erythematosus, mixed connective tissue disease, Sjögren syndrome, and
dermatomyositis/polymyositis. Hematologic abnormalities may also cause RP.

Various drugs or toxins can also precipitate or exacerbate RP through various mechanisms. Drugs that
enhance vasoconstriction include beta-blockers, clonidine, ergot alkaloids, and dopaminergic alkaloids
such as bromocriptine, sympathomimetics, stimulants, and cyclosporine. Drugs that cause endothelial
dysfunction include chemotherapeutic agents such as cisplatin and bleomycin. Inferons can increase
blood viscosity and tyrosine kinase inhibitors can cause RP via an unknown mechanism.

Heroin, cigarette smoking, and alcohol use are not associated with RP.

Although hypothyroidism and carpal tunnel syndrome can be associated with RP, there is nothing in the
history to suggest these diagnoses. Hypothyroidism may predispose to RP and improvement of cold-
induced vasospasm may occur with thyroid hormone replacement. Occupational and environmental
causes of RP include vascular trauma (e.g., injury to the distal ulnar artery in the hypothenar hammer
syndrome), the use of vibrating tools, frostbite, and carpal tunnel syndrome.
Answer: B

Key Points

• Raynaud’s phenomenon is characterized by a classic sequence of digital color changes including

pallor (due to vasoconstriction), cyanosis (deoxygenation of the blood), and rubor (hyperemia);
however, a partial presentation is possible.
• Initial therapies for Raynaud’s phenomenon include avoidance of cold and other triggers (e.g.,
stress or caffeine), as well as maintenance of warm core body temperature (e.g., wearing multiple
layers, hats, gloves).

Question 5 :
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A 55-year-old man with hypertension and obstructive sleep apnea is referred to you for evaluation of
peripheral arterial disease (PAD) after undergoing ankle-brachial indices (ABIs). He does not exercise
regularly but is able to perform his daily activities without limitations. His medications include aspirin 81
mg daily, amlodipine 5 mg daily, and lisinopril 20 mg daily.On examination, his heart rate is 68 bpm,
blood pressure (BP) is 118/70 mm Hg, and body mass index is 32 kg/m2. His heart rate is regular. There
are no cardiac murmurs and lungs are clear to auscultation. Extremities are warm without edema. Dorsalis
pedis and posterior tibial pulses are 1+ on the right and normal on the left. Femoral and popliteal pulses
are 2+ bilaterally.
Laboratories include sodium 142 mg/dL, potassium 4.0 mEq/dL, creatinine 1.0 mg/dL, hemoglobin A1c
5.9%, total cholesterol 158 mg/dL, high-density lipoprotein 38 mg/dL, low-density lipoprotein (LDL) 70
mg/dL, triglycerides 250, aspartate aminotransferase 42 U/L, and alanine aminotransferase 56 U/L. ABIs
are 1.1 on the left and 0.8 on the right.
Addition of which of the following medications is most appropriate in his care?
A. Ticagrelor 90 mg twice daily.
B. Atorvastatin 40 mg daily.
C. Metformin 500 mg twice daily.
D. Chlorthalidone 25 mg daily.
E. Pentoxifylline 400 mg twice daily.

This patient has PAD, as indicated by his diminished pulses and abnormal ABI result (ABI <0.9). PAD is
a manifestation of atherosclerotic cardiovascular disease and therefore should be managed with guideline-
directed medical therapy for secondary prevention. The management of patients with lower extremity
PAD is aimed at relieving symptoms and lowering the risk of cardiovascular disease progression and
complications. Therefore, he should receive high-intensity statin therapy regardless of LDL level to
achieve a 50% LDL reduction. Statin therapy is not contraindicated due to mild elevation in liver function
tests but should be monitored after initiation of therapy.

Ticagrelor may provide additional benefit to aspirin in patients with symptomatic PAD and prior
myocardial infarction (see the PEGASUS-TIMI 54 [Prevention of Cardiovascular Events in Patients With
Prior Heart Attack Using Ticagrelor Compared to Placebo on a Background of Aspirin–Thrombolysis in
Myocardial Infarction 54] trial), but this patient has asymptomatic PAD and no prior history of
myocardial infarction, so ticagrelor would not be appropriate.

In patients with lower extremity PAD, treatment of diabetes mellitus can be effective for reducing
complications; a goal of control of blood glucose levels to an A1C of <7.0% is recommended. However,
this patient does not have diabetes mellitus, so oral hypoglycemic therapy is not indicated.

Hypertension is a major risk factor for PAD. However, there are no data evaluating whether
antihypertensive therapy alters the progression of PAD. Nevertheless, hypertension should be controlled
to reduce morbidity from cardiovascular and cerebrovascular disease, with a goal BP <120/70 mm Hg.
However, this patient's BP is adequately controlled, so additional antihypertensive therapy is not
warranted.
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Pentoxifylline increases the deformability of red blood cells, reduces blood viscosity, decreases
fibrinogen concentration, and reduces platelet adhesiveness; it has been found to improve functional
symptoms in patients with PAD. This patient has asymptomatic PAD, and therefore treatment would not
be warranted.
Answer : B
Key Point
Treatment of peripheral artery disease includes a focus on prevention of stroke and myocardial infarction:

• Smoking cessation: in the form of counseling at every visit, pharmacotherapy, and referral to
cessation programs.
• High-intensity statin therapy: appropriate for patients =75 years, or moderate-intensity statins in
those who are not candidates for high-intensity statin therapy.
• Glycemic control: patient-specific strategy with hemoglobin A1c target tailored to the patient.
• Antihypertensive therapy: target of <130/80 mm Hg when appropriate. Angiotensin-converting
enzyme inhibitor/angiotensin-receptor blocker may offer additional benefit.
• Antiplatelet therapy: aspirin or clopidogrel monotherapy. Ticagrelor offers no additional benefit,
but may be used when other indications are present.
• Anticoagulation: consideration for low-dose rivaroxaban plus aspirin in patients with peripheral
artery disease and acceptable bleeding risk.

Question6:
A 65-year-old woman presents with a 2-day history of worsening headache, nausea, and vomiting. Her
past medical history is significant for diabetes mellitus, hypertension, and hyperlipidemia. Her
medications include metformin 1000 mg twice daily, lisinopril 40 mg, amlodipine 10 mg, chlorthalidone
25 mg, and atorvastatin 40 mg.On examination, she is confused and restless. Her blood pressure is
200/112 mm Hg, her heart rate is 90 bpm, and she is afebrile. Fundoscopic examination is normal. Her
carotids are without bruits and neck veins are flat. Her lungs are clear bilaterally and heart has normal S1,
S2, and S4. Her extremities are without edema.She undergoes urgent head computed tomography (CT),
which shows no evidence of ischemia or hemorrhage. She is treated with intravenous nitroprusside and,
over the next few hours, becomes less confused and her headache improves.
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Which of the following diagnoses is most likely in this patient?

A. Cerebral hemorrhage.
B. Hypertensive retinopathy.
C. Seizure disorder.
D. Atypical migraines.
E. Hypertensive encephalopathy.

This patient exhibits signs of cerebral edema (including headache, nausea, and vomiting) and nonfocal
neurologic symptoms. These improve with treatment of her hypertension and therefore are consistent with
hypertensive encephalopathy. If left untreated, this can further progress to seizures and coma.
Hypertensive encephalopathy is a diagnosis of exclusion and imaging should be performed to rule out any
other causes of neurologic symptoms. Cerebral edema may be seen on T2 weighted images of a magnetic
resonance image and would further support this diagnosis.

A normal head CT and nonlocalizing symptoms make hemorrhage a less likely diagnosis. Fundoscopic
examination is normal, making hypertensive retinopathy a less likely diagnosis. Her presentation is not
consistent with a seizure disorder because her examination is nonfocal and the symptoms resolved with
treatment of hypertension. Atypical migraines do not usually resolve with the treatment of hypertension.
Answer: E
Key Point
The clinical features of hypertensive encephalopathy include headache, impaired consciousness, and
seizures. Focal neurologic signs could also be observed.

Question7:
You are rounding on a 65-year-old man with type 2 diabetes mellitus, hypertension, peripheral vascular
disease, and multivessel coronary artery disease who had undergone a complex percutaneous coronary
intervention the day prior. During your rounds, he is oliguric and his creatinine has risen from 0.8 g/dL to
2.8 g/dL over the prior 48 hours. He reports no dysuria and is afebrile on examination. On physical
examination, he has livedo reticularis of his feet. A urinalysis and urine chemistries are ordered.
Which one of the following would confirm the diagnosis in this patient?
A. Hyaline casts.
B. Urine eosinophils.
C. Microscopic hematuria.
D. Ketonuria.
E. Nephrotic range proteinuria.

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This patient has atheroembolic embolization (also known as cholesterol embolization syndrome)
following his recent cardiac catheterization. Atheroembolic disease can include any organ system and the
manifestations depend on the affected vascular bed. The most specific peripheral finding of atheroembolic
disease is livedo reticularis (mottled reticulated vascular pattern; Figure 1) and blue-toe syndrome. In
addition, atheroembolic renal disease is characterized by progressive renal dysfunction occurring in
staggered steps or a staircase pattern separated by periods of stable renal function. Eosinophilia is seen in
≤80% of patients with cholesterol embolization due to the inflammatory response to the cholesterol
emboli.

Eosinophiluria can also be found in acute interstitial nephritis, pyelonephritis, prostatitis, cystitis, or
rapidly progressive glomerulonephritis. Hematuria is commonly seen in glomerulonephritis or vasculitis.
Nephrotic range proteinuria would be more suggestive of a primary glomerular disorder. Elevated urine
ketones would suggest ketoacidosis. Hyaline casts are a nonspecific finding and would not be specific for
an atheroembolic event.

Answer: B
Key Point
The majority (>70%) of atheroembolism is iatrogenic, occurring as a complication of catheter-based
angiography or vascular surgery.

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Question8 :
A 32-year-old man with a history of Marfan syndrome and thoracic aortic aneurysm presents after a
recent chest computed tomography (CT) angiogram. He is 6'3" and weighs 193 lbs. His blood pressure is
130/78 mm Hg and heart rate is 55 bpm. Cardiac examination reveals pectus excavatum, normal S1 and
S2, and no murmurs. Lungs are clear. Abdomen is thin with no organomegaly.On CT, his aortic root
measures 4.2 cm (compared with 3.9 cm the prior year), ascending aorta 4.5 cm, and aortic arch 3.3 cm.

What is the best next step in his care?

A. Add losartan 25 mg daily.
B. Add aspirin 81 mg daily.
C. Refer for surgical repair.
D. Chest computed tomography angiography in 3 months.
E. Transthoracic echocardiogram.

Marfan syndrome, affecting 1 in approximately 5,000 individuals, is an autosomal-dominant connective-

tissue disorder due to mutations in the gene encoding fibrillin-1 (FBN1).

This patient would benefit from further blood pressure lowering to prevent progression of the aortic root
dilation. This could be with either a beta-blocker or angiotensin II blocker. In a 2014 trial, patients with
Marfan syndrome were randomly assigned to losartan or atenolol; they showed no differences in aortic
dilation rate or presence of clinical events between treatment groups.

A repeat CT angiogram in 3 months is too soon and should not be performed again until 6 months.

Aspirin is beneficial for patients with aneurysms due to peripheral atherosclerosis to reduce cardiac
events, but not in patients with Marfan.

An echocardiogram is not indicated since he had a recent chest CT for sizing of his aneurysm.

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The threshold for surgical repair in patients with Marfan syndrome is an external diameter of 5 cm.
Factors that will prompt repair at a diameter <5 cm include rapid growth of >0.5 cm in 1 year, family
history of aortic dissection at a diameter <5 cm, or the presence of significant aortic regurgitation.
Answer : A
Key Point
Medical therapy for thoracic aortic aneurysm disease includes beta-blockers and the use of a vasodilating
agent (such as an angiotensin-converting enzyme inhibitor or angiotensin-receptor blocker) if blood
pressure remains elevated. Losartan has not proven more effective than atenolol for slowing the rate of
aortic root enlargement in young patients with Marfan syndrome. Statin and aspirin therapy should be
provided according to current guideline recommendations for patients with atherosclerotic disease.

Question 9:
A 29-year-old woman presents to the emergency department with a painful and tender right calf, which
she notices upon waking in the morning. As a result of the pain, she is having difficulty with ambulation.
She denies any trauma but had returned the night prior from a long overseas flight. She has no significant
past medical history and takes no medications or over-the-counter supplements.Duplex ultrasound
demonstrates an occlusive thrombus in the right iliofemoral vein.

What is the most appropriate next step?

A. Warfarin 5 mg daily with goal international normalized ratio 2-3.
B. Rivaroxaban 20 mg daily.
C. Apixaban 10 mg BID.
D. Dabigatran 150 mg BID.
E. Catheter-directed thrombolysis.

Initial therapy for deep venous thrombosis (DVT) generally involves anticoagulation, both to prevent
extension of the clot or embolism (i.e., pulmonary embolus) and to decrease the late sequelae of DVT,
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including post-thrombotic syndrome and chronic thromboembolic pulmonary hypertension. The risk of
complications due to DVT is higher in proximal DVT; therefore, there is a stronger indication to
anticoagulate in the setting of a proximal DVT (i.e., popliteal, femoral, and iliac veins). Furthermore, the
mortality rate in proximal DVT is significantly higher than in distal DVT (i.e., below the knee).

There are numerous options for anticoagulation in this setting and the decision regarding the appropriate
agent has to take many factors into account, including comorbid conditions, bleeding risk, need for
reversal options, cost, convenience, etc. In a patient without significant comorbid conditions (i.e., renal or
hepatic impairment) or cancer, factor Xa inhibitors and direct thrombin inhibitors are generally preferred
over a vitamin K antagonist.

Apixaban 10 mg twice daily is the correct answer. This dose should be given for 7 days followed by 5 mg
twice daily.

Rivaroxaban is incorrect, as the dose listed is not appropriate for treatment of DVT, which would be 15
mg BID for 21 days followed by 20 mg daily.

Dabigatran as an initial choice is incorrect, as this should be given after 5-10 days of parenteral treatment
with low molecular weight heparin (LMWH).

Warfarin without a LMWH bridge is incorrect, as it could take a week to achieve a therapeutic
international normalized ratio.

At this point, there is no clinical indication for catheter-directed thrombolysis or inferior vena cava filter
insertion.
Answer: C
Key Point
Venous thromboembolism, including deep vein thrombosis and pulmonary embolism, is the third most
common cause of cardiovascular morbidity and mortality after myocardial infarction and stroke.

Question 10:
A 30-year-old woman is referred to your clinic because of a history of deep venous thrombosis (DVT). A
year prior, she had been treated for an unprovoked left lower extremity DVT. Her medical history is
significant for varicose veins. Her medications include an oral contraceptive. There is no family history of
pulmonary embolism or DVT.

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Her vital signs show temperature 98.1°F, blood pressure 120/80 mm Hg, heart rate 88 bpm, and oxygen
saturation 99% on room air. The left lower extremity is mildly swollen to the mid thigh and varicose
veins are noted, although the remainder of the examination is unremarkable.

What is the best next step?

A. Contrast venography of the lower extremities.
B. No further testing.
C. Magnetic resonance venography of the pelvis.
D. Transthoracic echocardiography.
E. Hypercoagulable panel.

May-Thurner syndrome is a rarely diagnosed condition in which patients develop iliofemoral DVT due to
an anatomical variant in which the right common iliac artery overlies and compresses the left common
iliac vein against the lumbar spine. This variant has been shown to be present in >20% of the population
and so should be considered in the differential diagnosis of DVT, particularly in patients with other risk
factors. Failure to correct the anatomic substrate can lead to recurrent DVTs.

Risk factors for May-Thurner syndrome include left rather than right lower extremity DVTs, scoliosis,
female sex, and oral contraceptive use or recent pregnancy. Patients may have left lower extremity
swelling in the absence of DVT, as in this case.

Hypercoagulable workup after first-provoked DVT is not recommended. DVT is a very common
disorder, and recent discoveries of clotting abnormalities have led to increased testing without proven
benefit.

The presentation with chronic unilateral leg swelling is not consistent with heart failure.

Although contrast venography is the gold standard for diagnosis, it is an invasive test and has been
replaced by computed tomography or magnetic resonance imaging.
Answer: C
Key Point
The May-Thurner syndrome should be recognized clinically in a young adult with left leg swelling and
deep vein thrombosis from extrinsic compression of the left common iliac vein by the right common iliac
artery.

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Question11:
A 65-year-old man presents with transient numbness of his left hand and forearm a few days prior. He has
hypertension, hyperlipidemia, and ongoing tobacco abuse. His symptoms resolve within a few hours and
he feels well at the time of presentation. He is actively exercising for 30 minutes several times a week
without any symptoms. His vital signs are heart rate 70 bpm, blood pressure 132/80 mm Hg, and
respirations 12 per minute. His examination is notable for normal carotid upstrokes with no bruits, regular
rhythm with no murmurs, and a nonfocal neurologic examination. He is on aspirin 81 mg daily, lisinopril
10 mg daily, hydrochlorothiazide 25 mg daily, and atorvastatin 10 mg daily.

Which of the following is the most appropriate next step?

A. Transesophageal echocardiography.
B. Ambulatory blood pressure monitor.
C. Reassurance.
D. Carotid ultrasound.
E. Exercise treadmill test.

The patient presents with symptoms concerning for a transient ischemic attack with multiple risk factors
for carotid artery disease. The absence of a carotid bruit is not sufficient to exclude significant carotid
stenosis, which if present would warrant treatment. His symptoms are not typical for angina and he
reports no exertion symptoms. An ambulatory blood pressure monitor would not provide additional
diagnostic information since he already carries a diagnosis of hypertension.

Transesophageal echocardiography (TEE) could be considered as either an initial or supplemental test for
evaluation for cardiovascular source of embolus with no identified noncardiac source, but carotid disease
should be ruled out before proceeding to TEE.
Reassurance without diagnosis or explanation of the symptoms would be inappropriate.
Answer : D
Key Point
Auscultation of carotid bruits has limited clinical utility in the detection of clinically significant carotid
artery stenosis.

Question 12:

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A 65-year-old woman with a history of hypertension, peripheral arterial disease, status/post right
superficial femoral artery stenting, active tobacco abuse, and chronic obstructive pulmonary disease
presents for further evaluation of dull abdominal pain after meals over the prior year associated with 20
lbs of weight loss. She denies night sweats, hematemesis, melena, or hematochezia.
Which of the following is most likely to yield the diagnosis?
A. Right upper quadrant ultrasound.
B. Vasodilator myocardial perfusion imaging.
C. Colonoscopy.
D. Gastric emptying scintigraphy.
E. Abdominal computed tomography angiography.

