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TAPVR — RACP Paediatrics MCQ

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HardCardiologyTAPVRRACP Paediatrics

A term neonate is noted to be cyanotic at 4 hours of age. SpO2 is 70%. CXR shows a 'snowman' or 'figure-of-eight' cardiac silhouette with plethoric lung fields. Echocardiogram shows all four pulmonary veins draining into a vertical vein connected to the innominate vein. What is the most likely diagnosis?

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Correct answer: A — Total anomalous pulmonary venous return

A 'snowman' or 'figure-of-eight' cardiac silhouette (due to the dilated vertical vein and innominate vein) with cyanosis and pulmonary plethora is characteristic of supracardiac TAPVR (total anomalous pulmonary venous return). If the venous return is obstructed, the presentation is a surgical emergency. Urgent surgical correction is required.

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