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Systemic sclerosis-associated PAH — SCE Respiratory MCQ

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ModeratePulmonary Vascular DiseaseSystemic sclerosis-associated PAHSCE Respiratory

A 31-year-old woman with systemic sclerosis has exertional syncope and dyspnoea. Echocardiography suggests pulmonary hypertension; HRCT shows no significant ILD and left ventricular function is normal. Right-heart catheterisation shows mPAP 34 mmHg, PAWP 9 mmHg and PVR 5 Wood units. What is the most likely diagnosis?

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Correct answer: C — Connective-tissue-disease associated pulmonary arterial hypertension

Pre-capillary pulmonary hypertension on right-heart catheterisation in systemic sclerosis with minimal ILD supports CTD-associated PAH. A normal wedge pressure argues against left heart disease. CTEPH still needs exclusion, but the data given make systemic-sclerosis PAH the most likely diagnosis.

Reference: ESC/ERS Pulmonary Hypertension Guideline 2022