The American College of Radiology appropriate use guidelines recommend computed tomography
angiography for diagnostic evaluation of suspected mesenteric ischemia. The patient's symptoms are
characteristic of mesenteric ischemia and she has the typical demographic and risk-factor profiles (female,
>60 years of age, smoker, other vascular disease).
A stress test to assess for coronary ischemia may be positive given her other vascular disease, but would
not be the cause of her presenting symptoms and weight loss.
A right upper quadrant ultrasound would not diagnose stenoses of the mesenteric vessels; however, an
abdominal ultrasound with Doppler could be a useful screening test in this patient.
A gastric emptying scintigraphy study would not be of likely diagnostic utility because it evaluates for
diabetic gastroparesis and the patient does not have diabetes mellitus. Similarly, a colonoscopy is not the
best test to diagnose mesenteric ischemia.
Answer: E
key Point
Symptoms of chronic mesenteric ischemia are often insidious. Classic symptoms include postprandial
abdominal pain, food avoidance, and weight loss. Weight loss is an essential component of clinically
significant chronic mesenteric artery disease.

Question 13 :
A 74-year-old man presents to your office for evaluation. He has had right calf pain on ambulation for the
prior year, presently worsening to the point that he has aching pain even at rest, worse when he elevates
his leg. He has a history of coronary artery disease status/post bypass surgery 5 years prior, hypertension,
dyslipidemia, diabetes mellitus, and osteoarthritis. His medications include aspirin 81 mg daily,
atorvastatin 80 mg daily, metoprolol XL 25 mg daily, lisinopril 40 mg daily, and hydrochlorothiazide 25
mg daily.

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On examination, his heart rate is 74 bpm and blood pressure is 128/70 mm Hg. Lungs are clear, heart is
regular without murmurs, and abdomen is soft and nontender. The appearance of his right foot is shown
in Figure 1.
Ankle-brachial indices (ABIs) are 0.62 on the right and 0.95 on the left.

Which of the following is the best next step?

A. Toe-brachial index measurement.
B. Iliofemoral invasive angiography with run-off.
C. Exercise treadmill ankle-brachial index measurement.
D. Transcutaneous oxygen pressure measurement.
E. Magnetic resonance angiography of the lower extremities.
Critical limb ischemia (CLI) is a condition characterized by chronic (>2 week) ischemic rest pain,
nonhealing wound/ulcers, or gangrene in one or both legs attributable to objectively proven arterial
occlusive disease. This patient has the classic risk factors of coronary artery disease and diabetes mellitus,
as well as rest pain with ulcers and dependent rubor.
The diagnosis of CLI is a constellation of both symptoms and signs. In patients with a history or physical
examination suggestive of peripheral artery disease (PAD), the ABI has good validity as a first-line test in
the diagnosis of PAD. Patients with ABI ≤0.90 are diagnosed with PAD.
By definition, CLI results from extensive PAD that limits tissue perfusion. Because timely diagnosis and
treatment are essential to preserve tissue viability in CLI, it is often most effective and expeditious to
pursue invasive angiography with endovascular revascularization directly, without delay and potential
risk of additional noninvasive imaging.

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Those with ABI 0.91-0.99 may possibly have PAD and should undergo exercise ABI if the clinical
suspicion of PAD is significant. Exercise ABI can differentiate claudication from pseudoclaudication in
individuals with exertional leg symptoms. If the post–exercise treadmill ABI is normal, alternative causes
of leg pain are considered.
ABI values >1.40 indicate that the arteries are not able to be compressed, which is more common among
individuals with diabetes mellitus and/or advanced chronic kidney disease. Toe-brachial index (TBI) is a
noninvasive test that is useful to evaluate for PAD in patents with noncompressible arteries, which cause
an artificial elevation of the ABI. A TBI ≤0.70 is abnormal and diagnostic of PAD because the digital
arteries are rarely noncompressible.
In patients with normal (1.0-1.4) or borderline (0.91-0.99) ABI in the setting of nonhealing wounds or
gangrene, it is reasonable to diagnose CLI by using transcutaneous oxygen pressure or skin perfusion
pressure. If perfusion measures are normal or only mildly impaired, alternative causes of the nonhealing
wounds are considered.
Duplex ultrasound, computed tomography angiography, or magnetic resonance angiography of the lower
extremities is useful to diagnose anatomic location and severity of stenosis for patients with symptomatic
PAD in whom revascularization is considered. However, as noted earlier, in the case of CLI, invasive
angiography is the best step because imaging studies cause unnecessary delay when the goal is prompt
revascularization and limb salvage.
Answer : B
Key Point
Treatment of critical limb ischemia revolves around the following key principles:
Revascularization should be performed when possible to establish in-line flow.
The method of revascularization (surgical or endovascular) is best decided by an interdisciplinary care
team and depends on a variety of anatomic and clinical features.
Regular wound care after revascularization is vital to achieving complete wound healing and preserving
the functioning foot.

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Question 14:
A 75-year-old man presents to the emergency department (ED) with a 30-minute episode of right arm
tingling and weakness. By the time he arrives at the ED, his symptoms have resolved. His history
includes hypertension and dyslipidemia. His medications include aspirin 81 mg daily, amlodipine 5 mg
daily, and atorvastatin 40 mg daily.On examination, he is alert but anxious. His heart rate is 92 bpm and
blood pressure is 146/80 mm Hg. There are no carotid bruits. Jugular venous pressure is 4 cm H20. Lungs
are clear, heart is regular with no murmurs, and extremities are warm without edema. His neurologic
examination shows no deficits.
Laboratories reveal creatinine 1.2 mg/dL and low-density lipoprotein cholesterol (LDL-C) 120 mg/dL. An
electrocardiogram shows normal sinus rhythm with no ST-T changes. A carotid ultrasound demonstrates
an 80-90% left internal carotid artery stenosis and a 50% right carotid artery stenosis.
What is the best next step?
A. Left carotid to subclavian artery bypass.
B. Change atorvastatin to evolocumab.
C. Staged left then right carotid endarterectomy.
D. Add ticagrelor.

The 2011 multisociety Guideline on the Management of Patients With Extracranial Carotid and Vertebral
Artery Disease states that patients at average or low surgical risk who experience nondisabling ischemic
stroke or transient cerebral ischemic symptoms within 6 months (symptomatic patients) should undergo
carotid endarterectomy (CEA) if the diameter of the lumen of the ipsilateral internal carotid artery is
reduced >70% as documented by noninvasive imaging or >50% as documented by catheter angiography,
and the anticipated rate of perioperative stroke or mortality is <6%.

This patient has symptomatic carotid stenosis, and left internal carotid artery disease would explain his
right-sided symptoms. Thus, the correct answer is left CEA. Carotid artery stenting (CAS) is indicated as
an alternative to CEA for symptomatic patients at average or low risk of complications associated with
endovascular intervention, but the decision to proceed with CEA versus CAS is often deferred to the
treating clinicians.

Right CEA is not indicated because the stenosis is not significant by ultrasound (70% or greater is
significant by ultrasound).

Carotid to subclavian artery bypass may be used to treat symptomatic subclavian artery stenosis, but is
not indicated for patients with symptomatic carotid stenosis.

His LDL-C is elevated despite atorvastatin therapy. Intensifying his medical therapy with higher doses of
statin and/or ezetimibe would be undertaken before changing to a proprotein convertase subtilisin/kexin
type 9 (PCSK9) inhibitor.

Dual antiplatelet therapy with clopidogrel after ischemic stroke is recommended (Class IIa), but there is
no role for ticagrelor for ischemic stroke patients (Class III).
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Answer : E
Key Point
Carotid endarterectomy is the mainstay of treatment in patients suffering from transient ischemic attack or
ischemic stroke within the past 6 months secondary to moderate to severe (≥50% stenosis) extracranial
carotid artery stenosis (Class I). Endovascular stenting is an alternative option for revascularization in
such patients (Class IIa). In contrast, surgical or endovascular therapies should be reserved for highly
selected cases with extracranial vertebral artery stenosis, as there is no evidence of benefit from
randomized studies in this population.

Question 15 :
A 66-year-old man presents to your office 1 week after coronary artery stenting for angina. Angiography
is performed via the right femoral approach with good results. He has a history of peripheral arterial
disease status/post left femoral-popliteal bypass 2 years prior.
He reports that his chest discomfort is better, but for the prior 3 days, he has had a low-grade fever and
myalgias, as well as has noted a rash on his legs. His medications include aspirin 81 mg daily, clopidogrel
75 mg daily, atorvastatin 80 mg daily, metoprolol XL 25 mg daily, and lisinopril 10 mg daily.
On examination, temperature is 100.4°F, heart rate is 68 bpm, and blood pressure is 132/80 mm Hg.
Lungs are clear, heart is regular with no murmurs, abdomen is soft, and extremities are warm with a 1+
right posterior tibial pulse and a 2+ left posterior tibial pulse. The right groin has no hematoma, thrill, or
bruit. Examination of his legs is shown in Figure 1.
Laboratories demonstrate potassium 5.1 mEq/L, creatinine (Cr) 2.3 mg/dL (Cr 1.2 mg/dL prior to
angiogram), and white blood cell count 14.6/mcL.

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What is the best next step?

A. Order a skin biopsy.
B. Continue atorvastatin 80 mg daily.
C. Start intravenous unfractionated heparin.
D. Check urinalysis.
E. Check a right lower extremity arterial ultrasound.
his patient's clinical presentation is classic for cholesterol emboli syndrome (CES): a patient with vascular
risk factors develops nonspecific symptoms along with skin findings of livedo reticularis and renal failure
after angiogram. CES is a rare arterio-arterial embolization syndrome that occurs when cholesterol
crystals located in an atherosclerotic plaque in a large-caliber artery (typically the aorta) embolize to
small- or medium-caliber arteries, which then results in end-organ damage secondary to either mechanical
obstruction and/or inflammatory response.

CES is a manifestation of generalized atherosclerosis. Cholesterol crystal emboli that arise from the
descending thoracic aorta may lead to renal failure, mesenteric ischemia, and emboli to the skeletal
muscles and skin. Cutaneous manifestations of CES include livedo reticularis (as shown in Figure 1),
gangrene, cyanosis, ulceration, nodules, and purpura.

In addition to specific signs of end-organ damage, CES is often characterized by systemic signs and
symptoms due to nonspecific acute inflammatory response.

Usually, the diagnosis of CES is established clinically based on signs and symptoms that are specific to
affected vascular beds and often in patients with a recent history of imaging or surgical procedures
involving the aorta or its large branches. A typical patient is a middle-aged or elderly male with risk
factors for atherosclerosis, such as systemic hypertension, diabetes mellitus, hypercholesterolemia, and a
history of tobacco use.

Treatment for CES is generally supportive and focuses on prevention of recurrent episodes of cholesterol
emboli. In nonrandomized trials, statin therapy reduces the risk of CES.

Diagnosis of CES is clinical; therefore, a skin biopsy or urinalysis would not change management.

A right lower extremity ultrasound would be normal because the patient has intact distal pulses, the
cholesterol emboli target smaller arteries, and pulses are generally preserved in CES.

The use of heparin, warfarin, or thrombolytic agents has been associated with the development of CES,
although a causal relationship has never been proven. Thus, intravenous heparin is not indicated unless
there is another specific reason, such as atrial fibrillation, to initiate anticoagulation.
Answer: B
Key Point
The majority (>70%) of atheroembolism is iatrogenic, occurring as a complication of catheter-based
angiography or vascular surgery.
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Question 16 :
A 60-year-old man presents for elective surgical revascularization of coronary artery disease with angina.
His past medical history includes diabetes mellitus, hypertension, and hyperlipidemia. He is a life-long
nonsmoker. His medications include aspirin 81 mg, valsartan 240 mg daily, insulin, atorvastatin 80 mg,
metoprolol 200 mg extended release, and isosorbide mononitrate 60 mg daily. His vital signs are pulse 60
bpm, blood pressure 120/60 mm Hg, and respirations 12 per minute. There is no carotid bruit. Cardiac
examination reveals regular rhythm; there are no murmurs, rubs, or gallops. Peripheral pulses are 2+
throughout.

Laboratory evaluation is normal. Coronary angiogram reveals 90% proximal left anterior descending
artery, 80% proximal circumflex, and 70% mid right coronary artery stenoses. Left ventricular ejection
fraction is normal with mild mitral regurgitation on echocardiogram.
Which of the following is most appropriate prior to coronary artery bypass graft (CABG) surgery?
A. Carotid duplex ultrasound.
B. Computed tomography angiography of the neck.
C. Dental evaluation.
D. Ankle-brachial index.
E. No further testing.

Carotid duplex ultrasound screening is reasonable before CABG in patients >65 years of age and in those
with left main coronary artery stenosis, peripheral artery disease (PAD), history of smoking, history of
stroke/transient ischemic attack, or carotid bruit. This patient has none of these risk factors and so carotid
duplex ultrasound is not indicated.

Computed tomography angiography of the neck is not indicated without signs or symptoms of
extracranial cerebral arterial disease.

Dental evaluation may be considered for patients undergoing valve surgery but is not routinely performed
in patients without dental complaints prior to CABG.

Screening for PAD prior to CABG, such as ankle-brachial index, is not indicated in the absence of signs
or symptoms of arterial insufficiency.
Answer: E

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Key Point
There is little evidence to support the notion that asymptomatic carotid disease is an important source of
stroke in coronary artery bypass grafting (CABG) surgery patients. It may be reasonable to defer routine
revascularization at the time of CABG.

Question 17:
A 75-year-old woman presents to the emergency department with severe substernal chest pain. Her past
medical history includes hypertension, dyslipidemia, and prior carotid endarterectomy. Her medications
are aspirin 81 mg daily, rosuvastatin 40 mg, carvedilol 6.25 mg BID, and hydrochlorothiazide 25 mg
daily. Her vital signs are heart rate 98 bpm and blood pressure 169/102 mm Hg. Her physical examination
reveals diaphoresis, a soft ejection systolic murmur, and palpable pedal pulses.

Electrocardiography reveals sinus rhythm with left ventricular hypertrophy but no acute ischemic
changes. Troponins are normal. A chest X-ray is unremarkable.

A chest computed tomography (CT) demonstrates a crescentic, high attenuation area measuring
approximately 3 mm in diameter in the posterior ascending aorta that does not enhance with contrast.
There is no involvement of the great vessels or the aortic arch. There is no evidence of an intimal flap or
compression of the lumen on this study. There is no pericardial effusion.
What is the best next step in this patient's management?
A. Emergent surgical consultation.
B. Coronary angiography.
C. Urine toxicology.
D. Intravenous hydralazine.
E. Transesophageal echocardiography.

The scenario describes a patient with ongoing symptoms due to an acute aortic syndrome. The CT report
is suggestive of a type A intramural hematoma (IMH) and no evidence of intimal disruption. The
definitive treatment for this pathology is emergent surgical repair due to the high risk of aortic rupture.
The mortality of medical treatment alone may reach ≤40%. Hence, the most important next step is
emergent cardiothoracic surgical consultation.

Intervention in the event of hemodynamic compromise is incorrect, as this may signify a life-threatening

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extension, including aortic rupture or dissection leading to tamponade.

Coronary angiography would delay definitive therapy and carries risks of worsening the acute aortic
syndrome.

Urine toxicology prior to beta-blocker therapy may be considered if cocaine use is suspected in patients
with hypertensive disorders and chest pain, but would not be the best choice with the imaging findings in
this case.

The patient should undergo aggressive blood pressure control. Vasodilating drugs including hydralazine
should not be given prior to adequate beta-blockers (Class III recommendation). Echocardiography is
reasonable to evaluate for progression to aortic dissection with involvement of the aortic valve
architecture. However, all these steps are adjuncts in management, with the most important step being
emergent surgical repair.
Answer : A
Key Point
Acute aortic syndromes comprise a spectrum of disease entities that threaten central aortic pressure, vital
organ perfusion, survival, and functional recovery. These include aortic dissection, intramural hematoma,
penetrating aortic ulcer, rapid aneurysm expansion, and trauma-induced aortic rupture. Aortic dissection
is most commonly described according to the DeBakey (I, II, III) or Stanford (type A, type B)
classification schemes.

Question 18 :
A 68-year-old man with a past medical history of hypertension, hyperlipidemia, insulin-dependent
diabetes mellitus (type 2), and tobacco use presents to your office with 3 weeks of right foot and calf pain
unresponsive to over-the-counter analgesics. The pain is worse with ambulation. He has had a small sore
on his right great toe for the prior 6 months.

His medications at the time of presentation include aspirin, lisinopril, atorvastatin, metformin, and insulin.
On physical examination, his blood pressure is 128/72 mm Hg, pulse is 76 bpm, and oximetry is normal.
Pedal pulses are nonpalpable but Dopplerable in the right leg. The right toe has a dry, black eschar at the
tip and a 1 x 1 cm, foul-smelling wound on the dorsum without purulent drainage.
Which of the following is the most appropriate next step in his treatment?
A. Referral for revascularization.
B. Toe amputation.
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C. Cilostazol.
D. Supervised exercise program.
E. Computed tomography angiography.

This patient is presenting with critical limb ischemia (CLI), as evidenced by his symptoms of rest pain for
>2 weeks, a nonhealing ulcer and dry gangrene on the right toe, and the need for prompt
revascularization. Prior to revascularization, it is essential to have an anatomic assessment with
ultrasound, magnetic resonance angiography, or computed tomography angiography (CTA). Thus, CTA
is the correct answer, which may help to determine the best options for revascularization (i.e., surgical vs.
percutaneous).

When evaluating a patient with CLI, it is important to differentiate between viable, threatened, and
nonviable (Figure 1).

I. Viable: limb not immediately threatened; no sensory loss; no muscle weakness; audible arterial and
venous Doppler.
II. Threatened: mild to moderate sensory or motor loss; inaudible arterial Doppler; audible venous
Doppler.
III. Irreversible: major tissue loss or permanent nerve damage inevitable; profound sensory loss,
anesthetic; profound muscle weakness or paralysis (rigor); inaudible arterial and venous Doppler.

Patients with viable and threatened should undergo urgent revascularization. Primary amputation is
reserved for patients with irreversible leg ischemia. In this case, amputation of the toe would not be
indicated in the presence of a Dopplerable pulse, which suggests potential viability.

Cilostazol may help with symptom management in claudication but has no role in the management of
CLI.

Supervised exercise is beneficial in patients with symptomatic claudication but is not useful in the
management of patients with CLI.

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Answer: E
Key Point
Anatomic assessment should be performed when revascularization is planned with either duplex
ultrasound, CTA, MRA or invasive angiography.

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Question 19 :
A 38-year-old woman presents to your clinic for evaluation of bilateral arm pain. She describes a feeling
of "heaviness" in both arms when carrying groceries or picking up her young children. She also reports
lightheadedness and neck pain. For the prior few months, she has also had poor appetite, leading to
weight loss of 12 lbs and drenching night sweats and fatigue. She has no past medical history and takes
no medicines.

A physical examination reveals blood pressure 106/70 mm Hg in the right arm and 90/66 mm Hg in the
left arm. Pulse is 77 bpm. Height is 5'6" and weight is 118 lbs. There is a bruit audible over the left
clavicular fossa. Left brachial and radial pulses are not palpable; right brachial and radial pulses are 1+.
Computed tomographic angiography of the chest and neck would most likely show which one of the
following?
A. Thoracic aortic aneurysm.
B. Atherosclerosis of the left subclavian.
C. Thickening of the aortic wall.
D. Thoracic aortic dissection.
E. Aneurysms of multiple arteries and veins.

