Pulmonary Arterial Hypertension — RACP Adult Medicine MCQ
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Correct answer: B — Pulmonary 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