TUTORIAL 2

Heart failure, Congenital heart

diseases, ITP
 01

HEART
FAILURE IN
CHILDREN
 CLINICAL FEATURES
~ ~ -

& xfailure to thrive,

Infants: poor feeding,
In tachypnea, and diaphoresis with feeding.
C =
Older children: shortness of breath, easy fatigability, and
-
edema.

=
The physical examination findings depend on whether pulmonary &
venous congestion,
-- O
systemic venous congestion, or both are present. Tachycardia, a gallop rhythm, and
-
~

thready pulses may be present with either cause.

-
- L
Left-sided failure : Tachypnea, orthopnea, wheezing, and pulmonary edema.
>VP)
: Hepatomegaly, edema, and distended neck veins. c+
Right-sided failure& -
- - -

Tachy ,
gallop rhythm

Marcdante, K. J., Kliegman, R., & Nelson, W. E. (2015). Nelson essentials of pediatrics. Elsevier/Saunders.
 I ETIOLOGY OF HEART FAILURE
Oh is
neardoveraand
❏
00
Cmetabolic needs (inadequate oxygen delivery). 3
with
G
Heart failure occurs when the heart is unable to pump blood at a rate commensurate

❏
-
It may be due to a change in myocardial contractility that results in low cardiac
-
output or to abnormal loading conditions being placed on the myocardium.

~ The abnormal loading conditions may beC

-

❏ -
afterload (pressure overload: aortic stenosis,

-
pulmonary stenosis, or coarctation of the aorta) or preload
-
(volume overload: ventricular
-
septal defect [VSD], patent ductus arteriosus [PDA],
-

or -
valvular insufficiency).
❏ Volume overload is the most common cause of heart failure in children.

Marcdante, K. J., Kliegman, R., & Nelson, W. E. (2015). Nelson essentials of pediatrics. Elsevier/Saunders.
 ETIOLOGY OF HEART FAILURE
- -

>
~

Marcdante, K. J., Kliegman, R., & Nelson, W. E. (2015). Nelson essentials of pediatrics. Elsevier/Saunders.
 MANAGEMENT OF HEART FAILURE
-tr y
,

1. Initial treatment: Improve myocardial function and optimizing preload and afterload

- (Diuretics, inotropic support, and, often, afterload reduction are employed)

&
T
-S
2. Long-term therapy usually consists of diuretics followed by afterload reduction.
3. Long-term therapy with B-blockers also may be beneficial, although this remains
somewhat controversial in pediatric patients.
4.
G
Spironolactone is usually added to the medical regimen because of its effect on cardiac
remodeling.

dinelloenonductor
Marcdante, K. J., Kliegman, R., & Nelson, W. E. (2015). Nelson essentials of pediatrics. Elsevier/Saunders.
 MANAGEMENT OF HEART FAILURE

Marcdante, K. J., Kliegman, R., & Nelson, W. E. (2015). Nelson essentials of pediatrics. Elsevier/Saunders.
 MANAGEMENT OF HEART FAILURE

Marcdante, K. J., Kliegman, R., & Nelson, W. E. (2015). Nelson essentials of pediatrics. Elsevier/Saunders.
 LO2: Recall the types and clinical features of
innocent murmurs.
 inner
lite

80
·
10
-

6
month'
syst
-
C >
Innocent murmurs
-

first 13
- C
Occur in at least 80% of normal infants and children.
- Heard most often during the-
first 6 months of life, from 3-6 years of age, and in early adolescence.
-

- Characteristic findings:
1. quality of sound ~

2. lack of significant radiation w ~

3. significant alteration in intensity with positional changes
-

- Cardiovascular history and examination are otherwise normal.