Takayasu arteritis, also known as pulseless disease, is an idiopathic vasculitis of the elastic arteries,
involving the aorta and its branches. The diagnosis of Takayasu arteritis may be made using the 1990
American College of Rheumatology criteria: 1) age of onset <40 years; 2) intermittent claudication; 3)
diminished brachial artery pulse; 4) subclavian artery or aortic bruit; 5) systolic blood pressure variation
of >10 mm Hg between arms; and 6) angiographic (computed tomography, magnetic resonance) evidence
of aorta or aortic branch vessel stenosis (Figure 1). When three of the criteria are manifest, the sensitivity
and specificity for diagnosis are 90.5% and 97.8%, respectively. Markers of inflammation, such as C-
reactive protein and erythrocyte sedimentation rate, are elevated in approximately 70% of patients in the
acute phase and 50% in the chronic phase of disease. Treatment is with high-dose steroids to reduce
inflammation; elective revascularization of stenosed branch vessels should be delayed until acute
inflammation has resolved.

The absence of risk factors for atherosclerotic disease and young age make atherosclerotic aneurysm of
the aorta unlikely; atherosclerotic aneurysmal disease would not explain the subacute constitutional
symptoms in this case. She has normal stature and no signs to suggest a syndromic disease affecting the
thoracic aorta to cause aneurysm or dissection.

Small aneurysms at multiple sites and affecting both arteries and veins describes the typical appearance of
Behçet's disease. The diagnostic criteria were established by the International Group for Behçet's disease
and require oral ulceration and two of these three lesions: recurrent genital ulceration, uveitis or retinal
vasculitis, or skin lesions, such as erythema nodosum, pseudofolliculitis, or pathergy. In addition to these
cardinal manifestations, vascular involvement may occur in one-third of patients. Because she has no
other manifestations, Behçet's is not the preferred answer choice.
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Answer: C

Key Point
Takayasu disease should be suspected when there is inflammation of the aortic arch, inflammation, and/or
stenosis of its branches in patients <50 years of age.

Question20:
For which of the following patients would temporal artery biopsy be most likely to yield the correct
diagnosis?
A. A 45-year-old woman with painless, netlike mottling of the legs and thighs.
B. A 64-year-old man with receding hairline and hypothenar atrophy.
C. A 78-year-old woman with headaches, jaw fatigability with chewing, and shoulder stiffness.
D. A 38-year-old man with history of tobacco use and ulcers on his fingertips.
E. A 28-year-old Asian woman with mild fevers and arm fatigue when brushing her hair.

The 78-year-old woman has typical symptoms of giant cell arteritis. The diagnosis may be confirmed by
temporal artery biopsy. The 28-year-old woman has symptoms suggestive of subclavian artery stenosis
and belongs to a demographic group (young woman of Asian descent) for which Takayasu arteritis seems
most likely. The 38-year-old man has signs and history consistent with Buerger's disease (thromboangiitis
obliterans), for which the most important therapy is smoking cessation. The 45-year-old woman has
livedo reticularis, which in isolation does not prompt further workup. The 64-year-old man has male-
pattern balding and likely an ulnar nerve lesion, for which temporal artery biopsy is not indicated.
Answer: C
Key Point
Giant cell arteritis should be suspected in patients >50 years of age with constitutional symptoms and new
onset headache or visual disturbances, or in those with an isolated large-vessel vasculitis.

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Question21:
An 80-year-old man is brought to the emergency department due to slurred speech. On physical
examination, he has left upper and lower extremity motor weakness with an unsteady gate. National
Institutes of Health Stroke Scale (NIHSS) is 3. Head computed tomography reveals a single old lacunar
infarct with no evidence of acute infarct or bleed. Electrocardiography shows sinus rhythm at 62 bpm
with left ventricular (LV) hypertrophy and nonspecific ST changes. Carotid ultrasound shows <50%
stenosis of the bilateral internal carotid arteries. His echocardiogram shows normal LV function, mild LV
hypertrophy, aortic sclerosis, and left atrial enlargement.
Which of the following is the most appropriate strategy for secondary prevention of stroke in this patient?
A. Clopidogrel.
B. Aspirin.
C. Rivaroxaban.
D. Aspirin and clopidogrel.
E. Aspirin and warfarin.

Clopidogrel monotherapy and aspirin–extended-release dipyridamole are of equal efficacy in secondary

prevention of ischemic stroke; clopidogrel is better tolerated and, therefore, may be preferred. The
combination of aspirin and clopidogrel is not recommended over monotherapy with either one of these
antiplatelet agents.

The PRoFESS (Prevention Regimen for Effectively Avoiding Second Strokes) trial showed that
clopidogrel monotherapy and aspirin–extended-release dipyridamole have similar benefits for secondary
stroke prevention, but the risk of bleeding was higher in the aspirin–extended-release dipyridamole arm.

There is no indication for anticoagulation therapy within the given vignette, such as atrial fibrillation, LV
thrombus, multiembolic stroke, or significant aortic atheroma. Hence, rivaroxaban or aspirin plus warfarin
are incorrect.
Answer: A

Key Point
Clopidogrel monotherapy and aspirin/extended-release dipyridamole are of equal efficacy to aspirin
monotherapy in secondary prevention of ischemic stroke; clopidogrel is better tolerated and therefore may
be preferred. The combination of aspirin and clopidogrel is not recommended over monotherapy with
either one of these antiplatelet agents.

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Question22:
A 70-year-old man presents with sudden severe substernal chest pain radiating to his back. His past
medical history includes hypertension and hyperlipidemia. He smokes one pack per day. His medications
include amlodipine 10 mg daily, lisinopril 20 mg daily, hydrochlorothiazide 25 mg daily, and aspirin 81
mg. His vital signs are temperature 98.6°F, blood pressure 170/90 mm Hg in both arms, and heart rate
110 bpm. Oxygen saturation is 93% on room air. An examination reveals an uncomfortable man in
moderate distress. There are bilateral carotid bruits and decreased lower extremity pulses.
Which of the following findings on chest computed tomography aortography is associated with the
highest mortality in this patient?
A. Partial thrombosis of the false lumen.
B. Patency of the false lumen.
C. Complete thrombosis of the false lumen.
D. Dissection flap involving the sinuses of Valsalva.
E. Extent of coronary artery calcifications.

Type A aortic dissection, which involves the aortic root, has estimated mortality 1-2% per hour in the first
24 hours and 50% in the first 48 hours, without operation.

Type B acute aortic dissection has lower mortality, with approximately 8% in-hospital mortality and 60-
80% survival at 5 years. Partial thrombosis of the false lumen, as compared with complete patency, is a
significant independent predictor of postdischarge mortality in patients with type B dissection, but type B
aortic dissections have lower mortality overall than type A dissections.

Coronary artery disease is common in patients with aortic dissection but has less impact on mortality than
proximal aortic dissection.
Answer: D
Key Point
Acute type A aortic dissection is a surgical emergency.

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Question23:
An 82-year-old woman presents with nonvalvular atrial fibrillation, poorly controlled hypertension,
diabetes mellitus, history of stroke, stage IV chronic kidney disease (CKD), and a recent subdural
hematoma (SDH) resulting from head trauma in the setting of a fall at home 3 months prior. She is
accompanied by her daughter, who expresses concern regarding frequent falls at home.
The patient was on warfarin at the time of the fall and had an international normalized ratio (INR) of 2.8.
Warfarin was discontinued at that time and she had since been started on aspirin (ASA) 325 mg daily. A
repeat head computed tomography performed 1 month after her initial fall demonstrated improvement in
the size of the hematoma.
Which of the following is the most appropriate next step in management?
A. Resume warfarin, goal international normalized ratio 2.
B. Continue aspirin 325 mg daily.
C. Discuss percutaneous left atrial appendage occlusion.
D. Decrease aspirin dose to 81 mg daily.
E. Discontinue aspirin, start apixaban.
This patient has nonvalvular atrial fibrillation with a CHA2DS2-VASc score of 7 and a HAS-BLED score
of 5. Based on her annual stroke risk, she requires a long-term stroke prevention strategy. Unfortunately,
she is at high risk of recurrent bleed with oral anticoagulant use given her history of frequent falls. Thus,
the best option for this patient at high risk of both stroke and bleeding is to consider left atrial appendage
occlusion. A shared decision-making discussion with a clinician who does not perform the device
implantation is warranted prior to proceeding with left atrial appendage occlusion.

ASA monotherapy (325 mg or 81 mg) daily provides inadequate protection from a stroke; therefore,
neither is a correct answer.

This particular patient is not a good long-term anticoagulation candidate due to the risk of recurrent
intracranial bleeding. For those on warfarin for stroke prevention, the goal INR is 2.5.
Changing to a direct oral anticoagulant (DOAC; e.g., apixaban) might be reasonable in a patient on
warfarin with a history of labile INRs. However, her INR was within range at the time of her SDH and
there is no history of labile INRs. Furthermore, she has stage IV CKD and is therefore not a candidate for
treatment with a DOAC.
Answer: C
Key Point
Anticoagulant-related intracranial hemorrhages have a very high mortality (about 50% both for warfarin
and direct oral anticoagulants); thus, the familiarity of the cardiologist with these conditions is essential to

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identify the optimal preventive approaches, especially when alternatives exist such as left atrial
appendage closure for nonvalvular atrial fibrillation.

Question 24:
A 78-year-old man with a history of coronary artery disease, hypertension, and hyperlipidemia is admitted
to the intensive care unit with an acute ischemic stroke. He presents with right upper extremity weakness
of unknown duration and thus does not receive fibrinolytic therapy. His medications prior to admission
include aspirin 81 mg daily, atorvastatin 40 mg daily, amlodipine 5 mg daily, and metoprolol succinate 25
mg daily.
His heart rate is 68 bpm and blood pressure is 140/85 mm Hg. On examination, he is alert and oriented,
with a persistent focal neurologic deficit in his right upper extremity.
Electrocardiogram demonstrates sinus rhythm at a rate of 70 bpm with left ventricular hypertrophy.
Which of the following do you recommend?
A. Initiate ticagrelor 90 mg BID.
B. Initiate warfarin 5 mg tonight.
C. Anticoagulation is not indicated.
D. Initiate heparin infusion to goal partial thromboplastin time of 70-90 sec.
E. Initiate dabigatran 150 mg BID.

Anticoagulation for acute ischemic stroke of unclear etiology is a Class III recommendation.
Anticoagulation is only indicated in the presence of atrial fibrillation or thromboembolic disease. Thus,
heparin, warfarin, and dabigatran would not be indicated.

There is no clinical evidence for the use of ticagrelor in stroke.

Answer: C
Key Point
Anticoagulation increases the risk of potentially fatal intracranial hemorrhages and other major bleeds, so
it should only be used in patients with an approved indication who do not have contraindications to its
use. Nonvalvular atrial fibrillation and deep vein thrombosis are the two main indications for warfarin and
direct oral anticoagulants. Only warfarin with or without aspirin is indicated for patients with mechanical
heart valves.

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Question 25:
A 78-year-old man with diabetes mellitus, tobacco use, chronic kidney disease (stage III), and
atherosclerotic cardiovascular disease (ASCVD) is admitted with a non–ST-segment elevation myocardial
infarction and undergoes coronary angiography and stenting to the distal right coronary artery. Following
catheterization, he complains of discomfort and discoloration of his feet.

His medications include aspirin 81 mg daily, clopidogrel 75 mg daily, atorvastatin 40 mg daily,

metoprolol tartrate 25 mg every 6 hours, and lisinopril 5 mg daily.

On examination, his pulse is 60 bpm and blood pressure is 132/78 mm Hg. Cardiac auscultation reveals a
regular rhythm and no murmur, and lungs are clear. Creatinine has increased to 2.0 from a baseline of 1.5
mg/dL. Figure 1 shows the presentation of his foot.

Which of the following is the most appropriate next step in management?

A. No additional medical therapy.
B. Toe biopsy.
C. Computed tomography angiogram of the legs.
D. Unfractionated heparin infusion.
E. Apixaban 5 mg BID.

This patient had cholesterol embolization following cardiac catheterization, as evidenced by acute renal
failure and blue-toe syndrome, which are manifestations of embolization. This tends to occur in patients
with typical risks factors for atherosclerosis (smoking, older age) following cardiac catheterization or
other vascular procedures. The incidence of cholesterol embolization is not known, as it is often not
recognized. Management of cholesterol embolization syndrome is focused on aggressive secondary
prevention with aspirin, statin therapy, smoking cessation, and blood pressure control. Otherwise,
management of microvascular obstruction depends on the vascular bed affected but is generally
supportive. This patient is already on aspirin and a high-intensity statin.
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There is a lack of data from randomized trials to support the use of anticoagulation in the setting of
cholesterol emboli syndrome. Therefore, using unfractionated heparin or apixaban are incorrect options.
Furthermore, apixaban should not be used when renal function is unstable.

Biopsy is the only way to definitely confirm the diagnosis of cholesterol emboli syndrome; however, this
is generally for clinical situations where the diagnosis is unclear. This patient has typical risk factors for
ASCVD, typical symptoms of cholesterol embolization, and recently underwent a procedure placing him
at risk.

Computed tomography angiogram is relatively contraindicated in this setting due to acute kidney injury
and would be unlikely to change management.
Answer: A
Key Point
The majority (>70%) of atheroembolism is iatrogenic, occurring as a complication of catheter-based
angiography or vascular surgery.

Question 26:
A 62-year-old man with a history of hypertension, hyperlipidemia, type II diabetes mellitus, tobacco
abuse, and chronic obstructive pulmonary disease presents with progressive claudication over the
previous few months.

Blood pressures are as follows:

Left arm: 140/90 mm Hg

Right arm: 150/92 mm Hg
Left leg: 120/88 mm Hg
Right leg: 130/86 mm Hg
What is the correct ankle-brachial index (ABI) in this patient?
A. Left leg ankle-brachial index 0.83; right leg ankle-brachial index 0.90.
B. Left leg ankle-brachial index 1.2; right leg ankle-brachial index 1.12.

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C. Left leg ankle-brachial index 0.80; right leg ankle-brachial index 0.87.
D. Left leg ankle-brachial index 0.86; right leg ankle-brachial index 0.87.
E. Left leg ankle-brachial index 1.17; right leg ankle-brachial index 1.15.

The ABI is calculated as the ratio of blood pressure in the leg to the arm blood pressure. Patients with
peripheral arterial disease (PAD) frequently have subclavian stenoses causing a difference in arm blood
pressures; in this situation, the higher blood pressure should be used for calculating the ABI.

In this case, the calculation is 120/150 = 0.80 on the left leg and 130/150 = 0.87 on the right leg.

ABI can be >1 in the case of noncompressible leg arteries, commonly seen with diabetes mellitus or
chronic kidney disease. The toe-brachial index should be measured to diagnose patients with suspected
PAD when the ABI is >1.40 (Class I recommendation).
Answer: C

Key Point
ABI establishes the diagnosis of peripheral artery disease (PAD) in patients with a history or physical
suggestive of PAD. TBI should be obtained in those with supranormal ABI measurements (>1.4).

Question 27:
A 45-year-old man presents to the cardiology clinic. He reports that his sister recently underwent cardiac
surgery for aortic valve replacement and ascending aortic aneurysm repair related to bicuspid aortic valve.
He takes no medications.

His vital signs include blood pressure 120/78 mm Hg in the right arm and 118/78 mm Hg in the left arm,
pulse 74 bpm, and respirations 12 per minute. A physical examination reveals normally split S1 and S2
with no murmurs. Pulses are 2+ throughout.

A transthoracic echocardiogram is technically difficult.

What additional testing would you recommend?
A. Computed tomographic angiography of the brain.
B. No additional testing.

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C. Exercise stress testing.

D. Computed tomographic angiography of the chest.
E. Transesophageal echocardiogram.

Aortic imaging with computed tomographic (CT) angiography or magnetic resonance (MR) imaging/MR
angiography is recommended for first-degree relatives of patients with bicuspid aortic valves, especially
when there is a family history of thoracic aortic aneurysm and/or aortopathy.

Tomographic imaging with either CT or MR should be performed in addition to transthoracic

echocardiography if the aorta is not seen at a level ≥4 cm above the aortic valve.

Transesophageal echocardiography may be considered but requires sedation and still may miss segments
of the ascending aorta.

CT angiogram of the brain may be reasonable to screen for intracranial aneurysm in a patient with
coarctation of the aorta, but there is no evidence of coarctation.

Exercise stress testing is not indicated in asymptomatic patients.

Answer: D
Key Point
Several genetic conditions are associated with the development of thoracic aortic aneurysms, including
Marfan syndrome, Loeys-Dietz syndrome, Turner syndrome, bicuspid aortic valve disease, and familial
thoracic aortic aneurysm disease. First-degree relatives of affected individuals should be screened.

Question 28:
A 40-year-old right-handed woman presents to the emergency department with dysphasia and right-sided
hemiparesis after being a restrained passenger in a motor vehicle collision. Her family notes that, prior to
the onset of the dysphasia and hemiparesis, she complained of left neck and eye pain. Her vital signs are
blood pressure 145/90 mm Hg and heart rate 95 bpm, with normal respiratory rate and oxygen saturation.
Her physical examination is notable for left eyelid ptosis and left eye miosis.
Given the available history and examination data, which of the following is the most probable diagnosis?
A. Bell's palsy.
B. Carotid artery dissection.

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C. Ruptured berry aneurysm.

D. Cerebral artery thrombotic occlusion.
E. Hemiplegic migraine.

An acute ischemic cerebral syndrome preceded by neck pain or Horner's syndrome should prompt
suspicion of carotid dissection, particularly after trauma. In this case, features of Horner's syndrome are
present with ptosis and miosis of the left eye. Ischemic cerebral findings are present, indicative of injury
to the left cerebral hemisphere. This suggests a left internal carotid artery dissection causing local pain
and Horner's syndrome with involvement of the middle cerebral artery on the left side, producing right-
sided neurologic deficits.

Left cerebral artery occlusion might produce identical ischemic stroke symptoms but would not explain
the local pain or Horner's syndrome.

A ruptured berry aneurysm more commonly presents with headache.

Bell's palsy usually presents with dysfunction of facial nerve branches but would not explain hemiparesis,
which is a sequelae of central nervous system ischemia.

Hemiplegic migraine may present with hemiplegia but is less likely to cause focal pain and Horner's
syndrome.
Answer: B
Key Point
An acute ischemic cerebral syndrome preceded by neck pain or Horner’s syndrome should prompt
suspicion of carotid dissection.

Question 29:
A 77-year-old man is referred to you because of a recent sudden pain in the toes of his left foot that began
1 week prior. He has coronary artery disease status/post multiple percutaneous coronary interventions.
His medications include aspirin 81 mg daily, atorvastatin 80 mg daily, carvedilol 12.5 mg BID, and
clopidogrel 75 mg daily. He limps into your office and, on examination, has a cold left foot with no
pulses palpated below the knee. He has what appears to be early gangrene on the tips of three of his toes,
with discoloration and skin breakdown between the toes.

