Pediatric Cardiology

Cardiac Evaluation:
History:
Children dont present with the typical features of CHF as in adults
(orthopnea and nocturnal dyspnea are RARE findings in children)
- Age is very important when assessing a child:
Infants with heart failure present with:
- Feeding difficulties
- Poor wt. gain / wt. loss
- Sweating while feeding
- Tachypnea
- Easily fatigued
Older children present with signs and symptoms more like those seen
in adults:
- Shortness of breath
- Dyspnea on exertion
Physical Examination:
- Assess HR and RR according to age
- Height and weight assessment
- ALWAYS get upper and lower extremity BP and pulses regardless of
age ( adult form coarctation)
- Hepatosplenomegaly RHF
- Rales on auscultation suggestive of pulmonary edema and LHF
- Cyanosis and clubbing from chronic prolonged hypoxia

Note: jugular vein distension is hard to find in infants because they

have short thick necks (not seen in infants)
Heart murmur gradation:

Grade Quality
1 Soft, difficult to hear (needs quite condition to be heard)
2 Easily heard ( could be pathological)
3 Louder but no thrill
4 Associated with thrill
5 Thrill; audible with edge of the stethoscope
6 Thrill; audible with stethoscope off chest
Note:
Any murmur with a grade of 3-6 is ALAWYS pathological and CANT be
innocent.
Diagnostic tests: ( age dependent)
Chest radiographs:
Evaluate:
- Heart size
- Lung fields
- Ribs for notching
- Position of great vessels
Electrocardiogram ECG
Echocardiography- definitive diagnosis: best test for diagnosing all CHD
Others:
 Pediatric Cardiology
-
MRI - Catheterization - Angiography - Exercise testing ( stress
testing inn older children)
Innocent murmurs:
- Also known as functional, normal, insignificant or flow murmurs.
These result from (turbulent) blood flow through a normal heart,
vessels and valves.
They are easily heard in children because children have a higher
HR, higher flow rate and thinner chest walls.
- More than 30% of children ages 3-7 years may have an innocent
murmur heard at some times in their lives.
- Presentation:
Usually heard on routine PE; also easily heard in conditions
where HR is increased as in fever, anxiety or infection.
NEVER diastolic
NEVER greater than grade 2/6
Soft, vibratory and musical at the left lower mediastinum
- No diagnostic tests done and you just need to reassure the parents.

Murmur Age Location Characteristics

Stills Ages 2-7 years Mid-left sternal Grade 1-2, systolic
murmur border vibratory=buzzing=twanging
Loudest when supine
Louder with exercise
Pulmonic Any age Upper left sternal Grade 1-2, peaks early in systole
systolic border Blowing, high pitched
murmur Loudest when supine
Louder with exercise
(systolic
ejection
murmur)
Venous hum Any age but Neck and below the Continuous murmur
especially clavicle Heard only when sitting or
school age standing
Disappears if supine; changes
with jugular vein compression or
with neck flexion or extension

Fetal circulation ( quick review):

