VALVULAR

HEART DISEASE
 Valvular heart disease (VHD) is an
important, and challenging, clinical entity.
 During recent decades, important
changes have occurred in the
geographical distribution of the
aetiologies of VHD in Western countries.
 The continuous decline of acute
rheumatic fever explains the decrease in
the incidence of rheumatic valve disease,
while increased life expectancy partially
accounts for the increase in the incidence
of degenerative valvular diseases.
 The incidence of endocarditis remains stable
and other aetiologies, such as congenital,
inflammatory or carcinoid valve diseases,
remain rare .
 Emerging aetiologies that have been identified
more recently, in particular drug- or radiation-
related valve lesions, may also be an occasional
cause .
 Nevertheless, rheumatic valve disease still
remains a major public health problem in
developing countries, where it predominantly
affects young adults.
 Most patients were not aware of the diagnosis
and prophylaxis was under-used.
 AORTIC STENOSIS
Aetiology
 The most frequent aetiologies are
degenerative (accounting for 80% of
the cases in Western countries),
followed by rheumatic and congenital.
 Rheumatic disease is characterized by
commissural fusion and fibrosis, with
retraction and stiffening of the cusps.
 Bicuspid valves are of unequal size
and tend to progressively thicken and
calcify
 Other rare causes are: Paget disease,
ochronosis, Fabry disease, lupus
erythematosus, and drug-induced
diseases.
 PATHOPHYSIOLOGY
 Normal aortic valve area is 2–4 cm2.
 Inflammatory change occurs within the
valve, with calcium deposited along flexion
lines causing immobility, reduced
excursion, and reduced opening area.
 Rheumatic AS is due to adhesion and fusion
of commisures, with fibrosis and retraction.
 A gradient between left ventricle (LV) and
aorta appears if valve area is < 1.5 cm2
and AS is considered severe when the area
is < 1 cm2 or, more accurately, 0.6
cm2/m2 body surface area (BSA).
 Progressive narrowing of the valve orifice
results in compensatory LVH to maintain
stroke volume.
 LVH results in a stiff, non-compliant ventricle
with elevated end-diastolic pressure
(diminished coronary perfusion pressure).
 Atrial component of LV filling then becomes
more significant.
 Pressure overload eventually results in LV
failure (pulmonary oedema, low cardiac
output) and fall in transvalvular gradient
because of reduced EF through valve.
 The pressures in the left atrium and
pulmonary artery rise, leading to
dyspnoea.
 Syncope on exertion occurs when
elevated LV pressures stimulate
baroreceptors located in the LV,
inducing arterial hypotension,
decreased venous return and
bradycardia.
 CLINICAL FEATURES
History
 Frequently, the diagnosis is made when a
systolic murmur is detected during a
routine physical examination.
 Classical triad: angina, syncope, dyspnoea
 Syncope, or light-headedness, occurs on
exertion.
 Later, dyspnoea may progress to overt
heart failure.
 In practice, AS may be discovered during
attempted diagnosis of unexplained
congestive heart failure.
 Angina pectoris: result from increased
Oxygen demand due to LVH and
increased wall tension.
 sudden death from ventricular
arrhythmias
Physical examination
 In severe AS the carotid pulse is
typically slow rising and of small
volume.
 Narrow pulse pressure
 Prominent ‘a’ wave on JVP.
 Sustained, heaving apical impulse.
 Systolic thrill is felt in the aortic area
(2nd intercostal space on right)
 during full expiration.
Auscultation:
 S 1 normal or soft.
 In severe AS, there is paradoxical splitting
with A 2 after P 2
 S 4 is often heard (atrial contraction into stiff
ventricle) and systolic ejection click (if valve
pliable).
 Mid-systolic crescendo–decrescendo ejection
murmur is heard throughout precordium but
best heard at right upper sternal border in full
expiration radiates to carotids.
 As AS severity worsens, the murmur peaks
later, extends later through systole, and may
 obscure S 2 .
 INVESTIGATION
Electrocardiogram
 LVH with strain;
 Left bundle branch block (LBBB),
 Left atrial enlargement (LAE).
 Atrial fibrillation can be seen at a late
stage.
Chest radiograph
 calcifi cation in valve or aortic root,
post-stenotic dilatation in ascending
[Link]-stenotic dilatation of the
ascending aorta is frequent.
 Overall cardiac silhouette and
pulmonary vascularization are normal
unless cardiac decompensation is
present.
Echocardiography
 Define the valve anatomy,
 Assess the severity of stenosis,
 Identify the etiology
 Assessment of the other valves
 Assessment of the cardiac chambers
specially the LV .