Types of innocent murmurs -

still murmur

8t[ -
St
venous hum

carotid buit

Od -
&
7

>
-
or
carotid

& part
-

or noru to

Aem
- -

norum
-

ne
LO3: Ventricular
Septal Defect
 Pathophysiology of VSD
● Is a congenital heart defect characterized by an abnormal opening in the
ventricular septum which allows blood to flow from left ventricle to right
ventricle.
● Accounts for 25% of all congenital heart disease
● Membranous VSDs are the most common of all VSD

Hemodynamics:
● Small VSDs are often asymptomatic and cause minimal shunting
● Large VSDs are not symptomatic at birth because the pulmonary vascular
resistance is normally elevated at this time. As the pulmonary vascular
resistance decreases over the first 6-8 weeks of life, can lead to significant left
to right shunting resulting in increased pulmonary blood flow
Clinical features of VSD
all
Symptoms
Small VSD: Often asymptomatic
Large VSD
atomf
Hef
● Shortness of breath ~ ~
-

--
● Tachypnoea, tachycardia & enlarged liver from heart
failure 7
-

● Poor feeding
- ~
● Failure to thrive ~ crJB
Physical examination
-
-

Y
Small VSD: Characterized by loud, harsh pansystolic murmur
loud
,

heard best at lower left sternal border warn stone

& mea
Large VSD: -Murmur may be softer due to equalization of
- P arc
pressure between the ventricles.
-
Loud P2 can be heard.
- -

of
murmur soft dre to
equalization
pressure
 Diagnosis
GCHEST X-RAY
● Small VSD:
- normal; minimal cardiomegaly and borderline increase in pulmonary vasculature
● Large VSD:
(7)
~- gross cardiomegaly with prominence of both ventricles, left atrium and pulmonary
artery &
C
~ - pulmonary vascular marking increase Ventricht reff atrium ,

C -
~ - pulmonary edema
-

and pulm anty

m
Ap v
-

ELECTROCARDIOGRAM
.

oedema
● Small VSD: normal ; left ventricular hypertrophy prim
-

● Large VSD:
- Biventricular hypertrophy by 2 months of age
- P wave notched or peaked
● 2D ECHOCARDIOGRAM ( determine position and size of VSD; shunt
size,haemodynamic effect and pulmonary hypertension due to high blood flow in
large VSD )
● PULSED DOPPLER ( show whether VSD is pressure restrictive by calculating
pressure gradient)
ECHO of small VSD
 SD
inceae pulmand
Complications flow
~ 1.
v
2.
-
Congestive heart failure - Large VSDs can lead to left-to-right shunting, resulting in volume
overload of the left heart, leading to congestive heart failure if left untreated.
Pulmonary Arterial Hypertension - Chronic left-to-right shunting can cause increased pulmonary
blood flow
C &leading to pulmonary hypertension. Over time, this can result in irreversible pulmonary
-

vascular disease (Eisenmenger syndrome) where the shunt reverses (right-to-left), causing
cyanosis.
~ 3. ~
Infective Endocarditis - Children with VSD are at an increased risk of IE, particularly if the defect is
deted associated with
4.
associated with turbulent blood flow . -

turbulent
Aortic Regurgitation - VSDs, particularly those located near the aortic valve, can lead to aortic por
valve prolapse and subsequent aortic regurgitation due to the altered haemodynamics.
& 5. ~ Arrhythmias - Some patients may develop arrhythmias, particularly if there is enlargement of the
heart chambers or fibrosis around the defect -

wFailure
-

-
6. to thrive - Due to increased metabolic demands and poor feeding associated with heart
j -
failure, some infants with large
-
VSDs may not grow adequately -
Atrial septal
defect
Pathophysiology of atrial
septal defect
%
5-1
● Is a congenital heart defect characterized by an abnormal opening in the atrial septum which
allows blood to flow from left atrium to right atrium.left to right shunt
O
● Accounts for 5-10% of all congenital heart disease.
● Ostium secundum ( hole in the region of foramen ovale ) - most common type.
● Primum ASD / Partial ASD / Ostium Primum :-
● Defect of the AV septum
-G
● Located near the endocardial cushions
● Characterised by :
1. Interatrial communication between bottom end of atrial septum and AV valves.
2. Tricuspid valve tends to leak (Regurgitant valve)
● Common CHD in Down's Syndrome
 Clinical features
● Depends on: ● Size of defect ● Relative compliance of both ventricles

1)Prominent right ventricular impulse : At left lower sternal border (LLSB) - can be palpated