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What should be your first diagnostic test?

A. Ankle-brachial index.
B. Computed tomography angiography.
C. Contrast angiography.
D. Magnetic resonance imaging angiography.
E. Toe-brachial index.

The correct answer is contrast angiography. The patient presents with acute limb ischemia. This can be
due to a thromboembolic event, dissection, thrombotic occlusion of a narrowed vascular segment, or
embolic debris from a proximal vessel lesion. This is a medical urgency and the patient should go directly
to the vascular catheterization laboratory and undergo contrast angiography that should include imaging
of the aorta, iliacs, femorals, popliteals, and runoff of the distal vessels (Class I recommendation).

All the other studies will simply delay what is considered the gold standard for diagnosing acute limb
ischemia and appropriate intervention to salvage the foot and toes.
Answer: C
Key Point
Acute limb ischemia is vascular emergency and requires rapid assessment of arterial and venous perfusion
as well as sensory function and motor function to determine limb viability and salvageability.
Revascularization is performed urgently/emergently.

Question 30:
A 64-year-old former smoker with hypertension and diabetes mellitus presents for a routine annual
follow-up appointment. He has no symptoms. He admits to being relatively inactive at baseline but has
considered starting an exercise regimen.

His medications include metformin 1000 mg BID and lisinopril 20 mg daily.

His physical examination is notable for a heart rate of 82 bpm and blood pressure of 126/72 mm Hg.
Auscultation of the heart reveals a I/VI early peaking systolic murmur at the left upper sternal border
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without radiation. A lung examination is unremarkable. There is no lower extremity edema. He has 1+
posterior tibial pulses bilaterally.

An electrocardiogram (ECG) demonstrates normal sinus rhythm with T-wave inversions in the inferior
leads, which is unchanged from an ECG 6 months prior.
What is the most appropriate next step?
A. Echocardiogram.
B. No further testing indicated.
C. Ankle-brachial indices.
D. Ambulatory blood pressure monitor.
E. Exercise stress echocardiogram.

Recognizing risk factors for peripheral artery disease (PAD), particularly in asymptomatic patients, is
important. Risk factors for PAD include: 1) age >70 years; 2) age 50-69 years with a history of smoking
or diabetes mellitus; 3) age 40-49 years with diabetes mellitus and one other risk factor for
atherosclerosis; 4) abnormal pulses in the lower extremities on examination; 5) symptoms suggestive of
claudication with exertion or ischemic rest pain; and 6) atherosclerotic disease in other sites (carotid
stenosis, coronary disease, renovascular disease).

This patient has several risk factors for PAD and a concerning physical examination with diminished
arterial pulses in the lower extremities. Ankle-brachial indices is a Class I recommendation to assess for
PAD in a patient with a history or physical examination suggestive of PAD. Early detection of PAD is
important given that this identifies those at risk for atherosclerosis at other sites. PAD is also a risk factor
for cerebrovascular accident and death. Early detection of PAD allows for early treatment with aspirin
and statins, as well as aggressive risk-factor modification (i.e., smoking cessation, lipid lowering, etc.).
See Figures 1, 2, and 3.

Echocardiogram is not indicated because the murmur appears to be an innocent flow murmur versus
aortic sclerosis by examination.

Stress testing is not indicated because the patient has no symptoms of ischemia.

Ambulatory blood pressure monitoring would not be indicated because the patient's blood pressure is
reasonably controlled at his office visit.
Answer: C
Key Point
ABI establishes the diagnosis of peripheral artery disease (PAD) in patients with a history or physical
suggestive of PAD. TBI should be obtained in those with supranormal ABI measurements (>1.4).

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Question 31:
A 67-year-old man presents to the emergency department for evaluation of 3 days of right foot pain. He
has not had regular medical care in the past. He has a 50 pack-year history of tobacco use. On
examination, his blood pressure is 162/97 mm Hg, heart rate is 88 bpm, heart sounds are regular but
distant, and lung sounds are diminished. His right foot is cool and dorsalis pedis and posterior tibialis
pulses are nonpalpable.
Which of the following findings would prompt emergent revascularization within 6 hours?
A. Audible arterial Doppler, audible venous Doppler, no sensory loss, no muscle weakness.
B. Inaudible arterial Doppler, inaudible venous Doppler, sensory loss in foot, no motor function.
C. Inaudible arterial Doppler, inaudible venous Doppler, no sensory loss in foot, no muscle
weakness.
D. Audible arterial Doppler, inaudible venous Doppler, no sensory loss, no muscle weakness.
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E. Inaudible arterial Doppler, audible venous Doppler, sensory loss to midfoot, mild muscle
weakness.

Acute limb ischemia (ALI) is defined as acute (<2 weeks), severe hypoperfusion of the limb characterized
by these features: pain, pallor, pulselessness, poikilothermia (cold), paresthesias, and paralysis.

When assessing ALI, it is important to differentiate between viable, threatened, and irreversibly damaged
limbs. Characteristics of each of these include:

I. Viable: limb not immediately threatened, no sensory loss, no muscle weakness, audible arterial and
venous
Doppler.
II. Threatened: mild to moderate sensory or motor loss, inaudible arterial Doppler, audible venous
Doppler.
III. Irreversible: major tissue loss or permanent nerve damage inevitable, profound sensory loss,
anesthetic,
profound muscle weakness or paralysis (rigor), inaudible arterial and venous Doppler.

Patients with viable limbs should undergo urgent revascularization (within 6-24 hours). Patients with
threatened limbs should undergo emergent revascularization to prevent limb loss (within 6 hours).
Patients with irreversible ALI should undergo primary amputation .
Answer: E
Key Point
Acute limb ischemia is vascular emergency and requires rapid assessment of arterial and venous perfusion
as well as sensory function and motor function to determine limb viability and salvageability.
Revascularization is performed urgently/emergently.

Question32:
A 72-year-old woman with a history of hypertension and diabetes mellitus presents with 1 hour of left-
sided facial droop and slurred speech.
Which of the following is most likely to identify cerebral edema if present in this patient?
A. Computed tomography angiography.
B. Cerebral perfusion computed tomography.
C. Noncontrast head computed tomography.
D. Head magnetic resonance angiography.
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E. Head magnetic resonance imaging.

Cerebral ischemia, regardless of the etiology, results in intracellular changes that quickly lead to cell
death. These changes include changes in intracellular concentrations of electrolytes and eventually
increased intracellular water, resulting in cerebral edema. Vasogenic edema can also be seen as a result of
the breakdown of the blood–brain barrier, allowing extracellular proteins and therefore fluid to
accumulate in the extracellular space. This is best seen on T2 weighted magnetic resonance images,
which can identify edema.

Noncontrast computed tomography (CT) is the first screening imaging that should be performed in
patients suspected of stroke, but would not identify edema this early in the disease process.

Anatomic evaluation, such as magnetic resonance angiography or CT angiography, is helpful in

identifying targets for intervention, but is not able to identify local edema.

Cerebral perfusion CT can evaluate for preservation of cerebral autoregulation and potential salvageable
tissue.
Answer : E
Key Point
There is no circulating biomarker of cerebral infarction; diffusion-weighted magnetic resonance imaging
is the most sensitive test for acute ischemic cerebral injury.

Question 33:
An 80-year-old man with a history of coronary artery disease, heart failure with preserved ejection
fraction, hypertension, and persistent atrial fibrillation presents with altered mental status. His home
medications include aspirin 81 mg, metoprolol 100 mg extended release, losartan 50 mg, and warfarin.
On examination in the emergency department, he is afebrile, with heart rate 50 bpm and blood pressure
180/100 mm Hg. The Glasgow coma scale is 13/15. Neurologic examination reveals right-sided paralysis
with aphasia. International normalized ratio (INR) is 3. Head computed tomography reveals left
intracerebral hemorrhage.
In addition to reducing blood pressure, what is the best next step?
A. 4F-prothrombin concentrate complex 1000 units.
B. Six units of fresh frozen plasma.
C. Intravenous vitamin K 10 mg.
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D. 4F-prothrombin concentrate complex 1500 units.

E. 4F-prothrombin concentrate complex 60 units/kg.

This patient presents with a major bleed; thus, reversal of anticoagulation is indicated. See Figure 1.

Prothrombin concentrate complexes (PCCs) contain purified vitamin K–dependent clotting factors
obtained from pooled human plasma and are free of viral contaminants. Only 4F-PCCs are licensed for
rapid vitamin K–dependent anticoagulant (VKA) reversal. They do not require ABO compatibility and
can be stored at room temperature as a lyophilized powder; therefore, they can be rapidly reconstituted
and infused. They are dosed based on INR and body weight (INR 2-4 at 25 U/kg, INR 4-6 at 35 U/kg, and
INR >6 at 50 U/kg; maximum dose 5000 U capped at 100 kg body weight) for VKA reversal. Per unit
volume, 4F-PCCs contain approximately 25 times (25 U/mL) the concentration of vitamin K–dependent
factors as compared with plasma (1 U/mL). Therefore, PCC can be given in a much smaller volume at a
much faster infusion rate (eight times) compared with plasma and is preferred. In addition, a fixed dose
can be given with 1000 units for any bleed and 1500 units for intracranial bleed; therefore, either 1500
units fixed dose or 25 units/kg could be used in this case.

A unit of plasma (225-300 mL, fresh frozen, frozen, or thawed plasma) is usually obtained from a whole
blood donation. A unit of plasma contains not only vitamin K–dependent clotting factors, but also other
coagulation factors and proteins, and is hence considered a nonspecific reversal agent. Plasma transfusion
requires ABO blood type matching and thawing of frozen plasma. Thus, generally from the time of the
order to transfusion, actual administration of the first unit of plasma may take ≤90 minutes. The adverse
effects of plasma transfusion include circulatory volume overload allergic reactions and risk of
transfusion-related acute lung injury; these effects are not observed with PCC, which should hence be
preferred, especially in volume-sensitive patients.

Intravenous vitamin K administration will slowly reverse VKA, with earliest effect on the INR in 2 hours
and peak effect in 6-12 hours; thus, it would not be the preferred option for managing an acute
intracranial hemorrhage.
Answer: D
Key Point
Reversal of anticoagulation was never shown to improve outcomes, but that should be done promptly
with approved agents in the setting of acute anticoagulant-related brain bleeds. Current guidelines
recommend using a combination of prothrombin complex concentrates and vitamin K for warfarin,
idarucizumab for dabigatran, and andexanet alfa for rivaroxaban and apixaban.

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Question 34 :
A 58-year-old woman comes to your clinic for evaluation of weakness and cramping in her calves when
she walks up hills. The pain goes away within minutes if she stops to rest. She has hypertension,
hyperlipidemia, type 2 diabetes mellitus, mild (GOLD stage I) chronic obstructive pulmonary disease, and
stage 2 chronic kidney disease. Her current medications are aspirin 81, lisinopril 20 mg,
hydrochlorothiazide 25 mg, amlodipine 5 mg, rosuvastatin 40 mg daily, inhaled albuterol as needed, and
insulin. She smoked 45 pack-years before quitting earlier this year.

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Vital signs are pulse 72 bpm, respirations 14 per minute, and blood pressure 134/90 mm Hg in the right
arm and 130/88 mm Hg in the left arm. There is a right carotid bruit. The chest is clear with a prolonged
expiration time. Cardiac examination is regular rhythm with normal S1 and S2. Abdomen is obese and
nontender. Dorsalis pedis and posterior tibial pulses are 1+ bilaterally.

Carotid duplex ultrasound shows a 30-50% stenosis on the right side and <30% stenosis on the left side.
Abdominal ultrasound shows a 4.0 cm aneurysm of the infrarenal aorta. Her ankle-brachial index is 1.2
on the right and noncompressible on the left.
Based on the information above, which of the following is this patient most likely to experience in the
next 5 years?
A. Amputation.
B. Ruptured aortic aneurysm.
C. Hemodialysis.
D. Myocardial infarction.
E. Limb revascularization.

Peripheral artery disease (PAD) is most commonly the result of atherosclerosis in the arteries of the
extremities. Almost all patients have atherosclerosis in all vascular distributions as well. The presence of
PAD therefore confers a high risk of cardiovascular morbidity and mortality, with 25-30% all-cause 5-
year mortality and 20% 5-year cardiovascular mortality among patients with PAD and claudication. An
additional 20% of PAD patients will have nonfatal myocardial infarction (MI) or nonfatal stroke in the
same 5 years of follow-up.

Limb-specific outcomes are less common than cardiovascular outcomes in patients with PAD and
claudication: >70% of patients will have stable claudication at 5-year follow-up, 16% will have
progressive claudication, 7% will require surgical revascularization, and <5% will require amputation.

Chronic kidney disease progresses to end-stage renal disease requiring dialysis at variable rates, but large
cohorts suggest that the overall progression from mild elevations in creatinine to uremic states needing
dialysis occurs in 1.5% of patients per year, or approximately 7.5% over 5 years, much less than the
combined rate of fatal and nonfatal MI in PAD patients.

The rate of rupture for asymptomatic abdominal aortic aneurysms <5.0 cm is low, with 4 cm aneurysms
rupturing at a rate of <0.5%/year.
Answer : D
Key Point
Peripheral artery disease is highly prevalent, particularly with advancing age, affecting nearly 8.5 million
Americans, 19% of the population >70 years of age, and >202 million persons worldwide.

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Question 35 :
A 75-year-old woman presents to your office with left foot pain. She has a history of coronary artery
disease status/post prior percutaneous coronary intervention, diabetes mellitus, hypertension,
hyperlipidemia, and recently quitting smoking. Medications include aspirin 81 mg daily, lisinopril 10 mg
daily, atorvastatin 80 mg daily, and metformin 500 mg BID. On examination, her left foot is cold with
pallor and loss of sensation. There is no tissue breakdown. Her dorsalis pedis and posterior tibialis pulses
are difficult to palpate bilaterally.

Angiography of the left lower extremity shows an abrupt cutoff of flow in the anterior tibial artery and
extensive atherosclerotic stenoses of the popliteal and peroneal arteries.
Which of the following is the best next step in her management?
A. Amputation.
B. Revascularization.
C. Warfarin.
D. Hyperbaric oxygen.
E. Thrombolysis.

The clinical presentation is consistent with critical limb ischemia with resting pain and examination
findings of tissue hypoperfusion, including pallor and loss of sensation. With medical therapy, the risk of
amputation is 40% with critical limb ischemia. Revascularization is a Class I indication for critical limb
ischemia. The goal of surgical or endovascular revascularization is to provide in-line blood flow to the
foot through at least one patent artery, which will help decrease ischemic pain and allow healing of any
wounds while preserving a functional limb. Clinical trials have not demonstrated an advantage to surgical
versus endovascular approaches.

The usefulness of catheter-directed thrombolysis in patients with critical limb ischemia and a viable limb
is unclear (Class IIb recommendation).

Amputation would not be the first option in a patient with a salvageable limb.

Hyperbaric oxygen is used for nonhealing wounds and does not have a role in the management of acute
limb ischemia.

Warfarin could be an adjunct to management of thromboembolism after removal or revascularization of

the thrombosed vessel.
Key Point
Treatment of critical limb ischemia revolves around the following key principles:
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• Revascularization should be performed when possible to establish in-line flow.

• The method of revascularization (surgical or endovascular) is best decided by an interdisciplinary
care team and depends on a variety of anatomic and clinical features.
• Regular wound care after revascularization is vital to achieving complete wound healing and
preserving the functioning foot.

Answer: B

Question 36:
A 72-year-old man is admitted with pain in his right calf, ankle, and foot. An hour prior to coming to the
hospital, he felt dull aching pain in the calf and ankle while watching television. He had difficulty
standing up because the foot felt "heavy" and his family brought him to the hospital. His past medical
history includes coronary artery disease status/post coronary artery bypass grafting using the right
saphenous vein, hypertension, dyslipidemia, type 2 diabetes mellitus, obesity, and sleep apnea. His
current medications are aspirin 81 mg, chlorthalidone 50 mg, lisinopril 40 mg, atorvastatin 80 mg,
metoprolol 100 mg extended release, and metformin 1000 mg BID.

His vital signs are blood pressure 138/92 mm Hg, pulse 90 bpm, and respirations 16 per minute. A
physical examination reveals a man in moderate discomfort. The cardiac examination is regular S1 and S2
with a soft, early peaking murmur at the base. There are bilateral carotid bruits. The right lower extremity
is edematous and dusky colored from the knee down, with sluggish capillary refill. The shin and calf are
tender to the touch, with decreased sensation in the toes up to the mid foot. Plantar flexion and
dorsiflexion of the right foot are considerably weaker than the left foot. Right posterior tibial and dorsalis
pedis pulses are not palpable and arterial Doppler signals at these sites are inaudible. Venous Doppler
signs at the right ankle are audible but difficult to obtain.
In addition to anticoagulation, which of the following is the most appropriate next step in his care?
A. Computed tomography angiography.
B. Fasciotomy.
C. Amputation.
D. Duplex venous imaging.
E. Catheter-based thrombolysis.

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This patient has acute limb ischemia (ALI) with an immediately threatened limb, as evidenced by the rest
pain and sensory loss involving more than the digits (toes), muscle weakness, slow capillary refill, and
absent arterial Doppler signals with preserved venous Doppler signals. The limb is salvageable if
treatment with revascularization and anticoagulation is performed within 4-6 hours of symptom onset.
Revascularization can be performed with catheter-based techniques or surgical embolectomy. In this
patient with previous vein harvesting, catheter techniques may be preferred, but the objective is simply
rapid restoration of arterial flow with the least risk to the patient.

Prolonged duration of ischemia is the most common factor in patients requiring amputation for treatment
of ALI. Patients who have an insensate and immobile limb in the setting of prolonged ischemia (>6-8
hours) are unlikely to have potential for limb salvage with revascularization and would require
amputation.

In patients with suspected ALI, clinical evaluation should rapidly assess limb viability and potential for
salvage with symptom onset and duration, bedside motor and sensory assessment, and bedside arterial
and venous Doppler signals. Revascularization of a viable limb should not be delayed for imaging.

Compartment syndrome may occur after revascularization or trauma and requires urgent treatment with
fasciotomy. This patient does not have a preceding injury or the classic sign of "wood-like" firmness of
the affected muscles to support this diagnosis. However, he should be monitored closely postoperatively
for this complication.
Key Point
Acute limb ischemia is vascular emergency and requires rapid assessment of arterial and venous perfusion
as well as sensory function and motor function to determine limb viability and salvageability.
Revascularization is performed urgently/emergently.
Answer : E

Question 37:
A 42-year-old man comes to clinic for evaluation of leg pain. He states that, for the prior few months, he
has had bilateral calf pain when walking up stairs. He has no known medical problems except for a 40
pack-year smoking history. He is on no medications. His blood pressure is 132/82 mm Hg with a heart
rate of 74 bpm. His examination reveals pale hands with small dark ulcers on the third and fourth digits of
his left hand. There is a small ulcer on the big toe of his right foot. Dorsalis pedis pulses are diminished
bilaterally.
Which of the following would be the most likely diagnosis in this patient?
A. Peripheral arterial disease.
B. Thromboangiitis obliterans.
C. Cholesterol emboli.
D. Behçet's disease.
E. Scleroderma.