- Oxygenated blood from the placenta is carried through the
umbilical VEIN and eventually drains into IVC, then the right side of
heart.
(Remember a vein is any vessel that carries blood TO the heart and
an artery is any vessel that carries blood AWAY from the heart
REGARDLESS of oxygen content)
 Pediatric Cardiology
- Most of the blood crosses the foramen ovale into the left atrium
Left ventricle systemic circulation
- Some of the blood gets into the right ventricle; some of that blood
is pumped into the aorta via the ductus arteriosus. (mixing takes
place)
- When the baby is delivered, the cord is clamped and the baby takes
starts to breathe which reduces the pulmonary vascular resistance
allowing the blood from the right ventricle to be pumped into the
lungs for oxygenation.
- Oxygen tension closes the ductus arteriosus
- Systemic vascular resistance starts to increase (normal circulation).
Congestive heart failure:
Definition: inadequate oxygen delivery by the myocardium to meet the
metabolic demands of the body.
Pathophysiology: the signs and symptoms of CHF are due to
compensatory mechanisms that further increase the demands on an
already compromised myocardium.
1. Hypoperfusion of end organs causes the heart to pump more for
forcefully and at a higher rate (increased contractility and HR)to
increase the cardiac output.
2. Hypoperfusion of the kidneys causes them to retain salt and water
to increase blood volume. (RAAS)
3. Catecholamines released by SANS increase the HR and contractility.
Etiology:
1. Congenital heart disease CHD:
- Due to increased pulmonary blood flow as in large VSD/ large PDA/
transposition of the great arteries TGA/truncus arteriosus/ total
anomalous pulmonary venous connection TAPVC
- Due to obstructive lesions as in severe aortic, pulmonary, mitral
valve stenosis / caorctation of aorta/hypoplastic left heart/
interrupted aortic arch.
- Other causes: AV malformations / Mitral, tricuspid regurgitation
( overload the heart)
2. Acquired heart disease:
- Infections ; viral myocarditis (common in older children and
adolescents)
Endocarditis/ pericarditis.
- Metabolic (hyperthyroidism)
- Medications ( doxorubicin a chemotherapeutic agent)
- Cardiomyopathies
- Ischemic heart disease
- Dysrhythmias
3. Miscellaneous causes :
- Severe anemia ( high output CHF)
- Rapid IV infusion eps. In preterm
 Pediatric Cardiology
- Chronic hypoxia due to airway obstruction ( right sided heart
failure)
Enlarged tonsils or adenoids/ CF
Clinical features:
1. Pulmonary congestion- tachypnea, cough, wheezing and rales. Pulmonary
edema on CXR
2. Systemic venous congestion: hepatomegaly, peripheral edema
3. Impaired cordial performance- tachycardia, sweating, pale or ashen (very
pale) skin, diminished urine output. Enlarged cardiac silhouette on CXR.
4. Others: FTT/ poor feeding ( common in newborns) and exercise intolerance
( common in older children and adolescents)
5. Cyanosis and shock are late manifestations.
Management:
1. Medical management: improves myocardial function and relieve
pulmonary and systemic congestion.
Cardiac glycosides (digoxin); increase the efficacy of myocardial
contractility and relieve tachycardia.
Loop diuretics ( furosemide, ethacrynic acid); maximize sodium loss
to reduce the intravascular volume ( reduce afterload on the heart
diminish ventricular dilation)
IV inotropes (dobutamine, dopamine) for severe CHF
Amrinone, milrinone (phosphodiesterase inhibitors) improve
contractility and reduce the afterload.
2. Interventional catheterization procedures (e.g., balloon valvuloplasty for
critical aortic and pulmonary stenosis)
3. Surgical repair: the definitive treatment of CHF secondary to CHD.

Congenital heart disease:

Introduction:
- Mostly diagnosis is usually made by 1 month (some can be picked
up by a fetal echo)
- Murmurs may not be heard in early life because of increased
pulmonary vascular resistance ( from fetal to neonatal transition
physiology); it takes about 4-6 months for the pulmonary vascular
resistance to be normal.

- Etiology:
Most are unknown
Associated with teratogens such as alcohol ( fetal alcohol
syndrome) and rubella
Genetic predisposition : trisomies ( trisomy 21), Marfan,
Noonan, DiGeorge syndromes
- Classification:
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Shunting
Regurgitant Stenotic Right to left Left to right Mixing
Mitral valve Aortic stenosis TOF most PDA Truncus
prolapse common
cyanotic CHD
Pulmonic / Pulmonic Ebstein VSD most Total
Aortic stenosis anomaly common CHD anomalous
insufficiency (isolated or pulmonary
with other CHD venous return
defetcs)
Mitral/ tricuspid Coarctation Tricuspid ASD, Hypoplastic left
insufficiency atresia Endocardial heart,
cushion defect transposition
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Acyanotic congenital heart disease / Left to Right Shunts:
Atrial septal defect ASD:
Classification:

