paeds, congenital heart disease

1. Congenital Heart
Disease
By: Dr Ismah, Pediatric Department
1

2. OUTLINE
• Epidemiology
• Anatomy
• Types
• Clinical approach
- History, physical exams
- Investigation
- General management
2

3. EPIDEMIOLOGY
• 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 [2012]
• About 69% of babies born with critical CHDs are
expected to survive to 18 years of age [2012]
http://www.cdc.gov
• 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
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4. ANATOMY
http://www.stanfordchildrens.org
4

5. www.rch.org.au
5

6. TYPES
Acyanotic Cyanotic
• Atrial septal defects
(ASD)
• Ventricular septal
defects (VSD)
• Patent ductus
arteriosus (PDA)
• Tetralogy of Fallot
(TOF)
• Tricuspid atresia
(TA)
• Transposition of the
great vessels
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7. 7

8. Atrial Septal Defect
• Most commonly asymptomatic
• Features:
- 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
8
http://www.merckmanuals.com

9. Treatment
Small defects:
• No treatment
Large defects:
• Elective closure at 4-5 years age
9 Paeds Protocol 3rd Ed

10. Ventricular Septal Defect
Clinical findings
• Grade II-IV/VI,
medium- to high-
pitched, harsh
pansystolic murmur
heard best at the lower
left sternal border with
radiation over the entire
precordium
10
http://www.merckmanuals.com

11. Treatment
Small defects: Moderate defects: Large
defects:
No treatment;
high rate of
spontaneous
closure.
• SBE
prophylaxis.
• Yearly follow up
for aortic valve
prolapse,
regurgitation.
• Surgical closure
indicated if
prolapsed aortic
valve.
- Anti-failure therapy if heart
failure.
- Surgical closure if:
• Heart failure not controlled by
medical therapy.
• Persistent cardiomegaly on
chest X-ray.
• Elevated pulmonary arterial
pressure.
• Aortic valve prolapse or
regurgitation.
• One episode of infective
endocarditis.
Early primary
surgical
closure.
• Pulmonary
artery banding
followed by
VSD closure in
multiple VSDs.
11 Paeds Protocol 3rd Ed

12. Patent Ductus Arteriosus
• Pulses are bounding and pulse pressure is
widened
• Characteristically has continuous murmur is
heard best in the upper left sternal border,
machinery murmur
12
http://www.merckmanuals.com

13. Treatment
Small PDA:
• No treatment if there is no murmur
• If murmur present: elective closure as risk
of endarteritis.
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

14. Tetralogy of Fallot
14
http://my.clevelandclinic.org

15. Typical features
• 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
15
http://www.merckmanuals.com

16. Transposition of great arteries
16
http://mvpresource.com

17. Tricuspid atresia
17 www.riversideonline.com

18. HISTORY
• Antenatal scans (cardiac malformation, fetal
arrhythmias, hydrops).
• Family history of congenital heart disease.
• Maternal illness: diabetes, rubella, teratogenic
medications.
• Perinatal problems: prematurity, meconium
aspiration, perinatal asphyxia.
18 Paeds Protocol 3rd Ed

19. PHYSICAL EXAMINATIONS
• Dysmorphism: Trisomy 21, 18, 13;
Turner syndrome
• Central cyanosis.
• Tachypnoea.
• Weak or unequal pulses.
• Heart murmur.
• Hepatomegaly.
19 Paeds Protocol 3rd Ed

20. INVESTIGATIONS
- CXR
- 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.
- Echocardiography
20 Paeds Protocol 3rd Ed

21. GENERAL MANAGEMENT
• Correct metabolic acidosis, electrolyte
derangements, hypoglycaemia; prevent
hypothermia.
• Empirical treatment with IV antibiotics.
• Early cardiology consultation.
21 Paeds Protocol 3rd Ed

22. • 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,
hypotension.
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23. • If unresponsive to IV prostaglandin E,
consider:
- Transposition of great arteries, obstructed
total anomalous pulmonary.
- Blocked IV line.
- Non-cardiac diagnosis.
• Arrangement to transfer to regional
cardiac center once stabilized.
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24. THANK YOU
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