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The patient has a classic presentation for thromboangiitis obliterans (Buerger's disease). This is an
inflammatory vascular disease not associated with atherosclerosis that generally affects the small vessels.
It typically presents in men in their 40s who have a significant smoking history. Ischemia of the digits
(including ischemic ulcers) is the most common presentation. Patients may also have discoloration of the
hands (Raynaud phenomenon), especially during cold weather. The condition may sometimes progress so
that it affects larger arteries, leading to symptoms of claudication, as seen in this patient. Smoking
cessation is the only treatment.

Whereas peripheral arterial disease also presents with claudication in the lower extremities, it would not
be expected to have findings such as pallor and ulceration of the upper extremities. Cholesterol emboli
usually occur in older patients after a cardiac or vascular procedure with appearance of a lace-like mottled
purple skin discoloration (livedo reticularis). Behçet's disease presents classically with oral and genital
ulcers. Whereas scleroderma can present with Raynaud phenomenon and occasionally digital ulcers, there
are usually joint complaints and fatigue. It is also much more common in women than men.
Answer: B
Key Points

• Thromboangiitis obliterans is closely linked to tobacco use, which is thought to trigger this
inflammatory disease of the small and medium vessels of the arms and legs.
• Complete cessation of tobacco use is the mainstay of treatment of thromboangiitis obliterans.

Question 38:
For which of the following patients would resting ankle-brachial index (ABI) testing be recommended?
A. A 56-year-old man with swelling and tenderness behind the knee, worse with walking.
B. A 48-year-old man with a history of hypertension.
C. A 52-year-old woman with pale feet when lying in bed, reddish color when standing.
D. An 84-year-old woman with a draining ulcer over the medial malleolus.
E. A 73-year-old man with pain and tingling in both legs while walking, reduced with bending
forward.

The 52-year-old woman with elevation pallor and dependent rubor has signs of peripheral arterial disease,
and therefore an ABI is indicated (see Table 5 from the 2016 American Heart Association/American
College of Cardiology [AHA/ACC] Guideline on the Management of Patients With Lower Extremity
Peripheral Artery Disease [PAD]).

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The 73-year-old man with pain and tingling in both legs while walking, reduced with bending forward, is
consistent with neurogenic claudication, caused by spinal stenosis.

The 48-year-old man with hypertension does not meet indications for ABI testing (see Table 4 in the 2016
AHA/ACC guideline on PAD). In patients at increased risk for PAD but without symptoms, there is a
Class IIa indication for resting ABI testing (see Table 4 in the 2016 AHA/ACC guideline on PAD).

The 84-year-old woman with draining ulcer over the medial malleolus likely has associated venous
insufficiency, as these findings are characteristic of venous ulcers. Arterial ulcers tend to be dry and to
affect the lateral ankle or distal feet.

The 56-year-old man with swelling and tenderness behind the knee, worse with walking, likely has a
Baker's cyst, caused by excess synovial fluid production, usually in the setting of knee arthritis or torn
cartilage.
Answer: C
Key Point
ABI establishes the diagnosis of peripheral artery disease (PAD) in patients with a history or physical
suggestive of PAD. TBI should be obtained in those with supranormal ABI measurements (>1.4).

Question39:
A 76-year-old man with atrial fibrillation presents to the emergency department with sudden onset of
facial droop and word-finding difficulty. He denies headache. Other medical problems include
hypertension, hyperlipidemia, type 2 diabetes mellitus, ischemic heart disease with left ventricular (LV)
ejection fraction 40%, and obesity. His current medications are aspirin 81 mg, valsartan 80 mg,
indapamide 2.5 mg, atorvastatin 80 mg, metformin 1000 mg BID, metoprolol 100 mg extended release,
and warfarin, although he admits that he does not take his medications every day.

Vital signs are blood pressure 150/92 mm Hg, as well as pulse 108 bpm and irregular. His physical
examination is remarkable for left-sided facial droop and slurred speech. A cardiac examination reveals
irregularly irregular rhythm with no murmurs.

Laboratory evaluation includes normal electrolytes, international normalized ratio 1.6, and normal blood
counts.
Which of the following would be the most likely finding on computed tomography (CT) of the head?
A. Multiple small hypodensities in the cerebellum.
B. Extravasation along dependent areas of the skull.

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C. Tumor in the posterior fossa.

D. Loss of gray–white matter interface in the parietal lobe.
E. Diffuse white matter changes.

This patient has multiple risk factors for stroke including atrial fibrillation, hypertension, hyperlipidemia,
LV systolic dysfunction, and age >75 years; he also presents with acute stroke symptoms. The most likely
finding on head CT is an acute infarct, which would appear as blurring of the gray–white matter interface.
Note that, while cardioembolic strokes may be multiple, they can also be single; i.e., the number of
abnormalities on CT scan does not confirm the etiology of the stroke.

The acute presentation is not consistent with a tumor of the central nervous system.

The risk factor profile of this patient is more consistent with an ischemic rather than a hemorrhagic
process.

Multiple small hypodensities, so-called lacunar infarcts or lacunes, are commonly seen in hypertensive
individuals but would not be expected to produce acute symptoms such as this.

Diffuse white matter changes may be seen with normal aging or with dementia, but would not be
expected to procedure acute, focal symptoms.
Answer : D
Key Point
Ischemic stroke etiology cannot be determined simply based on the size/shape/location/number of infarcts
or the clinical presentation.

Question 40 :
A 43-year-old man is referred to your clinic for consultation by his primary care provider. He states that
he has recently noticed pain in the third and fourth fingers of his left hand. He also notes occasional
discoloration of the fingers in that same hand. He has a past medical history of type II diabetes mellitus,
hypertension, and hyperlipidemia. He has smoked 1.5 packs daily for the prior 25 years. His medications
are metformin 500 mg twice daily, lisinopril 20 mg daily, and atorvastatin 20 mg daily.
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His blood pressure (BP) is 146/84 mm Hg with heart rate 78 bpm. His examination is notable for a small,
dark ulcer on the fourth digit of his left hand. An Allen test is abnormal on the left hand.

His last hemoglobin A1c level is 6.9%.

Which of the following would be the most effective treatment for his presenting condition?
A. Increase statin dose.
B. Improve blood pressure control.
C. Begin regular aerobic exercise.
D. Abstain from smoking.
E. Improve diabetes mellitus control.

The patient has a classic presentation for Buerger's disease (thromboangiitis obliterans). This is an
inflammatory vascular disease not associated with atherosclerosis that affects the small vessels. It
typically presents in men in their 40s who have a significant smoking history. Ischemia of the digits
(including ischemic ulcers) is the most common presentation. Patients may also have Raynaud's
phenomenon changes with discoloration of digits in cold weather, as well as an abnormal Allen test on
examination. Smoking cessation is the only proven treatment for Buerger's disease. Whereas high-
intensity statin therapy, regular exercise, and improved BP/glucose control have benefits for other
cardiovascular conditions, they have not been shown to improve Buerger's disease.
Answer : D
Key Points

• Thromboangiitis obliterans is closely linked to tobacco use, which is thought to trigger this
inflammatory disease of the small and medium vessels of the arms and legs.
• Complete cessation of tobacco use is the mainstay of treatment of thromboangiitis obliterans.

Question 41:
A 67-year-old man presents to the emergency department with a sudden onset of tearing back pain. He
has a past medical history of hypertension and hyperlipidemia. On examination, his blood pressure is
146/88 mm Hg (left arm) and 142/76 mm Hg (right arm), pulse is 90 bpm, respiratory rate is 20 breaths
per minute, and oximetry is normal. He appears distressed. Radial, femoral, and pedal pulses are 2+ and
equal. The remainder of his physical examination is normal. Computed tomography angiography (CTA)
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is performed and reveals a dissection that originates distal to the left subclavian artery and extends to both
common iliac arteries. His initial laboratories are notable for a creatinine (Cr) level of 2.7 mg/dL (baseline
Cr is 0.8 mg/dL). The patient is started on intravenous labetalol with reduction in blood pressure to
108/68 mm Hg.
Which of the following is the most appropriate next step in his care?
A. Nephrology consultation.
B. Intravenous nitroprusside.
C. Urgent revascularization.
D. Computed tomography angiography in 24 hours.
E. Coronary angiography.

The patient has an acute type B aortic dissection. Patients with acute or late complications, including renal
failure, visceral ischemia, or contained rupture require urgent repair due to increased mortality rates as
high as 20% at day 2 and 25-50% within the first month. Because of his renal compromise, this patient
should be referred for urgent revascularization (Figure 1).

Initial management of an uncomplicated type B aortic dissection involves intravenous beta-blockers to

reduce heart rate to <60 bpm and systolic blood pressure to 100-120 mm Hg. Intravenous vasodilator
(nitroprusside or nicardipine) can be used to achieve blood pressure reduction in addition to beta-
blockers, if necessary. This patient achieved adequate reduction in blood with labetolol.

Although this patient may require renal replacement therapy due to renal vascular compromise, the most
important initial step is revascularization to improve renal perfusion. Revascularization should not be
delayed in order to obtain nephrology consultation. Similarly, revascularization should not be delayed for
coronary angiography or repeat CTA.

Answer: C
Key Point
Surgery or endovascular intervention may be required in the management of unstable or complicated
acute type B aortic dissection, such as in those with malperfusion syndrome or early expansion. In most
centers, endovascular therapy, including thoracic endovascular aortic repair (TEVAR), has replaced
surgery for this indication. The role of TEVAR for uncomplicated type B dissection requires further
study.

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Question42:
A 65-year-old man is evaluated for bilateral calf pain on walking 50 yards that resolves if he stops to rest.
He has a past medical history of dyslipidemia and a current 50 pack-year smoking history. He takes no
medications. Physical examination reveals a blood pressure of 140/80 mm Hg and heart rate of 70 bpm.
Lower extremities are warm and pink. There is no evidence of hair loss or nail changes. Pulses are faintly
palpable and are monophasic by Doppler evaluation.

The ankle-brachial index (ABI) is 1.1 and 1.0 on the right and left, respectively.
Which of the following are the best next steps in this patient?
A. Exercise ankle-brachial index testing.
B. No further testing.
C. Duplex ultrasound of lower extremities.
D. Lower extremity angiography.
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E. Magnetic resonance imaging of the spine.

The patient described above has typical claudication. The ABI values are normal at 1.0, raising the
suspicion of noncompressible vessels. Considering that the patient has claudication during walking, the
best next step in diagnosis is an exercise ABI. A decrement of >20% in ABI values with exercise is
diagnostic of arterial obstruction. While angiography may reveal arterial stenosis, invasive testing is not
indicated without noninvasive findings suggestive of arterial disease. Duplex ultrasound imaging may
show arterial stenosis, but functional testing is important to evaluate for peripheral vascular disease in
patients with exertional symptoms (Class I).

Magnetic resonance imaging of the spine could be considered for diagnosing spinal stenosis, but the
exertional symptoms, risk-factor profile, and abnormal pulses suggest arterial disease.

Ongoing symptoms in a patient with multiple risk factors for arterial disease warrant further evaluation.
Answer : A
Key Point
The exercise ankle-brachial index testing aids in:

1. Diagnosing peripheral artery disease in patients with claudication and normal ankle-brachial
indices at rest.
2. Discriminating claudication from pseudoclaudication.
3. Assessing functional capacity.

Question 43 :
A 70-year-old former smoker presents for evaluation of a newly diagnosed, incidentally discovered 5.0
cm abdominal aortic aneurysm (AAA). He feels well. He walks 1-2 miles daily for exercise and plays in a
men's basketball league two nights per week. His past medical history is notable for hyperlipidemia and
diabetes mellitus. His medications include metformin 1000 mg BID and atorvastatin 20 mg daily.

On examination, his heart rate is 72 bpm and blood pressure is 118/68 mm Hg. A pulsatile mass is notable
approximately just below the umbilicus.
What is the most appropriate next step?
A. Start metoprolol succinate 25 mg daily.
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B. Start aspirin 81 mg daily.

C. Increase atorvastatin to 80 mg daily.
D. Repeat imaging in 6 months.
E. Referral for aneurysm repair.

Repeating imaging (ultrasound, computed tomography, or magnetic resonance imaging) in 6 months to

assess rate of expansion is the correct answer. According to the Society for Vascular Surgery
recommendations, surveillance intervals for asymptomatic AAA are as follows:

1. >2.5 cm but <3.0 cm, rescreen after 10 years

2. 3.0-3.9, repeat imaging every 3 years
3. 4.0-4.9, repeat imaging in 12 months
4. 5.0-5.4, repeat imaging in 6 months

Indications for elective repair of an asymptomatic AAA include:

1. >2.5 cm but ≤5.5 cm;

2. rapid expansion; and
3. AAA associated with peripheral arterial aneurysms or peripheral artery disease.

As this patient is asymptomatic with a 5 cm aneurysm, there is not an indication for surgical repair.

Intensifying statin therapy is not unreasonable; however, there are no high-quality studies to support the
use of statins to reduce the rate of AAA expansion or rupture. There have been no randomized controlled
trials to evaluate the effect of statins in this population and several meta-analyses have shown no effect.
Similarly, there are no data suggesting a benefit to aspirin or beta-blocker usage in this clinical setting.
Answer : D
Key Point
Elective repair of asymptomatic abdominal aortic aneurysms is recommended at a diameter of ≥5.5 cm,
although, in women, many experts lower the threshold to 5.0 cm because of their tendency to rupture at
smaller diameters.

Question 44:

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A 63-year-old woman with a history of hypertension presents to the emergency department with acute left
facial droop without acute abnormalities on a noncontrast head computed tomography (CT) and is treated
with intravenous fibrinolytics with resolution of her facial weakness. The remainder of her examination is
normal. Electrocardiogram (ECG), inpatient telemetry, head and neck CT angiography, and
transesophageal echocardiography (TEE) with bubble study are normal.
Which of the following is the best next step?
A. 30-day Holter monitor.
B. Lower extremity venous Dopplers.
C. Electroencephalography.
D. Exercise stress testing.
E. Cardiac magnetic resonance imaging.

This patient presents with a cryptogenic stroke. Occult paroxysmal atrial fibrillation should be considered
in at-risk patients with cryptogenic ischemic stroke. Long-term ECG monitoring is recommended because
it has been shown to increase the detection rate of atrial fibrillation in patients with ischemic stroke, for
whom anticoagulation may be beneficial for secondary prevention. Thus, a 30-day Holter monitor would
be the best next step.

The patient does not have any signs or symptoms of coronary disease; therefore, stress testing is not
indicated.

There is no indication for electroencephalography (EEG) in this setting given the lack of symptoms
suggesting seizure or abnormalities on head CT. Furthermore, EEG is not routinely recommended for
evaluation of cryptogenic stroke.

Although paradoxical embolism may be a cause of cryptogenic stroke, this patient does not have any
examination findings suggestive of deep vein thrombosis, and the TEE excludes a patent foramen ovale as
a cause of right-to-left shunting. Therefore, lower extremity venous Dopplers should not be ordered.

There is no reason to perform a cardiac magnetic resonance image because the TEE revealed normal
cardiac function without evidence of thrombus, masses, vegetations, or intracardiac shunt.
Answer : A
Key Point
Occult paroxysmal atrial fibrillation should be considered in at-risk patients with cryptogenic ischemic
stroke. Long-term monitoring is recommended. .

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Question45:
A 72-year-old woman with a history of hypertension, hyperlipidemia, and tobacco use presents to your
office for an initial consultation for an abnormal carotid ultrasound result. Given her cardiovascular risk
factors, a friend from work had suggested that she try the local mobile cardiovascular screening unit for a
quick check-up, where a carotid artery ultrasound demonstrated 100% right occlusion and a 50-69% left
occlusion. She otherwise has been feeling well and is active without any symptoms. Physical examination
is notable for heart rate 80 bpm and blood pressure 146/90 mm Hg. There are no cardiac murmurs or
carotid bruits on auscultation.
Which of the following is the best next step in this patient's management?
A. Carotid artery stenting.
B. Medical management.
C. Carotid endarterectomy.
D. Exercise stress test.
E. Carotid angiography.

Although noninvasive ultrasound screening for carotid disease is not recommended in asymptomatic
patients, this patient was found to have significant bilateral carotid stenosis. At present, the management
of asymptomatic carotid stenosis remains controversial; however, given that the annual stroke risk in this
population is estimated to be 0.5-1% and the majority of patients who ultimately require intervention first
present with transient ischemic attack, guidelines suggest intensive medical therapy as first-line
management for stenoses<70% with annual screening to evaluate for progression. Importantly, in this
case, because the patient has a contralateral right carotid artery occlusion, the peak systolic velocity
measured by ultrasound in the left carotid artery may be falsely elevated due to collateral flow, leading to
overestimation of the stenosis severity. This false-positive “jet effect” can also be seen in the ipsilateral
carotid artery when there is nonocclusive severe stenosis contralaterally. Therefore, carotid interventions
(endarterectomy or stenting) are not indicated at this time.

In an asymptomatic patient potentially with overestimation of the severity of occlusion, further anatomic
evaluation of carotid anatomy by angiography is not indicated at this time. However, if there is
progression of disease or development of symptoms, further evaluation may be warranted.

This patient is asymptomatic without signs or symptoms of obstructive coronary disease; therefore, stress
testing is not appropriate at this time. Guideline-directed medical therapies remain most appropriate.
Answer: B
Key Point
Carotid ultrasound may overestimate the severity of stenosis in the setting of contralateral carotid
occlusion and may not distinguish between subtotal and complete occlusion.
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Question 46:
A 55-year-old man is seen in the office for 6 months of exertional calf pain while walking that resolves
with rest. His past medical history includes hypertension, diabetes mellitus, and heart failure with reduced
ejected fraction. His medications include aspirin 81 mg daily, metformin 1000 mg twice a day, lisinopril
20 mg daily, and carvedilol 12.5 mg BID. Vital signs are blood pressure 110/70 mm Hg and heart rate 70
bpm. His cardiac examination is unremarkable. There are diminished dorsalis pedis pulses.

The right tibial artery blood pressure is 88 mm Hg and the left is 90 mm Hg.
Which of the following therapies would you recommend next?
A. Cilostazol.
B. Structured exercise program.
C. Pentoxifylline.
D. Chelation therapy.
E. Revascularization.

This patient has peripheral artery disease (PAD) with claudication and resting ankle-brachial index of 0.8
and 0.82. Patients with PAD should receive a comprehensive program of guideline-directed medical
therapy, including structured exercise and lifestyle modification, to reduce cardiovascular ischemic events
and improve functional status.