Ostium primum Ostium secundum Sinus venosus

Locatio Lower atrial septum Middle part of atrial septum High in the septum
n in the region of the fossa near the junction of
ovalis the right atrium and
SVC
Notes -common in Down The MOST COMMON type of The right pulmonary
Syndrome ASD veins drain
-anterior mitral valve anomalously into the
leaflet cleft or deviation right atrium or SVC
may be present casing instead of into the
mitral valve regurgitation. left atrium
Pathophysiology:
Blood flows across the ASD from higher resistance to lower resistance;
from the left atrium to the right atrium. This increases the size of the
right atrium and right ventricle and increases pulmonary blood flow.
Clinical features
- Few symptoms early in life if any because of the structure of low
flow
The atria re not high pressure nor high flow chambers and therefore
there isnt a lot of shunting of blood from L to R
In older children with large defects, there could be exercise
intolerance (often 3rd decade of life)
o If the defect does not close spontaneously there will be
persistent shunting from left to right that could end up in
eisenmenger complex BUT AFTER REALLY LONG PERIOD OF
TIME (less flow than in VSD)
- Ostium primum ASD with MV regurgitation is an exception; pt. could
develop CHF
physical examination findings:
1. Systolic ejection murmur from excessive pulmonary blood flow best
heard at mid and upper left sternal borders. (NOT the sound of the
blood rushing through the defect)
2. With hemodynamically significant lesions also a low-pitched mid-
diastoilc filling rumble representing excessive blood flow through
the tricuspid valve best heard at the lower sternal border.
3. Wide fixed splitting of the second heart sound S2. (in ASD the right
side of the heart is always receiving excess blood requiring more
time to empty and therefore longer time for the pulmonic valve to
close) due to increased pulmonary blood flow, the normal
physiologic variation in timing of aortic and pulmonic valve closure
with respiration is absent.
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4. Increased right ventricular impulse due to right ventricular
overload.

Diagnosis:
- Echocardiogram is the best definitive test
- CXR:
Right atrial and right ventricular enlargement
Increased pulmonary vascular markings and pulmonary
edema

- ECG:
Right axis deviation RAD
Right ventricular hypertrophy RVH
Right atrial enlargement RAE
Complications:
- Atrial dysrhythmias
- Paradoxical embolism
- Right ventricular heart failure
- Pulmonary hypertension
(Low flow lesion so doesnt require endocarditis prophylaxis)
Treatment:
- Most in term infants it closes spontaneously; symptoms often dont
appear until third decade
- If symptomatic:
Closure by open heart surgery or
Transcatheter device closure (interventional catheterization
procedures)

Ventricular septal defect VSD:

The most common CHD
Classification based on location:
- Most are membranous
- Muscular (trabecular)
- Inlet
- Outlet ( supracrista)
Pathophysiology:
Blow flows through the VSD from the left ventricle to right ventricle due to
decreased pulmonary resistance after birth.
Clinical features:
Depend on the degree of L to R shunt which in turn depends on the
pulmonary vascular resistance and the size of the VSD.
- Small VSD:
May close spontaneously
Little or no shunting
PE:
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1. Grade 4-harsh high pitched (from high flow velocity
across a very restrictive small defect) HOLOSYSTOLIC
murmur at the lower left sternal border + thrill
S2 widely split
Note: as the size of the VSD decreases, the intensity of
the murmur increases
- Moderate VSD:
Large shunt resulting in CHF
Holosystolic murmur at lower left sternal border (intensity
depends on the size of shunt)
Low-pitched diastolic rumble across the mitral valve best heard
at the apex (mitral filling rumble due to excess blood from the
lungs passing through the mitral valve)
This occurs if there is excessive blood flow across the VSD
2:1 pulmonary to systemic flow; blood flow to the lungs is
twice as much as blood flow to the systemic circulation
- Large VSD:
CHF (dyspnea, feeding difficulties, poor growth, sweating),
higher incidence of pulmonary infections from excessive
blood flow
Systolic murmur is shorter lower in pitch (less turbulence
across large VSD)
Mitral diastolic filling rumble heard best at the apex.
Diagnosis:
- Echocardiogram is definitive
- CXR: 1st test to do
Small VSD: normal CXR
Moderate-large: cardiomegaly and increased pulmonary
vascular markings/ pulmonary edema
If pulmonary vascular resistance is elevated there will be
decreased pulmonary vascular markings
- ECG:
Small: normal or mild LVH
Moderate: LVH
If pulmonary hypertension is present: RVH
Eventually with time there could be biventricular hypertrophy
Complications:
- Large defects lead to CHF ,FTT
- Endocarditis (any high flow lesion is at risk of endocarditis; needs
prophylaxis for dental procedure)
- Pulmonary hypertension:
o With time the pulmonary vessels hypertrophy (medial layer)
in response to increased pulmonary blood flow. This
hypertrophy increases pulmonary vascular resistance.
o The right ventricular impulse is increased
o Single loud S2
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o The increase in PVR decreases the amount of shunt
( decrease pulmonary blood flow and less blood going to left
atrium, CHF symptoms may diminish)
This causes the mitral filling rumble to disappear
o If PVR becomes high enough to exceed the systemic vascular
resistance, it can reverse the shunt from left to right shunt to
become right to left shunt Eisenmenger syndrome
characterized by cyanosis.
This requires heart-lung transplant