 Assess the LV function
 Estimate the pulmonary pressure
 Detect any vegetation or thrombi
Exercise testing
 Exercise-induced hypotension, or
inadequate rise in blood pressure, is a
good predictor of poor outcome
Cardiac catheterization
 Assess for concomitant coronary artery
disease prior to aortic valve surgery
 NATURAL HISTORY
 Large population-based studies have
shown that AS has an important impact
on prognosis as it was associated with a
50% increased risk of cardiovascular
mortality and morbidity .
 Outcome is poor when any symptoms
are present, with survival rates of only
15–50% at 5 years
 MANAGEMENT
Medical treatment
 No medical treatment for AS is able to to
prevent or delay the disease process in
the AV leaflets and delay the inevitability
of surgery.
 There is no effective medical treatment to
improve prognosis
 Inotropes and diuretics may transiently
improve heart failure.
 B -blockers reduce myocardial O 2
demand and may improve coronary blood
flow
Surgical treatment
 Aortic valve replacement is the
definitive therapy for severe AS.
 It uses either a mechanical or a
bioprosthesis
 After successful valve replacement long-
term survival rates are close to those
expected, symptoms are less marked
and quality of life is greatly improved
 Surgery can be recommended in the
following circumstances:
 In patients with severe symptomatic AS
 In asymptomatic patients with severe
AS and EF<50 %
 AORTIC REGURGITATION
 AR may be the consequence of diverse
etiologies, the distribution of which has
changed over time.
 Aetiology influences patient
management, in particular when the
ascending thoracic aorta is involved.
 The severity of AR, its consequences on
LV, and the morphology of the
ascending aorta should be assessed at
an early stage to allow for timely and
appropriate intervention.
 ETIOLOGY
 Less common than AS.
 Can be acute or chronic
Acute AR
 Infective endocarditis
 Aortic dissection
 Trauma

Chronic AR
• Rheumatic heart disease
• Bicuspid aortic valve
• Endocarditis
• Hypertension
• Seronegative arthritides
• Aortitis, eg: Syphilis (now rare)
• Connective Tissue Diseases eg:Marfan’s syndrome,
Osteogenesis imperfecta (very rare)
. SLE , RA.
Pathogenesis
 Valvular disease: Valvular fibrosis and
fusion of cusps results in retraction of
cusps such that they cannot coapt
properly.
 Subacute bacterial endocarditis (SBE):
Direct destruction of valve
cusps/vegetations.
 Aortic root disease: Progressive
dilatation of aortic root causes failure of
coaptation of cusps and regurgitation.
 PATHOPHYSIOLOGY
 Acute severe AR in a non-dilated LV
causes a large increase in end-diastolic
pressure, whereas cardiac output
decreases.
 In chronic AR, progressive LV
enlargement limits the increase in LV
end-diastolic pressure and enables total
stroke volume to increase, thereby
compensating for the regurgitant
volume and helping preserve normal
cardiac output.
 Volume overload is compensated by
eccentric LV hypertrophy.
 As myocyte function deteriorates,
further LV dilatation occurs, leading to
LV decompensation.
 LV dysfunction is potentially reversible if
related to afterload mismatch but may
persist after the correction of AR if
related to structural myocardial injury.
 CLINICAL FEATURES
History
 In acute AR, dyspnoea is common
( increase forward CO in a setting of
noncompliant LV leads to pulmonary
oedema), hypotension, and cardiogenic
shock.
 Acute AR is a medical emergency.
 In chronic AR, initially patient is
asymptomatic.
 Chronic volume overload leads to LV
failure and CHF.
 Angina
Physical examination
 Collapsing pulse and wide pulse pressure.
 Corrigan’s sign (visible carotid pulsation)
 De Musset’s sign (head nodding with each
pulse)
 Müller’s sign (visible pulsation of uvula)
 Traube sign (‘pistol shot femorals’ — loud bruit
heard with stethoscope over femoral artery),
 Quincke sign (visible capillary pulsation in
nailbed),
 Duroziez sign (systolic and diastolic bruits
heard over femoral artery when artery is
digitally compressed).
 Apex beat displaced inferolaterally and
diffuse/hyperdynamic.
 May feel apical systolic thrill.

Auscultation:
 A 2 may be normal (or louder) if AR is
due to aortic root pathology, and may
be soft or absent if AR is due to aortic
valve pathology.
 S 3 may be heard
 Early diastolic murmur, usually maximal at
lower left sternal edge, but may be best
heard in the aortic area, particularly if the
aorta is significantly dilated for any reason,
best heard with patient sitting up and leaning
forward in expiration.