An individualized approach to revascularization for claudication is recommended for each patient to

optimize outcome. Revascularization is but one component of care for the patient with claudication, as
each patient should have a customized care plan that also includes medical therapy, structured exercise
therapy, and care to minimize tissue loss. If a strategy of revascularization for claudication is undertaken,
the revascularization strategy should be evidence based and can include endovascular revascularization,
surgery, or both.

Chelation therapy is not beneficial for the treatment of claudication (Class III).

Pentoxifylline is not effective treatment for claudication (Class III).

Cilostazol is a phosphodiesterase (PDE) inhibitor and a direct arterial dilator that can be effective in
patients with claudication, but it is contraindicated in patients with heart failure because of reports of
increased mortality with other oral PDE inhibitors.
Answer : B
Key Point
Treatment to improve the symptoms of claudication are:

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• Diagnosing peripheral artery disease in patients with claudication and normal ankle-brachial
indices at rest.
• Supervised exercise: a supervised exercise program should be discussed prior to intervention.
• Cilostazol: cilostazol effectively improves symptoms in patients with claudication, but is
contraindicated in patients with heart failure.
• Endovascular therapy: Endovascular therapy is effective and reasonable in patients with an
inadequate response to exercise and medical therapy.

Question 47 :
A 65-year-old woman presents to her primary care clinician's office with right lower extremity pain. The
pain has progressively worsened over the prior week. Her past medical history is significant for
hypertension. Her current medication is amlodipine 5 mg daily. Her vital signs are blood pressure 128/84
mm Hg, heart rate 75 bpm, and respirations 14 per minute with oxygen saturation 99% on room air.
Examination reveals a tender, mildly swollen right leg with erythema of the calf and ankle. Pulses are
normal. Her Duplex ultrasound is shown in Figures 1 and 2.

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Which of the following is the most likely diagnosis?

A. Baker's cyst.
B. Arterial stenosis.
C. Deep vein thrombosis.
D. Polymyalgia rheumatica.
E. Polyarteritisnodosa.

The ultrasound study reveals a vein that is thin walled and not collapsible with compression that is
diagnostic of a deep vein thrombosis (DVT); an echogenic mass is visible within the lumen of the vein
(Figure 3). Other significant findings of lower extremity DVT include visualization of anechoic thrombus,
increased venous diameter, loss of phasic flow with breathing, augmentation with calf squeeze, and
absence of color flow.

A Baker's cyst would be a well-circumscribed mass with an echolucent center.

Arterial stenosis is suspected on two-dimensional imaging when atheroma and stenosis are visualized and
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confirmed by spectral Doppler with significant increase in velocities as well as color Doppler, which
shows flow acceleration.
Polyarteritisnodosa typically presents with systemic symptoms of rash, fatigue, and weight loss. The
hallmark sign is small aneurysms like the beads of a rosary ("rosary sign"). The most commonly involved
organ is the kidney, including possible renal arterial aneurysms.
Polymyalgia rheumatica (PMR) is an inflammatory disorder typically seen in older adults that causes
widespread aching, stiffness, and flu-like symptoms. It is more common in women than men, and is seen
more often in Caucasians than any other race. Diagnosis is established by elevated inflammatory markers.
Imaging may demonstrate bursitis but PMR does not involve the lower extremity vasculature.
Answer : C
Key Point
Algorithms for diagnosis of deep vein thrombosis and pulmonary embolism offer the greatest accuracy
when incorporating an assessment of pretest clinical probability with appropriate use of D-dimer testing
and imaging.
Question48 :
A 67-year-old man with poorly controlled type 2 diabetes mellitus, hypertension, hyperlipidemia, chronic
kidney disease, and current tobacco use presents to your clinic with right foot pain. He first noticed the
pain several months prior and describes that it typically worsens when walking and improves with rest.
More recently, the discomfort has progressed with less exertion; over the prior few weeks, he has noted a
burning pain in the foot when lying down to sleep. Physical examination is notable for 1+ posterior tibial
and dorsalis pedis pulses on the left, and Dopplerable pulses in the right foot with delayed capillary refill
and distal lower extremity hair loss. You order noninvasive arterial tests that demonstrate ankle-brachial
index of 0.55 on the right and 0.82 on the left. A lower extremity computed tomography angiogram
demonstrates severe right superficial femoral arterial disease with single-vessel runoff to the foot.
In addition to smoking cessation and structured exercise, which of the following is the best next step in
this patient's management?
A. Intermittent pneumatic compression.
B. Aspirin and clopidogrel.
C. Pentoxifylline.
D. Cilostazol.
E. Endovascular stenting.

This patient has critical limb ischemia (CLI), defined as severe peripheral arterial disease (PAD)
associated with rest pain, nonhealing ulcers, or gangrene. Patients with CLI are at high risk of major
morbidities, including cardiovascular ischemic events and amputation. A systematic review of 13 studies
revealed a 22% all-cause mortality rate and a 22% rate of major amputation at 1 year in CLI patients who
did not undergo revascularization. Therefore, a diagnosis of CLI should warrant rapid consideration of
peripheral revascularization by an endovascular or surgical approach in order to establish in-line blood
flow to the foot to help decrease ischemic pain, facilitate wound healing, and preserve limb function.
However, if the limb is nonviable, revascularization is not warranted. In addition to revascularization, CLI
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patients should be treated with intensive medical therapy including aspirin or clopidogrel, statin, and
antihypertensive medications. Although there is a benefit of clopidogrel over aspirin in patients with
PAD, dual antiplatelet therapy with aspirin and clopidogrel in the absence of peripheral stent implantation
has not been demonstrated to provide incremental benefit. Cilostazol and intermittent pneumatic
compression have been shown to improve symptoms, but is not effective for CLI. Pentoxifylline has not
demonstrated effectiveness in the treatment of PAD.
Answer: E
Key Point
Treatment of critical limb ischemia revolves around the following key principles:

• Revascularization should be performed when possible to establish in-line flow.

• The method of revascularization (surgical or endovascular) is best decided by an interdisciplinary
care team and depends on a variety of anatomic and clinical features.
• Regular wound care after revascularization is vital to achieving complete wound healing and
preserving the functioning foot.

Question 49:
A 74-year-old man comes to the emergency department because of leg pain. He states that it was
uncomfortable when he awoke in the morning that day and had worsened throughout the day. His history
is significant for coronary artery disease, status/post coronary artery bypass grafting 10 years prior,
hypertension, and hyperlipidemia. His medications include aspirin 81 mg, atorvastatin 40 mg, lisinopril
20 mg, and amlodipine 10 mg daily.

On examination, his blood pressure is 158/86 mm Hg, heart rate is 92 bpm and regular, and he appears
uncomfortable. Lungs are clear and heart has a normal S1 and S2, as well as a II/VI early peaking systolic
murmur. His left dorsalis pedis and posterior tibial pulses are 2+ and the right pulses are not palpable, but
venous Doppler signals are appreciated. His right leg is cool and slightly mottled. Decreased pinprick
sensation is noted on the right leg below the ankle, and he has 4/5 strength on the right lower extremity
and 5/5 on the left.
What is the best next step in this patient's care?
A. Urgent revascularization.
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B. Systemic tissue plasminogen activator.

C. Lower extremity angiography.
D. Computed tomography angiography.
E. Lower extremity magnetic resonance angiography.

Acute limb ischemia requires prompt revascularization and should not be delayed for further imaging.
This patient has signs of an immediately threatened limb. The six "Ps" can be used to evaluate limb
ischemia: pain, pallor, poikilothermia, pulselessness, parasthesia, and paralysis. Loss of pulses does not
necessarily indicate an immediately threatened limb and may be seen early on in acute limb ischemia.
Sensory loss and evidence of muscle weakness, as seen in this patient, signal an immediately threatened
limb and should prompt immediate revascularization (Figure 1).

In patients with a marginally threatened limb, time to obtain imaging, such as with computed tomography
angiography or lower extremity angiography to help direct revascularization, is appropriate.

Systemic thrombolytics is not indicated in the management of acute limb ischemia. Catheter-directed
thrombolytics, however, may be considered depending on local expertise.
Answer : A
Key Point
Acute limb ischemia is vascular emergency and requires rapid assessment of arterial and venous perfusion
as well as sensory function and motor function to determine limb viability and salvageability.
Revascularization is performed urgently/emergently.

Question 50 :
A 78-year-old man with chronic obstructive pulmonary disease and chronic stable coronary disease
presents to the emergency department with dysarthria and weakness affecting his right arm that began >6
hours prior. Head computed tomography reveals an acute ischemic stroke involving the left middle

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cerebral artery. He does not receive thrombolytics and is admitted to the hospital for further management.
He is afebrile with a heart rate of 82 bpm and blood pressure (BP) of 190/100 mm Hg.
What is the most appropriate management of this patient's BP in the first 24 hours of hospitalization?
A. Antihypertensive therapy is not currently indicated.
B. Treat with oral medications; goal blood pressure <185/110 mm Hg.
C. Treat with intravenous medications; goal blood pressure <185/110 mm Hg.
D. Treat with intravenous medications; goal blood pressure <140/90 mm Hg.
E. Treat with oral medications; goal blood pressure <140/90 mm Hg.
There are no good data from randomized controlled trials for guidance of BP management during the
acute phases of an ischemic stroke. However, observational data suggest worse outcomes with
lowering BP in the first 24 hours after stroke onset. During this period, there may be vulnerable penumbra
that can be damaged if cerebral blood flow is reduced by lowering BP.
When thrombolysis is not used, BP should only be treated if it is >220/120 mm Hg. However, treatment
to a lower BP goal is indicated in the presence of certain comorbid conditions, including acute aortic
dissection, pre-eclampsia, eclampsia, unstable coronary syndrome, or acute heart failure. As this patient's
blood pressure is 190/100 mm Hg and he has no comorbidities, it is most appropriate to observe him off
antihypertensive therapy.
If thrombolytics are used, BP should be lowered to <180/110 mm Hg prior to administration of
thrombolytic therapy and maintained <180/105 mm Hg for at least 24 hours post-thrombolysis.
Answer: A
Key Point
Intensive blood pressure lowering to <130/80 mm Hg is associated with worse outcomes in the acute
postischemic stroke period

Question 51:
A 55-year-old man with a history of smoking, coronary artery disease status/post coronary artery bypass
graft, and atrial fibrillation presents to the emergency department with acute-onset pain in his left leg that
began suddenly 1 hour prior.
In addition to pain, paralysis, and pulse deficit, which of the following is most consistent with a diagnosis
of acute limb ischemia?
A. Hypothermia.
B. Purple discoloration.
C. Pachyderma.
D. Poikilothermia.
E. Numbness.
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The "5 Ps" of limb ischemia are: pain, pallor, paralysis, pulse deficit, paresthesia, and poikilothermia. The
temperature change seen in acute limb ischemia is poikolothermia (assumption of ambient temperature)
rather than hypothermia. Pachyderma (thick skin) and purple discoloration are associated with venous
outflow obstruction or lymphatic drainage limitations. The alteration in sensation in critical limb ischemia
is described as paresthesia ("pins and needles" sensation) and not numbness.
Answer: D

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Systemic diseases

Question1:
A 48-year-old woman hospitalized with pneumonia developed facial swelling and dyspnea while
receiving intravenous ceftriaxone. Vital signs were pulse 130 bpm and thready, blood pressure 82/52 mm
Hg, and respirations 20 per minute. Physical examination revealed a diaphoretic woman in acute distress.
There was a raised reddish rash over the trunk.
In addition to stopping the ceftriaxone infusion, which of the following medications is the best next step
in her care?
A. Epinephrine 1.0 mg (1:10,000) intravenous bolus.
B. Epinephrine 0.1 mg/min infusion.
C. Vasopressin 0.01 units/min infusion.
D. Epinephrine 0.3 mg (1:1000) intramuscular.
E. Diphenhydramine 25 mg intravenous bolus.

Epinephrine should be administered early by intramuscular injection to all patients with signs of a
systemic allergic reaction, especially hypotension, airway swelling, or difficulty breathing. The
recommended dose is 0.2-0.5 mg (1:1000 concentration) intramuscular, to be repeated every 5-15 minutes
in the absence of clinical improvement. In both anaphylaxis and cardiac arrest, the immediate use of an
epinephrine autoinjector is recommended if available.

Epinephrine 1.0 mg (1:10,000 concentration) is the appropriate dose for cardiac arrest and would not be
appropriate at this time due to the potential for fatal toxicity. However, in a patient with anaphylaxis but
not cardiac arrest, epinephrine 0.05-0.10 mg (i.e., 5-10% of the cardiac arrest dose) could be given
intravenously, if intravenous access is already in place.

Careful titration of a continuous infusion of intravenous (IV) epinephrine, based on severity of reaction
and in addition to crystalloid infusion, may be considered in treatment of anaphylactic shock or postarrest
management of anaphylaxis patients. The recommended dose is 5-15 mcg/min, not 0.1 mg/min (100
mcg/min).

Diphenhydramine, inhaled beta-adrenergic agents, and IV corticosteroids can be used to manage

nonsystemic allergic reactions such as rash or hives and may have some benefit in combination with
epinephrine for anaphylactic shock, but should not be used alone and are not the first-line treatment.

Vasopressin and other alpha agonists such as norepinephrine, methoxamine, and metaraminol may be

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useful for the treatment of anaphylactic shock that does not respond to epinephrine, but no prospective
studies have evaluated their use.
Answer : D

Question2:
A 52-year-old woman with a recent diagnosis of nonischemic cardiomyopathy presented to the
emergency department with complaints of fatigue and shortness of breath for the prior 4 days. Her
medications included carvedilol 6.25 mg twice daily, digoxin 0.25 mg daily, furosemide 20 mg daily,
lisinopril 40 mg daily, and spironolactone 50 mg daily. Her blood pressure was 102/74 mm Hg with a
heart rate of 62 bpm. She had mild jugular venous distension. Lungs were clear and there were no cardiac
murmurs. There was trace edema at the ankles.An electrocardiogram (ECG) was obtained (Figure 1).

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Which of the following is the most likely diagnosis in this patient?

A. Hypomagnesemia.
B. Hypercalcemia.
C. Digoxin toxicity.
D. Hyponatremia.
E. Hyperkalemia.

The ECG shows evidence of peaked T waves, most prominent in the precordial leads. These findings
would be most concerning for hyperkalemia, especially for a patient who is on both lisinopril and
spironolactone. Careful monitoring of potassium is required with initiation of spironolactone to detect
hyperkalemia, which can early on be asymptomatic or present with nondescript symptoms such as fatigue.
ECG findings consist of peaked T waves with a shortened QT interval followed by PR and QRS
prolongation at higher potassium levels. In severe hyperkalemia, the P wave can disappear and the QRS
widens significantly to a sine wave pattern. If untreated, this can eventually lead to cardiac arrest.Digoxin
administration can present with downsloping ST depression on the ECG. Actual digoxin toxicity is
usually associated with brady- and tachyarrhythmias, sometimes in combination with atrioventricular
block, which are not seen on this ECG.There is nothing in the clinical vignette to suggest hypercalcemia,
whose ECG findings consist primarily of QT shortening.Hypomagnesemia would be unlikely given the
relatively low dose of loop diuretic; ECG changes are sometimes seen and consist primarily of QT
prolongation.
There are no specific ECG findings in patients with hypernatremia.
Answer : E

Question 3:
A 37-year-old woman presented to your office for evaluation of hypertension. She saw her internist for a
routine physical one month prior and her blood pressure was 150/90 mm Hg. She denied symptoms and
did not exercise because she was too busy with work. She was on no medications and denied use of over-
the-counter medications or supplements. She did not smoke cigarettes. She had one alcoholic beverage
nightly and denied illicit drug use.
On examination, she was 5'2" tall and 115 lbs. Heart rate was 78 bpm and blood pressure 155/92 mm Hg.
Lungs were clear. Heart was regular with an S4 gallop. Abdomen was soft and nontender. Extremities
were warm without edema.
Laboratories showed sodium 143 mEq/L, potassium 3.6 mEq/L, bicarbonate 30 mEq/L, magnesium 1.9
mg/dL, calcium 9.5 mg/dL, and phosphorus 3.0 mg/dL.

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Which of the following is most likely to yield the diagnosis?

A. Outpatient polysomnography.
B. Transthoracic echocardiography.
C. Plasma renin activity and aldosterone concentration.
D. Serum parathyroid hormone level.
E. 24-hour urine fractionated metanephrines and catecholamines.

Secondary hypertension should be suspected in patients with severe or resistant hypertension, an acute
rise in blood pressure in patients with previously stable hypertension, age <30 in patients who are not
obese or black and who have no family history of essential hypertension, hypertension associated with
hypokalemia, or metabolic alkalosis. This patient has severe hypertension with electrolyte abnormalities,
and thus merits an evaluation for secondary causes.

The main clinical clue suggestive of primary aldosteronism is otherwise unexplained or easily provoked
hypokalemia, and metabolic alkalosis and hypernatremia are often seen. This patient is hypokalemic and
thus it is appropriate to check plasma renin activity and plasma aldosterone concentration as a first
screening test for primary aldosteronism.Obstructive sleep apnea may cause hypertension, but this patient
does not have risk factors or symptoms of daytime somnolence and thus polysomnography is likely to be
low yield.
An echocardiogram could assess for aortic coarctation, a secondary cause of hypertension. Arguing
against coarctation is that she is presenting at an older age with hypertension for the first time and that
there is no classic prominent to-and-fro machinery murmur on examination.Hyperparathyroidism can
cause hypertension but would be associated with hypercalcemia, which she does not
have.Pheochromocytoma can cause hypertension, usually in paroxysms and associated with headaches,
palpitations, and sweating. As she has none of these features, testing for urinary catecholamines will be
low yield.
Answer : C

Question4:
A 35-year-old woman presented to the emergency department with lightheadedness and syncope. She
described a recent cough with low-grade fever at home. She fainted while getting up from bed this
morning. Her past medical history included systemic lupus erythomatosis. Her current medications
included prednisone 5 mg daily and hydroxychloroquine 400 mg daily. Her vital signs were temperature
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99.8°F, blood pressure 123/60 mm Hg (dropping to 90/60 mm Hg with standing), and heart rate 100 bpm.
An examination was remarkable for malar rash. There was no jugular venous distention. Chest was clear
bilaterally. Cardiac examination revealed normal S1 and S2 with a grade II/VI systolic murmur; there was
no lower extremity edema.

Laboratory evaluation revealed white blood cell count 9,000 per mm3, hematocrit 35%, and platelets
100,000 per mm3.
Which of the following tests is the most likely to identify the cause of her syncope?
A. Morning cortisol.
B. Procalcitonin.
C. Tilt-table test.
D. Plasma norepinephrine.
E. Blood glucose.

This patient on chronic steroid therapy has orthostatic syncope. Chronic corticosteroid use inhibits the
adrenal cortical axis, producing relative adrenal insufficiency, which may have been unmasked by viral
illness in this case. Morning cortisol level, or cosyntropin stimulation test if the morning cortisol is
equivocal, can confirm the diagnosis of adrenal insufficiency.