Treatment:
- Small MUSCULAR VSD are more likely to close in the first two years
of life than MEMBRANOUS ( as the child grows, the heart grows and
the muscles grow closing the defect)
- Medical management of CHF for symptomatic children with
moderate to large VSD as they are less likely to close. ( control CHF
and prevent pulmonary vascular disease)
- Surgical closure is indicated in the following circumstances:
Heart failure refractory to medical management
Large VSDs with pulmonary HTN are usually surgically closed
at 3-6 months of age.
Small to moderate VSDs are usually surgically closed
between 2 and 6 years of age.
You treat CHF medically and wait till the child is a bit older so
that the surgery is easier to perform

Endocardial cushion defect:

Patients with trisomy 21 are at a higher risk for endocardial cushion defects.
Pathophysiology:
The endocardial cushion is where the valve and septum originate so
a defect results in:
- ASD + VSD + atrioventricular valve insufficiency esp. mitral valve;
increases volume load on the ventricle resulting in early heart
failure, infections, minimal cyanosis, hepatomegaly and FTT
- Left to right shunts at both atrial and ventricular levels ; some right
to left shunting with desaturation (mild, intermittent cyanosis); it is
a left to right shunt but it can quickly become R to L shunt; there is
so much blood shunting occurring here Eisenmenger syndrome
Physical examination:
Heart failure early in infancy (hepatomegaly, FTT); high degree of
shunting
Eisenmenger occurs earlier
Moderate-to-severe increase in heart size with hyperdynamic
precordium (precordial bulge and lift)
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Widely fixed split S2 (like an isolated ASD)
Loud Pulmonary systolic ejection murmur with a thrill at the mid and
upper left sternal border
Low-pitched diastolic rumble at the apex/ bilateral diastolic flow
murmurs from insufficinecy
Apical harsh holosystolic murmur radiating to the axilla due to MV
insufficiency
Diagnostic test:
- Echocardiogram is the gold standard
- CXR:
Significant cardiomegaly
Increased pulmonary blood flow and edema
- ECG:
Biventricular hypertrophy
RAE
Superior QRS axis
Complications:
- Without surgery- death from CHF
- With surgery- arrhythmias, congenital heart block
Treatment:
Surgery must be performed in infancy; surgery is more difficult with heart
failure and pulmonary HTN (increased pulmonary artery pressure by 6-12
months of age)

Patent ductus arteriosus:

Stenotic lesions:
Coarctation of the aorta:
Definition:
Narrowing of the aortic arch just below the origin of the left subclavian
artery and typically at or just proximal to the ductus arteriosus;
juxtaducxtal coarctation (90%)
Pathophysiology:
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The narrowed segment obstructs or diminishes flow from proximal to
distal aorta.
Adult versus childhood
- Discrete juxtaductal
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