 Apical mid-diastolic murmur (Austin Flint
murmur )
 INVESTIGATIONS
Electrocardiography
 LV hypertrophy is the main feature of AR.
 Left axis deviation

Chest radiograph
 Cardiomegaly is the main abnormality in
chronic AR.
 Signs of left heart failure are only
observed at an advanced stage, except
in acute AR.
 Dilated ascending aorta with aortic root
pathology.
Echocardiography
 Assess the anatomy of aortic leaflets and
the aortic root
 Identification of the etiology of AR.
 Quantify AR
 Assessment of the other valves
 Assessment of the cardiac chambers
specially the LV .
 Assess the LV function
 Estimate the pulmonary pressure
 Detect any vegetation or thrombi
Exercise testing
 The main interest of exercise testing is
to objectively evaluate exercise
capacity.
Imaging of ascending aorta
 Morphology of the ascending aorta can
be analysed and accurately quantified
using computerized tomography or
magnetic resonance imaging.
Cardiac catheterization
 Quantification of AR, LV volumes and
ejection fraction is generally obtained
non-invasively using echocardiography.
 Coronary angiography is required in
preoperative evaluation
 MANAGEMENT
Medical treatment
 symptoms of CCF respond to loop
diuretics and
 Vasodilators (ACE-Is, calcium-channel
blockers) offer good symptomatic relief
and may improve haemodynamic
profile.
 Anginal chest pain can be treated with
nitrates but use B -blockers with
caution.
Surgical treatment
 If AR is acute, then surgery is the
treatment of choice.
 In chronic AR:
 if there are symptoms, replacement
should be offered.
 EF <50 %
 LV dilatation
 MITRAL STENOSIS
Aetiology
 Rheumatic heart disease is almost
the unique cause of MS(fusion of one or
both commissures; thickening, fibrosis,
and calcification of the valves; and
shortening, thickening, and fusion of the
subvalvular apparatus.).
 Other valves are also involved in over
one-third of cases, the most frequent
associated lesions being tricuspid
disease and AR
 Other rare aetiologies are
 Degenerative mitral annular calcification
 Congenital
 Carcinoid disease
 Fabry disease
 Mucopolysaccharidosis
 Whipple disease
 Gout
 Rheumatoid arthritis
 SJE
 Methysergide therapy
 PATHOPHYSIOLOGY
 Normal mitral valve area is 4–6 cm2.
 After rheumatic attack, the alterations of
the valve slowly progress, mostly driven
by the abnormal flow dynamics caused
by the initial and eventually repeated
rheumatic insults.
 A diastolic transvalvular gradient
between left atrium and LV appears if
valve area is less than 2 cm2.
 MS is significant if valve area is < 1.5
cm2, or 1 cm2/m2 BSA in large
individuals.
 Valve obstruction progressively limits cardiac
output and increases pressure in the left
atrium, which, in turn, raises pulmonary
circulation pressure and pulmonary
hypertension.
 Pulmonary oedema, occurs when mean
capillary pressure is > 25 mmHg.
 Chronic
 pulmonary hypertension causes right
ventricular hypertrophy, which, possibly
exacerbated by tricuspid regurgitation (TR),
causes failure of the right ventricle.
 Intrinsic LV contractility is usually preserved
 Atrial fibrillation, which is not strictly linked
to the severity of MS, is a consequence of
left atrial dilatation and hypertrophy, as
well as rheumatic insult to the atria
 Atrial fibrillation causes haemodynamic
compromise through decreased cardiac
output due to the loss of atrial contraction
and shortening of diastole.
 It also increases thromboembolic risk as a
result of left atrial enlargement, blood
stagnation and increased concentrations
of prothrombotic markers.
 CLINICAL FEATURES
History
 Usually, symptoms appear gradually over years,
with patients first reporting dyspnoea on exertion.
 Pregnancy, emotional stress, sexual intercourse,
infection or the onset of atrial fibrillation may all be
precipitating factors of marked dyspnoea or
pulmonary oedema.
 Haemoptysis, as well as chest discomfort, is
infrequent.
 Haemoptysis can occur for a variety of reasons:
alveolar capillary rupture (pink frothy pulmonary
oedema); bronchial vein rupture (larger
haemorrhage)
 Atrial fibrillation often begins in paroxysms and
eventually becomes established.
 Embolic events, which may be the
initial event in 20% of cases, are most
often cerebral and leave sequelae in
one-third of cases.
 At a more advanced stage, patients may
complain of discomfort due to
hepatomegaly, fatigue, weakness
and occasionally hoarseness.
