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Pulmonary Arterial Hypertension — RACP Adult Medicine MCQ

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HardRespiratoryPulmonary Arterial HypertensionRACP Adult Medicine

A 35-year-old woman presents with a 3-month history of progressive exertional dyspnoea, syncope on exertion, and peripheral oedema. Echo shows dilated right ventricle, elevated PASP of 75 mmHg, and normal left heart. CT pulmonary angiography excludes chronic thromboembolic disease. ANA is positive and she has Raynaud phenomenon. What is the most likely diagnosis?

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Correct answer: BPulmonary arterial hypertension

Exertional dyspnoea and syncope with echo evidence of elevated PASP and RV dilatation, normal left heart, excluded CTEPH, and features of connective tissue disease (ANA positive, Raynaud) suggests PAH associated with CTD (likely SSc). Right heart catheterisation is required to confirm PAH (mPAP >20 mmHg, PVR >2 WU). PAH-specific therapies (ERA, PDE5i, prostacyclin analogues) are indicated.

Reference: PHSANZ – 2024 – Pulmonary Hypertension Guidelines; ESC/ERS 2022 PH