Congenital Heart Disease

By: Dr Ismah, Pediatric Department

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OUTLINE• Epidemiology

• Anatomy

• Types

• Clinical approach

- History, physical exams

- Investigation

- General management

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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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ANATOMY

http://www.stanfordchildrens.org

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www.rch.org.au5

TYPES

Acyanotic Cyanotic

• Atrial septal defects (ASD)

• Ventricular septaldefects (VSD)

• Patent ductusarteriosus (PDA)

• Tetralogy of Fallot(TOF)

• Tricuspid atresia (TA)

• Transposition of the great vessels

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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

8http://www.merckmanuals.com

Treatment

Small defects:

• No treatment

Large defects:

• Elective closure at 4-5 years age

9 Paeds Protocol 3rd Ed

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

10http://www.merckmanuals.com

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

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

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http://www.merckmanuals.com

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 vstranscatheter) depends on symptom severity, size of PDA and body weight.

13 Paeds Protocol 3rd Ed

Tetralogy of Fallot

14http://my.clevelandclinic.org

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

15http://www.merckmanuals.com

Transposition of great arteries

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http://mvpresource.com

Tricuspid atresia

17www.riversideonline.com

HISTORY

• Antenatal scans (cardiac malformation, fetal arrhythmias, hydrops).

• Family history of congenital heart disease.

• Maternal illness: diabetes, rubella, teratogenicmedications.

• Perinatal problems: prematurity, meconiumaspiration, perinatal asphyxia.

18 Paeds Protocol 3rd Ed

PHYSICAL EXAMINATIONS

• Dysmorphism: Trisomy 21, 18, 13; Turner syndrome

• Central cyanosis.

• Tachypnoea.

• Weak or unequal pulses.

• Heart murmur.

• Hepatomegaly.

19 Paeds Protocol 3rd Ed

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

GENERAL MANAGEMENT

• Correct metabolic acidosis, electrolyte

derangements, hypoglycaemia; prevent

hypothermia.

• 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, hypotension.

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• 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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THANK YOU

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