Physical examination
 Mitral facies or malar flush seen in <50 % .
 Prominent ‘a’ waves in jugular venous
pulse (JVP).
 Low-amplitude arterial pulse.
 Irregularly irregular pulse in AF.
 ‘Tapping’ apex beat — palpable S 1 .
 Diastolic thrill at apex.
 Left parasternal heave (due to right
ventricular hypertrophy (RVH)).
 Palpable pulmonary closure.
Auscultation:
 S 1 loud if in sinus rhythm and valve is pliable.
 P 2 accentuated.
 Opening snap (OS) of the MV is heard best at or
medial to the apex in expiration.
 A 2 –OS interval varies inversely with severity of
stenosis.
 Low pitched,rumbling, mid-diastolic murmur with
pre-systolic accentuation (if in sinus rhythm) is
heard best at the apex with the patient in a left
lateral position.
 Early diastolic murmur due to pulmonary
regurgitation from pulmonary hypertension
(Graham Steell murmur) may be heard rarely.
 INVESTIGATIONS
 Electrocardiography (ECG):
 P mitrale — bifid P wave (if in sinus rhythm) due
to LA enlargement most prominent in lead II.
 Tall and peaked P waves in pulmonary
hypertension.
 AF is frequent. Right-axis deviation and RV
hypertrophy.
 Chest X-ray (CXR):
 Straightening of the left heart border,
 prominent upper lobe veins,
 pulmonary artery enlargement,
 Kerley B lines — interstitial oedema.
 Large LA visible as a double shadow.
 Transthoracic ECHO (TTE):
 The mitral valve orifice can be
calculated and assess the severity
 Establish the underline etiology
 Assessment of the other valves
 Assessment of the cardiac chambers
specially the LV .
 Assess the LV function
 Estimate the pulmonary pressure
 Detect any vegetation or thrombi
 Transoesophageal
echocardiography (TOE):
 Provides better anatomic details, can
visualize small vegetations and thrombi
in the LA.
 Cardiac catheterization:
 Not always required.
 Assess pulmonary capillary wedge
pressure (PCWP).
 Assessment of co-existing coronary and
valvular lesions.
 MANAGEMENT
Medical management
 Mild symptoms: salt intake restriction
and oral diuretics (cautious).
 In AF: digoxin, B-blocker, or calcium-
channel blocker for rate control.
 Anticoagulation: recommended for
those with AF, prior thromboembolism,
or LA thrombus.
 There is no proven benefit if the patient
is in sinus rhythm.
Balloon valvotomy
 Suitable for patients with pliable valves
with minimal MR, no subvalvular
distortion, and without heavy
calcification
 Contraindicated in moderate or severe
MR or atrial thrombus.
 A guide wire is placed in the LA after
trans-septal puncture, and a balloon) is
directed across the valve and inflated at
the orifice.
Surgical treatment
 Indicated in symptomatic patients with
severe MS if the valve is not suitable for
percutanaous mitral balloon
valvuloplasty
 Mitral repair is occasionally possible
 Mitral valve replacement if repair is not
possible
 MITRAL REGURGITATION
 Acute:
 Infective endocarditis,
 acute myocardial infarction (MI),
 trauma.
 Chronic:
 Chronic rheumatic heart disease
 Mitral valve prolapse
 Left ventricular or annular dilatation of any cause
(e.g. cardiomyopathy, annular calcification)
 Degeneration of valve cusps
 Connective tissue disease eg:Marfan’s syndrome,
Osteogenesis imperfecta (very rare)
 Hypertrophic cardiomyopathy
 PATHOPHYSIOLOGY
 Damage to leaflets, chordae, papillary muscles, or LV
can cause MR.
 The LV ejects into the LA during systole as a results
from incomplete mitral valve closure, as well as
antegrade into the aorta.
 In acute MR:
 Acute MR, resulting from papillary muscle or chordal
rupture, induces an immediate decrease in afterload,
LV emptying increases, and left atrial pressure rises
acutely with little enlargement of the LA due to
normal compliance.
 This results in raised LA pressure which can be
transmitted back to the pulmonary circulation and
can result in pulmonary oedema
 There is no compensatory LV
enlargement, but LV function is normal
and ejection fraction is increased but
forward stroke volume is reduced
resulting in tachycardia to maintain
cardiac output.
In longstanding MR:
 There is enlargement of the LA, which
accommodates the volume overload
with minimal rise in LA pressure.
 The LV dilates, and large stroke volume
compensates for regurgitation,
maintaining the forward EF.
