• CHDs affect nearly 1% of or about 40,000 births
per year in the United States
• The most common type of heart defect is a
ventricular septal defect (VSD)
• About 95% of babies born with a non-critical CHD
are expected to survive to 18 years of age 
• About 69% of babies born with critical CHDs are
expected to survive to 18 years of age 
• A study on under five deaths in Malaysia in the
year 2006 showed that 10% of mortality was
directly related to CHD - http://mjpch.com
Atrial Septal Defect
• Most commonly asymptomatic
- Right ventricular heave
- S2 widely split and usually fixed
- Grade I-III/VI systolic murmur at the
upper left sternal border
- Cardiac enlargement on CXR
• No treatment
• Elective closure at 4-5 years age
9 Paeds Protocol 3rd Ed
Ventricular Septal Defect
• Grade II-IV/VI,
medium- to high-
heard best at the lower
left sternal border with
radiation over the entire
Small defects: Moderate defects: Large
high rate of
• Yearly follow up
for aortic valve
• Surgical closure
- Anti-failure therapy if heart
- Surgical closure if:
• Heart failure not controlled by
• Persistent cardiomegaly on
• Elevated pulmonary arterial
• Aortic valve prolapse or
• One episode of infective
VSD closure in
11 Paeds Protocol 3rd Ed
Patent Ductus Arteriosus
• Pulses are bounding and pulse pressure is
• Characteristically has continuous murmur is
heard best in the upper left sternal border,
• No treatment if there is no murmur
• If murmur present: elective closure as risk
Moderate to large PDA:
• Anti-failure therapy if heart failure
• Timing, method of closure (surgical vs
transcatheter) depends on symptom
severity, size of PDA and body weight.
13 Paeds Protocol 3rd Ed
Tetralogy of Fallot
• Symptoms include cyanosis, dyspnea with
feeding, poor growth, and
• Hypercyanotic "tet" spells (sudden, potentially
lethal episodes of severe cyanosis)
• A harsh systolic murmur at the left upper
sternal border with a single 2nd heart sound
(S2) is common
Transposition of great arteries
- Hyperoxia test:
• Administer 100% oxygen via headbox at 15
L/min for 15 mins.
• ABG taken from right radial artery.
• Cyanotic heart diseases: pO₂ < 100 mmHg;
rise in pO₂ is < 20 mmHg.
20 Paeds Protocol 3rd Ed
• Correct metabolic acidosis, electrolyte
derangements, hypoglycaemia; prevent
• Empirical treatment with IV antibiotics.
• Early cardiology consultation.
21 Paeds Protocol 3rd Ed
• IV Prostaglandin E infusion if duct-
dependent lesions suspected:
- Starting dose: 10 – 40 ng/kg/min;
maintenance: 2 – 10 ng/kg/min.
- Adverse effects: apnoea, fever,
• If unresponsive to IV prostaglandin E,
- Transposition of great arteries, obstructed
total anomalous pulmonary.
- Blocked IV line.
- Non-cardiac diagnosis.
• Arrangement to transfer to regional
cardiac center once stabilized.