2) ejection systolic murmur : Best heard at upper left sternal edge (right ventricular outflow
tract) Due to increased flow across the pulmonary valve because of left-to-right shunt
3) Fixed split S2 : Due to overload of the right ventricle with prolonged ejection into the
pulmonary circuit. Pulmonary valve closing later than aortic valve

● Mostly asymptomatic

● Some may have symptoms :-

F need
1) Failure to thrive
2) Frequent respiratory infection
3)Fatigue during feeding or activity
 Diagnosis
Physical Signs:
&
An ejection systolic murmur is best heard at left sternal edge - due to increased flow
across the pulmonary valve because of the left to right shunt.
=
A fixed and widely split second heart sound ( often difficult to hear )- due to the right
ventricular stroke volume being equal in both inspiration and expiration.

Chest Radiograph:
Shows cardiomegaly, enlarged pulmonary arteries and increased pulmonary vascular
markings.
 Diagnosis
ECG: w
May show right axis deviation and right ventricular enlargement.

Secundum ASD - partial bundle branch block is common, right axis deviation due to right
ventricular enlargement.

Partial AVSD - a superior QRS axis. Due to the defect of the middle part of the heart
where atrioventricular node is. The displaced node then conducts to the ventricles
superiorly, giving the abnormal axis.

Echocardiogram:
Would portray the anatomy and is the mainstay of
diagnostic investigations.
 Complications
● Eisenmenger’s syndrome:
In severe, long-standing cases with significant left-to-right shunt, pulmonary
hypertension can reverse the shunt direction, leading to cyanosis (low oxygen levels in
the blood) .

● Right Heart Enlargement and Heart Failure:

Increased blood flow through the ASD can enlarge the right atrium and ventricle,
eventually leading to right-sided heart failure.

● Pulmonary Hypertension:
Chronic increased blood flow to the lungs can lead to high blood pressure in the
pulmonary arteries, which can worsen over time and become irreversible.

● Paradoxical Embolism and Stroke:

An ASD allows blood clots to pass from the right to the left atrium, potentially
traveling to the brain and causing a stroke (paradoxical embolism).
Persistent
Ductus
Arteriosus
Nelson Essentials of Paediatrics 8th Edition Page 546,547
Paediatric Protocols 4th Edition Page 162, 163
 Etiology
- Ductus arteriosus allows blood to flow from the
pulmonary artery to the aorta during fetal life

- Failure of the normal closure of this vessel results in

PDA

- Left-to-right shunting of blood and increased

pulmonary blood flow

- PDA represents approximately 5-10% of CHD

(excluding premature infants)

- Risk factors = lack of antenatal steroids and

Respiratory distress syndrome
 Physical examination:
Clinical
1. Widened pulse pressure
Manifestations - The result of runoff of blood into the pulmonary
circulation during diastole

Symptoms depend on the amount of 2. Continuous, machine-like murmur

pulmonary blood flow - At the left infraclavicular area, radiating along the
pulmonary artery
The magnitude of shunt depends on : - Well heard over the life side of the back
- the size of the PDA (diameter,
length, tortuosity) 3. Splitting of S2
- the pulmonary vascular - Increased pulmonary blood flow
resistance
4. Thrill may be palpable
Small PDA:
- Asymptomatic In larger shunts with increased flow across the mitral valve=
5. Mid-diastolic murmur at the apex
Moderate to Large PDA:
- Symptoms of heart failure as the
6. Hyperdynamic precordium
pulmonary vascular resistance
- A forceful and hyperdynamic impulse can be palpated
decreases
during physical examination
Treatment Supportive therapy:
1. Adequate positive end-expiratory pressure
(PEEP) to reduce left-to-right flow and improve
- In full term infants: Spontaneous systemic circulation
closure of a PDA after a few 2. Maintenance of hematocrit at 35-40%
weeks of age is uncommon 3. Fluid restriction
4. Avoid loop diuretics (Furosemide), use thiazide
Moderate & Large PDAS diuretics (hydrochlorothiazide) instead
- Managed initially with diuretics
- Eventually require closure : Pharmacologic therapy
1. Coil embolization 1. Indicated for preterm infants
2. PDA closure device 2. Indomethacin, ibuprofen, paracetamol:
pharmacologic closure of PDA, inhibit
Prostaglandin synthetase activity, decrease
prostaglandin