Procalcitonin is a serum biomarker that can differentiate infection from other causes of inflammation and
would be elevated in patients with severe sepsis and septic shock, which is not present in this case.Plasma
norepinephrine levels may be useful for diagnosing autonomic dysfunction.Blood glucose levels would
assist in the diagnosis of hypoglycemia in patients with diabetes mellitus on hypoglycemic agents, which
is not the case in this patient.
Tilt-table testing would not add additional information since vital signs have already established postural
orthostasis.
Answer : A

Question 5:
A 55-year-old woman presented for elective surgery. She received prophylactic cefazolin and induction of
anesthesia with etomidate, fentanyl, and rocuronium. She became profoundly hypotensive, requiring
epinephrine and fluid volume resuscitation.
Elevated serum levels of which one of the following compounds should prompt skin testing
(radioallergosorbent [RAST]) for the specific cause of her hypotension during anesthesia?
A. Procalcitonin.
B. Endotoxin.
C. Troponin.
D. Tryptase.
Metanephrine.
E.
Any drug administered in the perioperative period can potentially produce life-threatening immune-
mediated anaphylaxis. However, anaphylaxis may be difficult to diagnose in the anesthetized patient, and
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other causes of hypotension such as myocardial ischemia or aspiration must also be considered. Blood
tests for histamine and tryptase, which are released from mast cells during systemic allergic reactions, can
be helpful, as can specific immunoglobin E concentrations. If these tests confirm an immune-mediated
event, investigating to identify the responsible agent and providing precise recommendations for either
desensitization or planning for future anesthesia is indicated. Of note, muscle relaxants and latex were
responsible for >80% of allergic reactions during anesthesia in a large French study, illustrating the
importance of identifying the causative allergen in such cases.

The negative predictive value of tryptase is poor, however; although elevated tryptase levels strongly
suggest anaphylaxis, normal levels do not rule out allergic reaction as the cause of hypotension.

Procalcitonin and endotoxin are elevated in septic shock. Troponin may be elevated due to a primary
ischemic event or due to poor myocardial perfusion from any cause of shock.

Metanephrine would be elevated in pheochromocytoma, which is a cause of hypertension after induction

of anesthesia, not hypotension.
Answer: D

Question 6:
A 48-year-old man presented to your office for evaluation of hypertension that had been difficult to
control. He denied chest pain, shortness of breath, or palpitations. He reported compliance with
medications. He exercised by walking 45 minutes three times/week. He had no other medical problems.
He took amlodipine 10 mg daily, chlorthalidone 25 mg daily, and losartan 100 mg daily. He denied
smoking and did not drink alcohol or use illicit substances.
On examination, his heart rate was 78 bpm and blood pressure (BP) was 160/90 mm Hg in both arms.
Jugular venous pressure was 6 cm H20. Lungs were clear to auscultation. Heart was regular with no
murmurs or gallops. Abdomen was soft and nontender. Extremities were warm without edema.
Laboratories showed sodium 145 mEq/L, potassium 3.5 mEq/L, bicarbonate 34 mEq/L, and creatinine 1.0
mg/dL. Plasma renin activity was 0.5 ng/mL/hr and plasma aldosterone concentration 18 ng/dL.
What is the best next step?

A. Laparoscopic adrenalectomy.
B. Adrenal vein sampling.
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C. Stop losartan and recheck aldosterone/renin ratio.

D. Oral sodium loading test.
E. Adrenal computed tomography scan.

The presenting signs of primary aldosteronism (PA) are hypertension and hypokalemia, but potassium
levels are frequently normal. PA may be associated with resistant hypertension, defined as failure to
achieve goal BP despite adherence to three drugs, including a diuretic. This patient has classic findings of
resistant hypertension, mild hypernatremia, hypokalemia, and metabolic alkalosis.

Testing for PA is recommended in patients with hypertension and spontaneous or low-dose, diuretic-
induced hypokalemia or severe and/or drug-resistant hypertension. The first step is measurement of a
morning plasma renin activity (or plasma renin concentration) and the plasma aldosterone concentration.
These can be used to calculate the aldosterone/renin ratio (ARR).

The threshold to define an abnormal ARR varies with the assay methods, but an ARR >30 generally
suggests PA. If the ARR suggests PA, confirmatory testing is required: oral sodium loading test, saline
infusion test, fludrocortisone suppression, or captopril challenge. Thus, oral sodium loading test is the
correct answer.

Losartan may cause a false-positive elevated ARR, but it is not recommended to stop losartan to repeat
the ARR; in cases where PA is suspected based on a high ARR, it is more efficient to proceed to a
confirmatory test. Thus, stopping losartan is not the best choice.

If PA is confirmed on subsequent testing, then an adrenal computed tomography should be performed to

exclude large masses that may represent adrenocortical carcinoma and to assist the interventional
radiologist and surgeon in management. Until PA has been confirmed, however, this is not an appropriate
test.

Adrenal vein sampling is done to make the distinction between unilateral and bilateral adrenal disease
when surgical treatment is feasible and desired by the patient. It is premature to perform until PA has
been confirmed.

Laparoscopic adrenalectomy is the preferred treatment for patients with unilateral PA because BP and
serum potassium concentrations improve in nearly 100% of patients postoperatively. However, it would
not be performed until PA is confirmed and unilateral disease established.
Answer : D

Question7:

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A 68-year-old African American woman with a history of hypertension and tobacco abuse presented to
the emergency department with new facial swelling. Three weeks prior, hydrochlorothiazide and lisinopril
were started for hypertension. She reported that she had never had such an episode before and had no
family history of such episodes. Her medications included aspirin 81 mg daily, hydrochlorothiazide 25
mg daily, and lisinopril 10 mg daily. Her blood pressure was 142/86 mm Hg with a heart rate of 78 bpm.
Her examination revealed mild lip and tongue swelling.
Which of the following would be the most appropriate laboratory test for diagnosis of her condition?
A. No laboratory testing.
B. Complement protein 4 level.
C. Antinuclear antibody.
D. Urinalysis.
E. Erythrocyte sedimentation rate.

The clinical presentation is consistent with angiotensin-converting enzyme (ACE) inhibitor–induced

angioedema. This classically presents in most patients within the first 3 months of initiation of ACE
inhibitor (e.g., lisinopril). Female sex, smoking, aspirin use, and especially African descent all increase
the risk of this condition. The diagnosis is made clinically when patients on an ACE inhibitor present with
classic facial swelling. No laboratory tests can diagnose ACE inhibitor–induced angioedema, which is
much more common than other causes of angioedema. Thus, additional laboratory testing is generally not
recommended unless there is suspicion of an underlying rare angioedema disorder (i.e., if there were
family history of angioedema, personal history of malignancy, or prior angioedema event), which is not
the case in this patient. The ACE inhibitor should be discontinued.

Obtaining a complement protein 4 level would be a reasonable first test if there were suspicion of an
underlying rare angioedema disorder—it would be normal in ACE inhibitor–associated angioedema.
Antinuclear antibody, urinalysis, and erythrocyte sedimentation rate can be considered in limited cases of
recurrent angioedema without a clear trigger.
Answer : A

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Question8:
A 51-year-old woman was referred to your office for evaluation of a murmur. She denied chest pain,
shortness of breath, or palpitations. She walked 30 minutes daily for exercise without limitations. She had
no medical problems and took no medications.

On examination, her heart rate was 78 bpm and blood pressure 138/70 mm Hg. Jugular venous pressure
was 4 cm H20. Lungs were clear. There was a II/VI early peaking systolic ejection murmur. Abdomen
was soft and nontender. Extremities were warm without edema. An echocardiogram showed left
ventricular ejection fraction 60% with a calcified aortic valve. The peak/mean gradient was 20/12 mm Hg
across the aortic valve with calculated valve area 2.1 cm2.

Laboratories showed potassium 3.9 mEq/L (normal 3.5-5.0 mEq/L), magnesium 1.7 mg/dL (normal 1.5-
2.5 mg/dL), calcium 11.1 mg/dL (normal 8.5-10.5 mg/dL), and phosphorus 2.5 mg/dL (normal 2.5-4.5
mg/dL).
What is the best next step?
A. Serum parathyroid hormone.
B. Serum protein electrophoresis.
C. Repeat echocardiogram.
D. Hydrochlorothiazide 25 mg daily.
E. Coronary computed tomography angiogram.

Primary hyperparathyroidism may be associated with cardiovascular disease, including hypertension,

arrhythmia, ventricular hypertrophy, and vascular and valvular calcification.

This patient has hypertension and aortic valve calcification causing aortic sclerosis. She has an elevated
calcium and low-normal phosphorus and low-normal magnesium, all consistent with primary
hyperparathyroidism. A serum parathyroid hormone level should be checked.

Serum protein electrophoresis (SPEP) may detect a monoclonal gammopathy consistent with amyloid
light-chain (AL) amyloidosis or multiple myeloma. However, there is nothing else in her history to
suggest this diagnosis aside from the hypercalcemia. Furthermore, serum immunofixation electrophoresis
is preferred over SPEP to diagnose AL amyloidosis.

The echocardiogram does not show aortic stenosis. Thus, surveillance echocardiography is not indicated
unless the patient develops new symptoms or a change in the murmur.

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The patient is hypertensive and should start antihypertensive therapy. However, hydrochlorothiazide (a
thiazide diuretic) impairs calcium excretion by the kidney and can cause hypercalcemia, so this would not
be the best agent for this patient.

In an asymptomatic patient with good exercise tolerance, there is no indication for coronary computed
tomography angiogram.
Answer : A

Question 9:
A 50-year-old man presented to your office for cardiovascular evaluation. He had no symptoms but he
was sedentary at baseline. His past medical history included obesity. He was on no medications.

An examination revealed blood pressure 132/86 mm Hg, pulse 80 bpm, height 5'8", and weight 214 lbs.
His waist circumference was 41 inches. The neck was supple and there were no carotid bruits. The
cardiac examination was unremarkable.

His fasting lipid profile total cholesterol was 205 mg/dL, low-density lipoprotein 133 mg/dL, high-density
lipoprotein (HDL) 32 mg/dL, triglycerides 201 mg/dL, and fasting glucose 105 mg/dL.
Which intervention is most likely to reduce cardiovascular risk for this patient?
A. Exercise.
B. Metformin.
C. Vegan diet.
D. Niacin.
E. Fish oil.

Metabolic syndrome is highly prevalent in the United States, with more than 1:3 adults meeting
diagnostic criteria. As stated in a joint report by the American Heart Association (AHA) and National
Heart, Lung, and Blood Institute (NHLBI), aggressive lifestyle modification is the primary goal for
patients with metabolic syndrome.

Pharmacologic interventions may be appropriate in certain patients; however, broad implementation is

inappropriate. Metformin prescription would be encouraged in patients with a diagnosis of diabetes
mellitus, which he does not have. Metformin may delay the development of diabetes mellitus in patients
with metabolic syndrome but is less effective overall than intensive lifestyle modification.

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A vegan diet has not been shown to reduce cardiovascular events in prospective clinical trials. A
Mediterranean diet may reduce cardiovascular risks in metabolic syndrome patients.

The patient has a low HDL but niacin has not been shown to improve cardiovascular outcomes in this
setting and may have harmful side effects.

Therapeutic doses of fish oil may be used for very high triglycerides but have not been shown to be
effective for modest elevations of triglycerides, which this patient has.
Answer : A

Question 10:
A 65-year-old man presented to the emergency department with lethargy, nausea, and generalized
abdominal pain. The patient was arousable to voice but unable to provide any history. His family was
present at the bedside and reported worsening fatigue over the last week, including nausea and vomiting
such that he had not been taking any medications. His past medical history included chronic obstructive
pulmonary disease (COPD) and hypertension. He was a smoker with a 75-pack-year history. He was
admitted to the hospital 6 months prior with COPD exacerbation that was treated with prednisone 40 mg
daily and albuterol and ipratropium inhalers. He had not seen a clinician since that hospital stay.
On examination, he was afebrile, pulse was 110 bpm, blood pressure was 76/32 mm Hg, respiratory rate
was 12/min, and oxygen saturation was 91% on 2 L oxygen. He was arousable to voice and oriented to
person only. He had central obesity with striae on his lower abdomen and ecchymoses on his upper and
lower extremities. His cardiovascular examination was normal except for tachycardia. His jugular venous
pressure was difficult to assess due to his body habitus. His lungs were clear to auscultation and abdomen
was soft but tender.
His initial laboratories included:
White blood cell count: 6,000/uL
Hemoglobin: 12 g/dL
Hematocrit: 36%
Platelet count: 175,000/uL
Sodium: 120 meq/L
Potassium: 5.6 meq/L
Bicarbonate: 22 meq/L
Calcium: 10.2 mg/dL
Magnesium: 1.9 mg/dL
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Blood urea nitrogen: 32 mg/dL

Creatinine: 1.4 mg/dL
Troponin I: 0.20 ng/mL
Lactic acid: 4.5 mmol/L

An electrocardiogram was performed, which revealed sinus tachycardia. Chest radiograph and
computerized tomography of the abdomen were also normal.
Which of the following is the most appropriate diagnostic study to determine the cause of the patient's
hypotension?
A. Adrenocorticotropic hormone stimulation test.
B. Transthoracic echocardiogram.
C. Thyroid function tests.
D. Procalcitonin.
E. Urinary drug screen.

The patient presents with undifferentiated hypotension. It is important to rapidly identify the etiology of
shock and administer appropriate therapy to prevent multisystem organ failure and/or death.

The patient has clinical features of early shock including hypotension, tachycardia, decreased mental
status, and elevated lactate. From the clinical history, the patient has some unique historical and clinical
features.

His examination describes Cushingoid features including central obesity, abdominal striae, and bruises.
The abrupt discontinuation of high-dose corticosteroids without taper (due to vomiting in this case) can
precipitate adrenal crisis, which may be the cause of this patient's shock.

The patient's presentation is consistent with adrenal crisis: Hyponatremia is common with adrenal crisis
due to sodium and volume loss with cortisol deficiency and compensatory vasopressin secretion.
Hyperkalemia is also a common finding.

With suspected adrenal crisis, an early morning cortisol level can exclude adrenal crisis. A value >11
mcg/dL excludes adrenal insufficiency, whereas a very low level (<3 mcg/dL) significantly increases the
likelihood of adrenal insufficiency. The confirmatory test is to perform dynamic testing using an
adrenocorticotropic hormone (ACTH) stimulation test (i.e., the cortisol response to ACTH), which can
help establish the diagnosis and in some cases differentiate the cause of adrenal insufficiency, so this is
the best answer.

Despite having a slightly elevated troponin I, patient does not have clinical features that are consistent
with cardiogenic shock. Thus, a transthoracic echocardiogram may be helpful but will not diagnose the
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patient with adrenal crisis.

Thyroid function tests will not be helpful in this patient. Altered mental status can be encountered with
myxedema coma but the patient's clinical features are not consistent with this condition (typically altered
mental status with hypothermia).

Procalcitonin is an acute phase reactant that is nondiagnostic and has limited clinical utility in the
evaluation of suspected adrenal crisis and/or shock.

Urinary drug screen can be useful in patients with altered mental status but the history is not suggestive of
drug overdose or common toxidrome.
Answer: A

Question11:
A 56-year-old man with a history of hypertension and tobacco abuse presented with chest pain and
elevated troponin consistent with non–ST-segment elevation myocardial infarction. He underwent cardiac
catheterization and received a drug-eluting stent to his right coronary artery. He was treated with aspirin
81 mg, ticagrelor 90 mg twice daily, lisinopril 40 mg, and atorvastatin 80 mg daily. During his
hospitalization, he complained of severe nicotine withdrawal symptoms.
Which of the following is the best next step in his care?
A. Nicotine patch.
B. Varenicline.
C. Nortriptyline.
D. Lorazepam.
E. Bupropion.
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In this inpatient with acute coronary syndrome, the first-line therapy to relieve symptoms of nicotine
withdrawal is nicotine patch or combination nicotine replacement therapy (NRT). At the time of
discharge, first-line therapy is combination NRT or varenicline. It is helpful to think about tobacco
cessation for patients with coronary artery disease (CAD) in the acute versus chronic setting when
considering pharmacotherapy choices to aid cessation. Many of the pharmacotherapies other than nicotine
replacement require 1-4 weeks of treatment prior to cessation of tobacco use. In the acute setting, such as
an inpatient, tobacco replacement has been shown to be safe and is appropriate to relieve withdrawal
symptoms. Buproprion is given 1-2 weeks prior to the quit date to improve the quit rate. It should be
avoided in anyone at risk for seizures. Varenicline is titrated during the first week and can be started 1-4
weeks prior to the quit date. It is centrally acting, relieves nicotine withdrawal symptoms, and blocks the
reward signals from smoking. Nortriptyline has not been well studied in patients with CAD and is not
approved by the Food and Drug Administration for smoking cessation. Benzodiazipines are not indicated
in the treatment of tobacco-withdrawal symptoms.
Answer : A

Question12:
You were seeing a 55-year-old man in clinic for follow-up of coronary artery disease. He had
percutaneous coronary intervention for stable angina a year prior and had no complaints today. He
smoked half a pack of cigarettes a day and wanted your advice on whether switching to smokeless
tobacco would reduce his risk of future cardiovascular events. His medical history included hypertension
and dyslipidemia. His current medications were aspirin 81 mg daily, atorvastatin 80 mg daily, lisinopril
20 mg daily, metoprolol 50 mg extended-release daily, and prasugrel 10 mg daily.
Which cardiovascular effect of tobacco smoking is most likely to be reduced by replacing cigarettes with
smokeless tobacco?
A. Myocardial infarction.
B. Heart failure.
C. Insulin resistance.
D. Coronary vasoconstriction.
E. Atrial fibrillation.

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Unlike cigarette smoking, smokeless tobacco does not appear to increase the risk of atrial fibrillation.
This finding is based on two large studies from Scandinavia, where a form of smokeless tobacco called
"snus" is the most commonly used product.

Cigarette smoking can cause coronary spasm and is a risk factor for vasospastic angina. It is unclear to
what extent these adverse effects of smoking on coronary blood flow are due to adverse effects on
endothelial function of nicotine versus smoke constituents.

Cohort studies in the United States and the InterHeart study in Europe did find increased rates of
myocardial infarction and heart failure in people who use smokeless tobacco compared with those who do
not.

Cigarette smoking is an important risk factor for developing type 2 diabetes mellitus and smokers have
increased insulin resistance compared with nonsmokers, although the mechanism of this effect is unclear.
Studies of nicotine gum have reported insulin resistance among long-term users, suggesting that nicotine
by any delivery mechanism is responsible for this effect.