 LV failure results from longstanding
volume overload.
 CLINICAL FEATURES
 acute severe MR:
 Pulmonary oedema is common,
 hypotension,
 cardiogenic shock
 Chronic MR:
History
 initially asymptomatic.
 Fatigue (due to low forward CO),
 Exertional dyspnoea, orthopnoea,PND
 Systemic embolization (less common
than MS).
 Palpitations
 Right heart failure in later stages
 Symptoms and risk factors of
endocarditis, ischaemic heart disease.
Physical examination
 Irregularly irregular pulse in AF.
 Prominent ‘a’ waves in JVP in patients in
sinus rhythm.
 Large ‘v’ waves if there is associated
tricuspid regurgitation (TR).
 Forceful apex displaced laterally (LV
dilatation).
 Systolic thrill at apex.
Auscultation:
 Pansystolic murmur loudest at the apex
and radiating into the axilla.
 Soft first heart sound.
 Wide splitting of S2 due to premature
aortic valve closure.
 Prominent low-pitched S3 .
 A mid-diastolic flow murmur may follow
S3 even in the absence of MS.
INVESTIGATIONS
ECG:
 LA enlargement
 left ventricular hypertrophy (LVH)
 RA enlargement in pulmonary
hypertension
 AF common in chronic MR

CXR:
 Cardiomegaly
 LA and LV enlargement
 pulmonary congestion
 Calcified mitral annulus may be seen
 Transthoracic ECHO (TTE):
 Demonstrates MV anatomy and assess
the severity of MR
 Establish the underline etiology
 Assessment of the other valves
 Assessment of the cardiac chambers
specially the LV .
 Assess the LV function
 Estimate the pulmonary pressure
 Detect any vegetation or thrombi
TOE:
 Shows the anatomy in greater details
and allows accurate assessment of the
feasibility of valve repair.
 Should be performed pre- or intra-
operatively.
Cardiac catheterization:
 Not always required.
 Detection of co-existing valve lesions
and coronary artery disease.
MANAGEMENT

Medical management
 Asymptomatic patients with mild MR are
managed conservatively with serial
echocardiograms.
 Vasodilators in symptomatic patients to
increase forward CO and reduce
regurgitant volume.
 In AF: rate control and anticoagulation
(goal international normalized ratio
(INR) 2–3).
Surgical treatment
 Indications:

a) Patients with severe MR who are

symptomatic despite optimum medical
management.
b) Asymptomatic patients with severe MR
may need surgery if there is:
1) Worsening of LV function (EF 30–60 %
or dilated LV)
2) New AF
3) Pulmonary hypertension.
 Mitral valve repair is strongly preferred
to valve replacement.
 Likelihood of repair is highly dependent
on surgeon expertise.
 Repair minimizes risk of haemorrhage
and thromboembolism, reoperation, LV
dysfunction, and endocarditis.
 TRICUSPID
REGURGITATION
Causes:
 Functional or secondary TR (normal
leaflets, RV annular dilatation) is
common.
 Rheumatic heart disease,
 Endocarditis (particularly intravenous
(IV) drug abuse),
 Transvalvular pacing wires,
 Marfan’s syndrome,
 Ebstein anomaly,
 carcinoid.
 CLINICAL FEATURES
History
 Usually minimal.
 Peripheral swelling.
 Abdominal distention.
 Nausea, anorexia, abdominal pain
(tender, congested liver) are late signs.
Physical examination:
 Cachexia/wasting,
 Jaundice,
 Oedema,
 AF common,
 Elevated JVP with systolic CV waves,
 Tender pulsatile hepatomegaly.
 Panstolic murmur audible at left sternal
edge (LSE) ( increase in inspiration).
 Murmur loudest in TR secondary to
pulmonary hypertension.
 INVESTIGATIONS
 ECG — non-specific, may show evidence
of underlying condition.
 CXR — cardiomegaly in patients with
functional TR, pleural effusion.
 Echo —confirms diagnosis and show
the underlying etiology.
 TREATMENT
 In the absence of pulmonary hypertension, TR is
well tolerated and may not require specific
treatment
 Symptoms of RV failure respond to diuretics and
fluid/salt restriction.
 If co-existent MV disease is being operated on and
TR is mild, TR may improve postoperatively as PA
pressure falls.
 TV annuloplasty is indicated in patients undergoing
MVR with severe or moderate TR and annular
dilatation or pulmonary hypertension.
 TR secondary to valve pathology (Ebstein,
carcinoid) may require valve replacement,
preferably with a large bioprosthesis to minimize
the risk of thrombosis