Surgical ligation
1. In persistent symptomatic PDA & failed /
contraindicated pharmacological treatment
 Imaging Studies
1. Chest X-ray:

● Cardiomegaly (due to left atrial and ventricular enlargement)

● Increased pulmonary vascular markings (especially in the perihilar region)

(indicates pulmonary overcirculation)

2. Echocardiography (Gold Standard):

● 2D Echo with Doppler shows:

○ Direct visualization of the PDA

○ Left-to-right shunting via color Doppler

○ Volume overload of LA and LV

● PDA size and flow velocity help guide management

3. Cardiac MRI/CT Angiography (less common in routine pediatrics):

● Used in older children or if echo is inconclusive

● Helps in anatomical visualization before surgery/device closure

 Complication
If untreated, PDA may lead to:

1. Congestive Heart Failure – from chronic volume overload

2. Pulmonary Hypertension – due to continuous high flow

3. Eisenmenger Syndrome – reversal of shunt to right-to-left,

causing cyanosis

4. Endocarditis or Endarteritis – risk of infection at the ductus

5. Failure to thrive – due to chronic energy expenditure

6. Arrhythmias – from cardiac enlargement

Tetralogy of
Fallot
 Pathophysiology
left Sunut
Rt to
-
● Ventricular Septal Defect- Allows mixing of
oxygenated
- blood with deoxygenated blood
● Pulmonary stenosis-Right ventricular outflow
obstruction
● Right ventricular hypertrophy- Consequences
of pulmonary stenosis
● Overriding Aorta- Abnormal positioning of
aorta which receive mixed blood flow
 Pathophysiology consequences
Haemodynamic system
- Right to left shunting: Depending on variety, initially acyanotic (pink tetralogy) then
when there is infundibular muscle hypertrophy/valve/supravalve stenosis, increase
the right ventricle resistance leading to cyanosis.

Systemic
- Hypoxemia and cyanosis: mixing of blood and less blood being oxygenated
- Polycythemia: physiological increase in RBC to compensate low oxygen
- Tet-spells: sudden worsening of cyanosis and hypoxemia after certain activities

Chronic
- cardiac arrhythmias
- Right ventricular dysfunction
 Clinical Features

Symptoms
● Cyanosis (Right to left shunting)
● Tet spells
● Dyspnea and fatigue
● Failure to thrive
● Delayed developmental milestones

Physical Examination
● Clubbing
● Heart murmur- systolic ejection murmur
 Diagnosis
Chest X-RAY
- “boot-shaped” Heart
- Pulmonary oligaemia
- Cardiomegaly (Right ventricle,Right atrium)
- Diameter of ascending aorta is larger
- Small pulmonary conus,(concave)

Electrocardiogram (ECG)
- Right axis deviation
- Right ventricular hypertrophy
 Complications

● Heart Failure - due to volume overload

● Pulmonary hypertension
● Arrhythmias
● Delayed Growth and development - due to increase in metabolic demand and
poor feeding associated with heart failure

↑
 O
LO 4 Describe the clinical features of aortic and pulmonary 6
G stenosis
Clinical Features reada
C
Aortic Stenosis 1. Symptoms:
man 4

creain
1. Accounts for 5% of all congenital
heart disease. -
● Mild to moderate: No symptom
- ↑
● More severe
C stenosis:
-
Easy fatigability,
T -
-

2. Occurs at valvular, subvalvular and exertional chest pain and syncope

-
-

supravalvular levels. ● Critical aortic stenosis: Symptoms of

-
3. The stenosis occurs due to: heart failure critical >
-
HF

● Failure of development of 2. Murmur: Systolic ejectionC murmur at right

-

three leaflets
-

- second intercostal space,- radiates to neck.

