3.
1
Pediatric Cardiology (Summary Table)
Ruby Ann Punongbayan, MD
CONDITION
ATRIAL SEPTAL DEFECT (ASD)
Isolated anomaly in 5-10% of all
CHDs
VENTRICULAR SEPTAL DEFECT
(VSD)
Most common form of CHD (15-
20%)
Perimembranous defects: Most
common (70%)
PATENT DUCTUS ARTERIOSUS
(PDA)
Occurs in 5-10% of all CHDs
(common in premature infants)
MITRAL VALVE PROLAPSE (MVP)
Occurs in older children &
adolescents
TRANSPOSITION OF THE GREAT
ARTERIES (TOGA)
Occurs in about 5% of all CHDs
(M>F)
Most common cyanotic heart lesion
among newborns
TETRALOGY OF FALLOT (TOF)
Occurs in 10% of all CHDs
Most common cyanotic heart defect
beyond infancy
PROVe
CLINICAL MANIFESTATIONS
CONGENITAL HEART LESIONS
ACYANOTIC CONGENITAL HEART LESION
Systolic ejection murmur at the 2nd
LICS
Widely split S2
Systolic regurgitant murmur at the LLSB ECG: LVH in moderate shunts;
Loud and single S2 in pulmonary
hypertension
Continuous machinery murmur at
the (L) infraclavicular area or ULSB
Bounding peripheral pulses with wide
pulse pressure
Midsystolic click with or without a
late systolic murmur best heard at the
apex
Click & murmur may be brought out by held
expiration, (L) decubitus position, sitting,
standing, leaning forward; may disappear on
inspiration
CYANOTIC CONGENITAL HEART LESION
Single & loud S2; no heart murmur is
heard in infants with an intact
ventricular septum
Systolic ejection murmur at the mid and
2nd ULSB heard at the 2nd LICS
Loud and single S2
(couer en sabot or boot-shaped
DIAGNOSTIC FINDINGS
Right sided enlargement
ECG: RVH & RBBB in
CXR: RAE & RVE & prominent PA
Left sided enlargement
Biventricular hypertrophy if with
Eisenmenger syndrome
Good dental hygiene and antibiotic prophylaxis
(Combined ventricular hypertrophy) CVH
in large defects
CXR: LAE, LVE, increased pulmonary
vascular markings, PVOD, enlarged MPA
(main pulmonary artery)
Left sided enlargement
Nonsurgical closure: Stainless coils
ECG: LVH (small to moderate shunts)
CXR: Shows prominent pulmonary
markings/vessels and enlarged left
ventricle
ECG: RVH, LVH, CVH
CXR: Egg-shaped cardiac silhouette
with a narrow, superior mediastinum
ECG: RAD, RVH
CXR: Small heart size, decreased
pulmonary vascular markings, concave
main PA with an upturned apex
heart)
Sept. 8, 2014
MANAGEMENT
Nonsurgical closure: Occlusion devices
Surgery for Qp/Qs >1.5:1
Delayed until 3-4 years old
Treatment of CHF
against IE
Surgery if no improvement within the 1st 6 months
of life
Surgical closure: PVOD is a contraindication;
done between 6 months & 2 years of age or when
diagnosis is made in an older child
Asymptomatic patients do not need treatment
or restriction of activity
Antibiotic prophylaxis against SBE
Chest pain may be treated with Propranolol (CI in
asthmatics)
Definitive repair: switch right- & left-sided blood
at 3 levels: atrial (Senning or Mustard),
ventricular (Rastelli), great artery level (Jatene);
Rashkind
Knee-chest position, Morphine sulphate, NaHCO3,
O2, Vasoconstrictors, Propranolol, Ketamine
Maintain good dental hygiene & antibiotic
prophylaxis
o Surgery: Blalock-Taussig (>3 months old) and
Gore-Tex shunt (< 3 months old)
o Total repair of the defect (patch closure of VSD &
widening of the RVOT by resection)

TRICUSPID ATRESIA
Components:
1. Atretic (Missing) Tricuspid Valve
2. Hypoplastic Right Ventricle
