Congenital Heart Disease and Pediatric Cardiology
Items test murmur localisation, the physiology of cyanotic lesions, duct-dependent circulations, and Kawasaki disease, which is the most frequently examined acquired paediatric cardiac condition.
Murmurs and lesions
- VSD: harsh holosystolic murmur at the left lower sternal border, often with a thrill.
- ASD: fixed split second heart sound and a pulmonary systolic flow murmur; closure if significant shunt or right ventricular enlargement.
- PDA: continuous machinery murmur at the left infraclavicular area.
- Tetralogy of Fallot: cyanosis, squatting, boot-shaped heart, diminished pulmonary flow.
- Coarctation: upper limb hypertension, weak femoral pulses, radio-femoral delay, rib notching.
Duct-dependent circulation
- Prostaglandin E1 at 0.01-0.05 micrograms/kg/min keeps the duct open in critical coarctation, interrupted arch, pulmonary atresia, and transposition; watch for apnoea.
- The hyperoxia test distinguishes cyanotic congenital heart disease from pulmonary disease: a arterial oxygen tension that stays low on 100% oxygen favours cardiac mixing.
Tetralogy spells
- Knee-chest position, oxygen, morphine, phenylephrine, and beta-blockade; avoid dehydration and prolonged crying.
Kawasaki disease
- Fever for 5 days or more plus conjunctivitis, mucositis, rash, extremity changes, and cervical adenopathy.
- Treat with intravenous immunoglobulin 2 g/kg and aspirin; risk of coronary aneurysm, greatest with delayed treatment, male sex, and persistent fever.
- Giant aneurysms exceed 8 mm and require long-term anticoagulation and cardiology follow-up.
Adult congenital disease
- Bicuspid aortic valve is the commonest congenital lesion and needs surveillance for stenosis, regurgitation, and aortopathy.
- Eisenmenger syndrome: avoid pregnancy and systemic vasodilators that drop systemic resistance.
Worked example
A 6-week-old infant presents with poor feeding, weak femoral pulses, metabolic acidosis, and severe hypertension in the right arm. The diagnosis is critical coarctation; start prostaglandin E1 and inotropes, then surgical repair.
Traps
- A normal oxygen saturation does not exclude duct-dependent lesions.
- Do not give oxygen as the sole treatment for a tetralogy spell; it can worsen systemic resistance.
- A murmur may be absent in severe obstruction or Eisenmenger physiology.