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Idiopathic pulmonary arterial hypertension — SCE Respiratory MCQ

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HardPulmonary vascular diseaseIdiopathic pulmonary arterial hypertensionSCE Respiratory

A 28-year-old woman with idiopathic pulmonary arterial hypertension is deteriorating despite initial oral therapy. Right-heart catheterisation shows mean pulmonary arterial pressure 56 mmHg, pulmonary vascular resistance 11 Wood units and low cardiac index. She has syncope on exertion and NT-proBNP remains markedly elevated. The specialist centre classifies her as high risk. What is the most appropriate management?

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Correct answer: B — Escalation to parenteral prostacyclin-based combination therapy

High-risk PAH with syncope, low cardiac index and high PVR requires specialist escalation, often including parenteral prostacyclin therapy. Diuretics help congestion but do not treat the pulmonary arteriopathy. Risk stratification drives PAH therapy rather than symptoms alone.

Reference: ESC/ERS Pulmonary Hypertension Guideline 2022