Atrioventricular Septal Defect — RACP Paediatrics MCQ

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HardCardiologyAtrioventricular Septal DefectRACP Paediatrics

A 6-month-old with trisomy 21 is found to have a large atrioventricular septal defect (AVSD). When should surgical repair ideally be performed to prevent irreversible pulmonary hypertension?

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Correct answer: B — By 3–6 months of age

Children with trisomy 21 are at accelerated risk of developing irreversible pulmonary vascular disease. Complete AVSD repair is recommended by 3–6 months of age (or earlier if symptoms are present) to prevent Eisenmenger syndrome. Delaying beyond 6 months in this population significantly increases the risk of fixed pulmonary hypertension.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Atrioventricular Septal Defect