Cigarettes, but not smokeless tobacco, also appear to activate platelets. Similarly, smokeless nicotine
delivery systems do not appear to elevate inflammatory markers such as leukocyte count, C-reactive
protein, and fibrinogen.
Answer : E

Question 13 :
A 23-year-old woman presented to the hospital after fainting at home. She had a history of familial
adenomatous polyposis with previous subtotal colectomy. Her examination was notable for a III/VI harsh
systolic murmur along the left sternal border. A transthoracic echocardiogram revealed asymmetric
thickening of the interventricular septum with a distinct area of increased echogenicity and central
calcification within the midmyocardium. The anterior mitral valve leaflet appearance and motion were
normal without systolic anterior motion. An interventricular gradient was present across the left
ventricular outflow tract.
What is the best next test?
A. Cardiac magnetic resonance imaging.
B. Transesophageal echocardiography.
C. Left heart catheterization.
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D. Electrophysiology study.
E. Endomyocardial biopsy.

Cardiac magnetic resonance imaging (MRI) would be the most useful test here to evaluate the suspected
cardiac mass located within the interventricular septum. Cardiac MRI can be very helpful in assessing
cardiac masses by providing information about vascularity, fibrosis, and water and fat content, as well as
regarding surrounding cardiac structures. Cardiac masses include thrombi and tumors. Metastatic tumors
to the heart are much more common than primary cardiac tumors. Among primary cardiac tumors,
approximately 70% are benign, including myxomas, fibroelastomas, lipomas, fibromas, and
rhabdomyomas. Malignant cardiac tumors include angiosarcomas and rhabdomyosarcomas. Fibromas
comprise approximately 4% of all primary cardiac tumors. Fibromas typically present in children or
young adults and are usually located intramurally within the ventricles, including the interventricular
septum or free wall. They may cause mass effects, outflow-tract obstruction, or arrhythmias; they can
present with syncope, palpitations, chest pain, heart failure, or sudden cardiac death. Unlike
rhabdomyomas (the most common benign pediatric cardiac tumors), fibromas usually present as a single
tumor and also often include central calcification. Due to the large amount of collagen they contain, they
show delayed gadolinium enhancement on cardiac MRI. Fibromas can be associated with polyposis
syndromes including familial adenomatous polpyposis. On echocardiography, fibromas may be visible as
a "distinct, well-demarcated, non-contractile and solid, highly echogenic mass within the myocardium"
with central calcification possible.2 Symptomatic tumors may be treated by resection.

Transesophageal echocardiography would not likely provide additional information. Left heart
catheterization may redemonstrate the interventricular gradients and asymmetric septal thickening
observed on echocardiography but is not necessary. An electrophysiology study may demonstrate
inducible arrhythmias arising from the region of the mass but would not help in diagnosis of the cardiac
mass. An endomyocardial biopsy is unlikely to be of benefit because the mass is intramural.
Answer: A

Question 14:
A 49-year-old man presented to your office for assessment of cardiovascular risk because his father
recently died from heart disease at the age of 68. He exercised three to four times per week for >30
minutes and had made dietary modifications to lose 10 lbs over the prior 6 months. He took no
medications. His blood pressure was 145/85 mm Hg. His waist circumference was 105 cm (42 in). His
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laboratories showed total cholesterol 170 mg/dL, low-density lipoprotein 130 mg/dL, triglycerides 180
mg/dL, and fasting glucose 110 mg/dL. His 10-year atherosclerotic cardiovascular disease (ASCVD) risk
was 3.4% based on current values.
What is the next appropriate step?
A. Metformin.
B. Atorvastatin.
C. Aspirin.
D. Hydrocholorthiazide.
E. Metoprolol.

The patient in this vignette has metabolic syndrome by virtue of having a waistline circumference >101
cm (>40 inches), blood pressure >130/85 mm Hg, elevated fasting blood glucose >100 mg/dL, and
elevated serum triglycerides >150 mg/dL. He has already engaged in lifestyle modification.
Pharmacologic intervention is warranted. Initiation of antihypertensive therapy with a thiazide-type
diuiretic, calcium channel blocker, angiotensin-converting enzyme inhibitor, or angiotensin receptor
blocker is warranted. Beta-blockers are not first-line antihypertensive therapy. Metformin would be
reasonable if this patient were to have a diagnosis of diabetes mellitus; his blood glucose is in the
prediabetes range (100-125 mg/dL). Statin therapy would not be recommended in this patient with 10-
year ASCVD risk of <7.5%. Aspirin use for primary prevention is of questionable benefit based on recent
clinical trials.
Answer : D
Question 15 :
A 54-year-old woman presented to the primary care clinic for an initial assessment. She had concerns
about her cardiac risk because her brother, 45 years of age, recently experienced a myocardial infarction.
She denied cardiac symptoms. She took no medications. Her blood pressure (BP) was 150/90 mm Hg.
Laboratories included a serum creatinine of 1.2 mg/dL, serum sodium of 140 mg/dL, total cholesterol 190
mg/dL, and fasting blood glucose 103 mg/dL.
Which additional measurement would confirm the diagnosis of metabolic syndrome in this patient?
A. Lipoprotein(a) 30 mg/dL.
B. High-sensitivity C-reactive protein 2 mg/L.
C. Waist circumference 95 cm (38 in).
D. High-density lipoprotein cholesterol 51 mg/dL.
E. Triglycerides 145 mg/dL.

The most widely used clinical definition of the metabolic syndrome is the presence of any three of the
following five criteria: abdominal obesity (waist circumference in men ≥102 cm or ≥88 cm in women),
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elevated triglycerides (>175 mg/dL), low high-density lipoprotein (<40 mg/dL in men or <50 mg/dL in
women), elevated BP ≥130/85 mm Hg, and elevated fasting plasma glucose (≥100 mg/dL). In the
vignette, waist circumference is the only metabolic syndrome criterion not given. Although elevated
lipoprotein(a) and high-sensitivity C-reactive protein are suggestive of increased cardiovascular risk, they
are not components of the metabolic syndrome definition.

Question16:
A 64-year-old woman with diabetes mellitus, coronary artery disease, and three-vessel coronary bypass
grafting presented with acute onset of dysarthria and left upper extremity weakness. Symptoms began
while she was working in her garden and her husband called 911. She was transported by ambulance to
the emergency department (ED). On examination, her heart rate was 70 bpm with a blood pressure (BP)
of 170/98 mm Hg. She underwent an urgent head computed tomography, which demonstrated no acute
pathology.
Her neurologic symptoms lasted for approximately 90 mins and resolved shortly after arrival in the ED
prior to receiving medical treatment. Repeat examination showed no focal deficits.
An electrocardiogram demonstrated sinus rhythm with new anterior T-wave inversions. Electrolytes and
renal function were normal. Troponin was negative.
In the following week, this patient was at highest risk for which of the following?
A. Stroke.
B. Takotsubo cardiomyopathy.
C. Myocardial infarction.
D. Atrial fibrillation.
E. Aortic dissection.

Transient ischemic attack (TIA) is defined as a transient neurologic deficit caused by focal brain, spinal
cord, or retinal ischemia without acute infarction. Patients with TIA are at high risk of stroke in the days
following TIA. The ABCD2 score is used to estimate risk of stroke after TIA and is based on:

1) Age > 60 (1 point)

2) BP: systolic BP >140 or diastolic BP >90 (1 point)
3) Clinical symptoms: focal weakness (1 point), focal weakness + speech disturbance (2 points)
4) Duration: 10-59 minutes, 60 minutes or longer (2 points)
5) Diabetes mellitus: diabetes mellitus present (1 point)

The patient described has 7 of a possible 7 points, putting her at very high risk for cerebrovascular
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accident. The 2009 American Heart Association/American Stroke Association (AHA/ASA) guidelines for
the evaluation of TIA suggest that it is reasonable to hospitalize patients with TIA within 72 hours of
symptoms with an ABCD2 score of 3 or higher given the high risk of acute stroke. Based on the studies
used to develop this scoring system, patients with a score of 7 had a 2-day risk of stroke of 8%.

Multiple studies consistently demonstrate the high risk for stroke following TIA, although precise
estimates of incidence are limited by varying definitions of TIA. Nevertheless, large cohort studies have
demonstrated high risk of early stroke following TIA, with approximately 10-15% having a stroke within
3 months; half of these occur within the first 48 hours.

Although there may be some risk for myocardial infarction, cardiomyopathy, atrial fibrillation, and aortic
dissection, stroke remains significantly more common in the days following a TIA.
Answer: A

Question17:
A 78-year-old man with osteoarthritis, hypertension, diabetes mellitus, stage IV chronic kidney disease
(CKD), and coronary artery disease was admitted to the cardiology service with chest pain. He had
become free of chest pain and comfortable on low-dose nitroglycerine infusion (10 mcg/min).
Medications included unfractionated heparin infusion, aspirin 81 mg daily, clopidogrel 75 mg daily,
lisinopril 10 mg daily, metoprolol tartrate 25 mg every 6 hours, and atorvastatin 20 mg daily.

On examination, his heart rate was 80 bpm with blood pressure of 136/80 mm Hg. Oxygen saturation was
98% on room air. Jugular venous pressure (JVP) was below the clavicle and mucus membranes appeared
dry. Auscultation of heart and lungs was benign and there was no lower extremity edema.

Sodium and potassium levels were within normal limits. Initial troponin T was 0.10. Creatinine was 2.0
(estimated glomerular filtration rate [GFR]: 25), which was stable when compared with prior values.
Echocardiogram revealed normal left ventricular ejection fraction of 55% with hypokinesis of the basal
and midanterolateral wall. Right ventricular function was normal and there was no significant valve
disease. He was scheduled for nonurgent coronary angiogram tomorrow.
Prior to coronary angiography, which of the following will minimize the risk of contrast-induced
nephropathy (CIN)?
A. Start isotonic saline infusion.
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B. Initiate hemodialysis.
C. Discontinue lisinopril.
D. Increase dose of atorvastatin.
E. Start furosemide infusion.

The incidence of CIN reported in the medical literature varies based on the definition used to define acute
kidney injury and the risk factors present in the population being studied. Risk factors for CIN include:
underlying CKD, diabetes mellitus, and conditions that impair renal perfusion (i.e., heart failure,
hemodynamic instability). Avoiding CIN involves both identifying at-risk patients and implementing
preventive measures.

The patient described has diabetic nephropathy with stage IV CKD (i.e., GFR: 15-29 mL/min) and is
therefore at high risk for CIN. Furthermore, the physical examination suggests possible hypovolemia. To
minimize the risk of CIN in this patient, care should be taken to avoid volume depletion and nephrotoxic
medications such as nonsteroidal anti-inflammatory drugs. Additionally, limiting contrast exposure
during the procedure to the lowest effective dose possible is advisable.

In this patient, there is evidence of hypovolemia based on the physical examination (i.e., JVP below
clavicle with dry mucus membranes). Hypovolemia can cause renal vasoconstriction, increasing the risk
of CIN, so efforts should be to replete the volume deficit in patients without contraindications to volume
expansion. Diuretics should be held for the same reason.

There are a few trials that studied the administration of intravenous (IV) fluids prior to cardiac
catheterization in both the emergent and nonemergent setting. All demonstrated some benefit. In two
trials of patients with acute myocardial infarction undergoing cardiac catheterization, the administration
of IV saline reduced the risk of CIN.

There is not sufficient data to support discontinuing angiotensin-converting enzyme inhibitors or

angiotensin-receptor blockers, prophylactic hemodialysis, or prophylactic diuretic use for prevention of
CIN. In fact, the use of prophylactic diuretics prior to contrast administration could provoke hypovolemia,
therefore increasing the risk of CIN, particularly in this patient with evidence of hypovolemia.
Answer : A

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Question 18 :
A 46-year-old woman with a history of hypertension, asthma, and gastroesophageal reflux disease
presented to the emergency department following an episode of vision loss and altered sensorium. She
was standing watching her daughter's gymnastic competition when she developed nausea, "blurry vision,"
and lightheadedness. She felt a "tingly" sensation in both hands and then experienced a brief loss of
consciousness. She laid down on the bleachers and her symptoms resolved after 10 minutes. She had no
residual neurologic deficits.
Which of the following is the most likely diagnosis?
A. Transient ischemic attack.
B. Seizure.
C. Vasovagal syncope.
D. Vertebral artery dissection.
E. Atypical migraine.

The differential diagnosis of transient neurologic symptoms include: transient ischemia attack (TIA),
migraine with aura, seizure, syncope, and functional/anxiety. The patient describes a typical prodrome for
vasovagal syncope with lightheadedness, gradual vision, and nausea. She had been standing in one place
for a prolonged period of time in a hot gymnasium, which can lead to blood pooling in the extremities and
thus precipitate vasovagal response. Her symptoms resolved completely after laying flat.

TIA is characterized by the abrupt onset of numbness, weakness, and vision loss. Loss of consciousness
almost never occurs. Symptoms resolve gradually and usually last <1 hour.

Migraines usually consist of positive symptoms (visual disturbances: scotoma or geometric patterns), last
20-30 mins, and are often accompanied by a headache, nausea, and vomiting. Loss of consciousness
almost never occurs.

A seizure typically consists of painful sensory disturbance, limb jerking, amnesia, and a postictal state
(somnolence or Todd's paralysis). Symptoms usually last <2 min.

Vertebral artery dissection is generally characterized by headache, dysarthria, dysphagia, gait

unsteadiness, and unilateral vision loss. It is not a typical cause of syncope.
Answer : C

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Question 19:
A 64-year-old man presented from home with 2 hours of left-sided weakness. He had not seen a clinician
in 30 years and took no medications or supplements. Physical examination was notable for blood pressure
(BP) of 190/115 mm Hg and a heart rate of 84 bpm. Cardiac examination was notable for a regular
rhythm and normal S1 and S2 with an S4. There were no murmurs appreciated. Lungs were clear
bilaterally. Abdomen was soft without bruits.

The stroke team was activated. A noncontrast head computed tomography was performed and showed no
acute abnormalities.
What is the goal BP in this patient prior to administering intravenous (IV) alteplase?
A. <140/90.
B. <165/100.
C. <185/110.
D. <200/110.
E. <225/100.

The target BP prior to administration of IV alteplase is a systolic BP <185 and diastolic BP <110 (class I
recommendation). Some observational studies suggest that the risk of hemorrhage after administration of
alteplase is greater in patients with higher BPs and in patients with more BP variability.

However, the optimal BP level that should be maintained in patients with acute ischemic stroke to ensure
the best outcome is not known.

Patients with acute ischemic stroke not treated with thrombolysis should not have their BPs treated unless
they have severe hypertension (>220/120 mm Hg) or severe acute comorbidities such as acute coronary
syndrome, aortic dissection, acute heart failure, or eclampsia. In these cases, antihypertensives can be
used to achieve BP reduction of ≤15%.

Starting or restarting antihypertensive medications has been shown to be associated with improved
control of the BP after discharge. Therefore, it is reasonable to start or restart antihypertensive
medications in the hospital once the patient is neurologically stable (class IIa).
Answer: C

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Question20:
A 65-year-old man with diabetes mellitus, hypertension, and coronary artery disease was brought into the
emergency department by police for evaluation of alcohol intoxication. He admitted to drinking
approximately 12 beers and had chewed four pieces of a nicotine-containing gum. He quit smoking 1
month prior and had been using nicotine replacement therapy. He used a 14 mg transdermal patch as well
as nicotine gum.
The acute physiologic effects of nicotine commonly include which of the following?
A. Hypertension.
B. Bradycardia.
C. Psychosis.
D. Coronary vasodilation.
E. Hypoxia.

Nicotine is an alkaloid primarily found in tobacco and most commonly absorbed in the form of cigarette
smoke, which confounds the physiologic effects on the cardiovascular system given the multitude of
adverse health effects of smoke inhalation. Nicotine is also found in chewing tobacco, cigars, electronic
cigarettes, and a variety of nicotine replacement therapies (NRT) such as transdermal patches, gums,
tablets, and lozenges.

Nicotine's cardiovascular effects are caused by stimulation of the sympathetic nervous system via
nicotinic cholinergic receptors, thereby increasing blood pressure and heart rate. One study examined the
cardiovascular and sympathetic effects of NRT in the form of tablets and demonstrated an increase in
blood pressure and heart rate. There should not be an effect on oxygenation with NRT, although hypoxia
can be precipitated by inhaling cigarette smoke.
Common neurologic symptoms resulting from NRT include headache, insomnia, and depression, but not
psychosis.
Cigarette smoking has been shown to cause coronary vasoconstriction, not dilation, in patients with
coronary artery disease. There is no substantial evidence that NRT has the same physiologic effect (i.e.,
vasoconstriction). Nevertheless, NRT would be unlikely to cause coronary vasodilation.
Answer: A

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Question 21:
An 83-year-old woman with a history of hypertension, hyperlipidemia, and chronic kidney disease stage
IIIa presented from home with right-sided weakness and sensory loss that began 60 minutes prior. Initial
physical examination showed blood pressure 170/95 mm Hg, heart rate 115 bpm, and oxygen saturation
of 97% on room air. The examination was notable for a right facial droop and weakness of the right upper
and lower extremities with 2/5 motor strength. On auscultation, heart rate was tachycardic with a soft
apical systolic murmur. Lungs were clear to auscultation.
What is the next best step?
A. Electrocardiogram.
B. Noncontrast head computed tomography.
C. Intravenous alteplase.
D. Unfractionated heparin.
E. Brain magnetic resonance image.

The patient is presenting with acute focal neurologic deficits consistent with a cerebrovascular accident
(most suggestive of an acute left middle cerebral artery occlusion). Thrombolytics should be administered
to all eligible acute stroke patients within 3 hours of onset of symptoms. Centers should attempt to
achieve door-to-needle times of <60 minutes in 50% of stroke patients treated with intravenous tissue
plasminogen activators.

Prior to initiation of thrombolytics, a noncontrast head computed tomography (CT) and glucose should be
performed.

An electrocardiogram is indicated to evaluate for atrial fibrillation; however, this will not change acute
management and therefore the most critical first step is a noncontrast head CT.

Unfractionated heparin should not be started because this patient is a likely candidate for thrombolysis
and an intracranial bleed has not been excluded.

A brain magnetic resonance image may provide additional information; however, the additional time
required to perform this study will delay the administration of thrombolytics.
Answer: B

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Question22 :
A 48-year-old man with familial dilated cardiomyopathy was undergoing evaluation for cardiac
transplant. His workup was significant for a history of tobacco use. He stated that he no longer smoked.
Elevation of which of the following will confirm current tobacco use?
A. Cotinine.
B. High-sensitivity C-reactive protein.
C. Carbon monoxide.
D. Fibrinogen.
E. Interleukin-6.
Cotinine is the primary metabolite of nicotine and can be measured in the hair, saliva, urine, and serum; it
is the best biomarker of tobacco smoke exposure. Measuring cotinine rather than nicotine is preferred
because cotinine has a longer half-life in the body (16 hours). Serum cotinine levels reflect recent
exposure to nicotine in tobacco smoke and elevated levels are associated with current/recent tobacco use.
Nonsmokers can have low levels as a result of second-hand smoke. Carbon monoxide can be elevated in
smokers, but there are no thresholds to determine whether or not someone is actively smoking. Although
smoking can increase the inflammatory state in a smoker, high-sensitivity C-reactive protein and other
markers of inflammation such as interleukin-6 and fibrinogen are not specific to tobacco exposure.

Answer: A

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