● Failure of resorption of 3. With valvular stenosis:
-
● Systolic ejectionO
~
tissue around the valve click
● Presence ofO thrill at right upper sternal
-

border/ suprasternal notch

● Aortic component of A2 may be
decreased in intensity
Reference: Nelson Essentials of Pediatrics 7th Edition
 mi
Pulmonary Stenosis Clinical Features
1. Accounts for 10% of all congenital 1. Symptoms depend on severity of obstruction:
heart disease. ● Mild: Asymptomatic

a
2. Occurs at valvular, subvalvular and ● Moderate to severe: Exertional dyspnea
supravalvular levels. and easy fatigability
3. The stenosis occurs due to: O
● Newborn with severe cyanosis: More
● Failure of development of three symptomatic and cyanosis (due to
C
-
-

valve leaflets right-to-left atrial shunt)

-

● Insufficient resorption of 2. Murmur: Systolic ejection murmur at second

-
infundibular tissue left intercostal space, radiates to back.
-

● Insufficient canalization of 3. Thrill may be present.

peripheral pulmonary arteries Eo
4. Wide split of S2 with quiet pulmonary
component.
5. When severe, there will be impulse from lower
left sternal border due to right ventricular
hypertrophy.
Reference: Nelson Essentials of Pediatrics 7th Edition
 LO5: Pathophysiology of Hypercyanotic
spells
1. Hypercyanotic spells is a sudden profound cyanosis and hypoxia found in Tetralogy of Fallot
2. Triggered by event that slightly decrease in oxygen saturation or decrease in systemic vascular resistance
3. Precipitated by Tachycardia, Hypotension, Crying, Exertion of feeding and defecation
4. Caused by sudden increase of right to left shunt and fall of oxygen saturation
5. Pulmonary stenosis will leads to Right ventricular outflow obstruction
6. Increase Pulmonary vascular resistance and reduce Pulmonary blood flow
7. Right to left shunt through VSD increase as Pulmonary stenosis worsen and bypass the lungs
8. It will decrease systemic vascular resistance, reduce pressure of left ventricle
9. It will exacerbate right to left shunt across VSD due to higher pressure in right ventricle compared to left
ventricle
10. There is a mixing of oxygenated and deoxygenated blood
11. Rapid Increase PaCO2 but decrease PaO2, cause oxygen desaturation (Hypoxia) and cyanosis
12. Increase Hyperpnea (deep and rapid breathing)
13. Increase negative intrathoracic pressure and increase venous return to right heart
14. Cycle repeated as it will also increase right to left shunt across VSD

Reference: NHSGGC Paediatrics Guidelines in Scotland, Malaysia Paediatrics Protocols, MSD Manuals, National
Library of Medicine (NIH),
 Management of Hypercyanotic spells
1. Place the baby at knee-chest or squatting position
2. It will reduce systemic venous return and increase systemic vascular resistance
3. Administer 100% oxygen
4. IV/IM/subcutaneous Morphine 0.1-0.2mg/kg (reduce distress and hyperpnoea)

If fails
5. IV Propranolol 0.05-0.1mg/kg slow bolus over 10 minutes
(Alternative: IV Esmolol 0.5mg/kg slow bolus over 1 minutes, followed by 0.05mg/kg/min for 4 minutes.
Can be given as continuous IV infusion 0.01-0.02mg/kg/min)
6. Crystalloid or colloid solution 20ml/kg rapid IV push: Increase preload
7. IV bicarbonate 1-2mEq/kg for metabolic acidosis
8. Heavy sedation, Intubation and mechanical ventilation needed
Resistant case
1. IV Phenylephrine 0.01 to 0.02 mg/kg slow bolus OR
2. Noredrenaline infusion 0.1-0.5 mcg/kg/min
(Increase systemic vascular resistance, reduce right to left shunt)

1. Emergency Blalock Taussig shunt

(Oral Propranolol 0.2-1mg/kg/dose 8 to 12 hourly should be started after stabilisation while waiting for surgical intervention)

Reference: Malaysia Paediatrics Protocols

LO6: Clinical Features & Management of SVT
A tachyarrhythmia originating above the ventricles, characterised by a rapid heart rate and
narrow QRS complex. The tachycardia has an abrupt onset and termination.

