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HLHS — RACP Paediatrics MCQ

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ModerateCardiologyHLHSRACP Paediatrics

A term neonate presents on day 2 with cardiovascular collapse, poor peripheral perfusion, and metabolic acidosis. Echocardiogram reveals a hypoplastic left ventricle, atretic mitral valve, and atretic aortic valve. All systemic blood flow is duct-dependent. What is the most likely diagnosis?

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Correct answer: A — HLHS

HLHS (hypoplastic left heart syndrome) is a duct-dependent systemic circulation lesion. As the ductus arteriosus closes, systemic output drops precipitously leading to cardiovascular collapse. Immediate prostaglandin E1 is lifesaving. Management involves staged surgical palliation (Norwood, Glenn, Fontan) or cardiac transplantation.

Reference: RCH Melbourne – 2023 – Cyanotic Newborn CPG; RACP Paediatric Curriculum – Cardiology