Hypoplastic Left Heart Syndrome — RACP Paediatrics MCQ

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ModerateCardiologyHypoplastic Left Heart SyndromeRACP Paediatrics

An infant with hypoplastic left heart syndrome is born at a peripheral hospital with SpO2 72%. What is the most important initial intervention?

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Correct answer: A — Prostaglandin E1 infusion

HLHS is a duct-dependent systemic circulation. PGE1 maintains ductal patency. Excessive oxygen should be avoided as it worsens the shunt balance.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Duct-Dependent Congenital Heart Disease