Clinical Features
● Infants: Poor feeding, Irritability, Pallor, Lethargy, Signs of heart failure: Difficulty
breathing, Hepatomegaly (severe cases)
● Older children: Palpitations, Chest pain, Dizziness, Fatigue, Syncope (severe episodes)
● General signs
○ Rapid, regular heart rate (> 220 bpm in infants and > 180 bpm in children)
○ Narrow QRS complex on ECG
○ If prolonged/ severe, signs of poor perfusion: Cool extremities, Delayed capillary
refill time, Hypotension/ Cardiogenic shock

Reference: Nelson Textbook of Pediatrics, Malaysian Pediatric Protocol

 LO6: Clinical Features & Management of SVT
Acute Management
1. Non-Pharmacological Measures (Vagal Maneuvers)
a. Infants: Application of a bag of ice/ cold stimulus to the face
b. Older children: Valsalva Maneuver/ Blowing through a straw
2. Pharmacological Therapy
a. Adenosine
i. 1st line drug
ii. Administered as rapid IV bolus, followed by a saline flush
iii. Start with 0.1 mg/kg (max 6mg) and increase to 10.2 mg/kg (max 18mg) if necessary
b. Alternatives:
i. IV Propranolol 0.02 mg/kg test dose, then 0.1 mg/kg over 10 mins
ii. IV Amiodarone 25 mcg/kg/min for 4 hours then 5-15 mcg/kg/min until conversion
3. Electrical cardioversion
a. Indicated for hemodynamic instability/ failure of pharmacological therapy
b. Synchronized cardioversion starting at 0.5-1 J/kg

Reference: Nelson Textbook of Pediatrics, Malaysian Pediatric Protocol

LO6: Clinical Features & Management of SVT

Chronic Management
1. Pharmacological maintenance
a. Beta Blockers (Propranolol) or Calcium channel blockers (Verapamil)
b. Amiodarone for refractory cases
2. Definitive therapy
a. Radiofrequency Ablation (RFA): Indicated for recurrent/ refractory SVT

Follow up
1. Regular monitoring of cardiac function
2. Growth and development assessment
3. Counseling parents about recognising symptoms of recurrence

Reference: Nelson Textbook of Pediatrics, Malaysian Pediatric Protocol

LO7: Etiology of purpura
 Immune
Thrombocytopenic
Purpura
 Definition

● Presenting with isolated

thrombocytopenia (<100 x 10^9/L), in the
absence of an underlying cause
 Pathogenesis

● Autoimmune disorder characterized by autoantibody

mediated immunologic destruction of normal
platelets, in response to a unknown stimulus
 Clinical Manifestations
● Onset is usually abrupt
● Duration from onset of thrombocytopenia to
normalisation of platelet counts can be a few days to 6
months
● History of viral infection in the preceding 2-4 weeks
● Petechiae -> mucosal bleedings (gum bleeds, epistaxis,
gross haematuria) -> life-threatening bleeds (intracranial
haemorrhage)
 Diagnosis

● History
● Physical Examination: absence of hepatosplenomegaly ot
lymphadenopathy
● Blood counts: isolated thrombocytopenia, with normal
haemoglobin and white cell count
● Peripheral Blood Film: normal apart from reduced, larger
platelets, no abnormal cells
 Management

● Remit spontaneously
● Platelet count <20 x 10^9/L at diagnosis
● 3 weeks most achieve platelet count > 50 x 10^9/L
without treatment
● Observation and monitoring of platelet count, without
specific treatment:
- Platelet count > 20 x 10^9/L without bleeding
- Platelet count > 30 x 10^9/L with only cutaneous purpura
 Management
● Consider Hospitalization
- Severe life-threatening bleeding regardless of platelet
count
- Platelet count < 20 x 10^9/L with evidence of bleeding
- Platelet count < 20 x10^9/L without bleeding but
inaccessible to health care
- Lack of confidence in home care.
● Choice of treatment
- Oral prednisolone 2mg/kg/day for 14 days then taper off
over 5 days
- Oral prednisolone 4mg/kg/day for 3-4 days
- IVIG 0.8g/kg/dose
 Emergency Management

● IV methylprednisolone 30mg/kg.day for 3 days

● IVIG 0.8g -1g/kg as a single dose
●