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IPAH — RACP Paediatrics MCQ

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ModerateCardiologyIPAHRACP Paediatrics

A 3-year-old presents with progressive exercise intolerance and syncope. Echocardiography shows elevated RV pressure with no structural heart disease. Cardiac catheterisation confirms mean pulmonary artery pressure of 45 mmHg with normal PAWP and no shunt. All secondary causes are excluded. What is the most likely diagnosis?

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Correct answer: A — Idiopathic pulmonary arterial hypertension

Elevated mean PAP (>20 mmHg) with normal PAWP and exclusion of secondary causes (congenital heart disease, lung disease, thromboembolic disease) after thorough workup defines idiopathic pulmonary arterial hypertension (IPAH). It is rare in children but carries significant morbidity and mortality. Treatment includes pulmonary vasodilators (phosphodiesterase inhibitors, endothelin receptor antagonists, prostanoids).

Reference: RCH Melbourne – 2023 – Pulmonary Hypertension CPG; RACP Paediatric Curriculum – Cardiology