3. Ventricular Septal Defect
4. Atrial Septal Defect
5. Pulmonic Stenosis
TRUNCUS ARTERIOSUS
1.2-2.5% of all congenital heart
disease
Associated with Di-George Syndrome
linked to deletion of chromosome
22q11
Components:
1. Pulmonary arteries arise from
aorta
2. Truncal valve (quadracuspid
stenotic and/or insufficient)
3. Large VSD (always present)
TOTAL ANOMALOUS PULMONARY
VENOUS RETURN (TAPVR)
PULMONARY STENOSIS (PS)
Associated with congenital rubella,
Noonan and William syndrome
Hemodynamics
RV pressure overload
RV pressure hypertrophy RV
failure
RV pressures maybe > systemic
pressure
AORTIC STENOSIS (AS)
Hemodynamics
Pressure hypertrophy of the LV and
LA with obstruction to flow from LV
COARCTATION OF THE AORTA
(COA)
7% of all CHD
Aortic valve: Bicuspid in more than
70%
Systolic regurgitant murmur at LLSB;
systolic ejection murmur at ULSB
Single S2
Systolic regurgitant murmur at LLSB
Minimal cyanosis in neonates; older
children: heart failure
Without obstruction: Mild cyanosis,
tachypnea, feeding difficulties
With obstruction: Rapid progression to
dyspnea, cardiac respiratory failure
Systolic murmur at LSB; murmurs may not
be present
OBSTRUCTIVE LESIONS
Asymptomatic to severe
Systolic murmur LUSB to back; soft P2
there is radiation to back
Usually asymptomatic only detected when
there is already left ventricular failure
Harsh systolic ejection murmur at RUSB
and radiates to the neck and left
midsternal border
Most children asymptomatic but can have
CHF if severe
Classic signs of coarctation are diminution
or absence of femoral pulses
Higher BP in the upper extremities as
Pediatric Cardiology (Summary Table)
Hypoplastic RV
CXR: Cardiomegaly (right, left, or
combined ventricular hypertrophy),
increased pulmonary circulation
CXR: Large supracardiac shadow can be
seen, which together with the normal
cardiac shadow forms a Snowman
sign
ECG: RAD, RBBB, if mild RVH (pure R
wave and upright T wave in V1)
CXR
o Normal or RV cardiomegaly
o Normal or dilated Main Pulmonay
Artery (post stenotic dilatation)
ECG: May show ischemia in severe
stenosis
ECG: LVH
CXR: Enlargement, Rib notching
(children > 5 years; around 7 years old)
3.1
Severely cyanotic neonates: Blalock-Taussig
procedure
Between 4-8 months: Bidirectional Glenn
Shunt
1.5 -3 years old: Modified Fontan Operation
Digitalis and diuretics for heart failure
Elective repair at 6 weeks old
o Surgery: Rastelli Repair
o Older patients who already have pulmonary
vascular obstruction, routine surgical treatment is
contraindicated and heart-lung transplantation is
the only option
Prostaglandin E before surgery to dilate the DV and
DA
Van Praagh surgical procedure
Balloon Valvuloplasty
Valvotomy (Brocks Procedure)
SBE prophylaxis
Balloon valvuloplasty
Valve replacement (Ross Procedure)
Secondary Bacterial Endocarditis (SBE) prophylaxis
Hypertension tx
Elective repair at 2-3 years old
Aggressive management of CHF
Balloon angiography (shunt placement)

Seen in Turners Syndome
Hemodynamics
Obstruction of the left ventricular
outflow pressure hypertrophy of
the LV
3.1
Pediatric Cardiology (Summary Table)
compared to the lower extremities o Treatment of choice: Surgical repair
o Systolic ejection murmur Primary re-anastomosis or a patch
aortoplasty
Subclavian flap aortoplasty (Waldhausen and
Nahrwold)