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Systemic sclerosis-associated PAH — SCE Respiratory MCQ

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HardPulmonary vascular diseaseSystemic sclerosis-associated PAHSCE Respiratory

A 44-year-old woman with systemic sclerosis has progressive exertional syncope and breathlessness. Echocardiography shows right atrial enlargement and estimated pulmonary artery systolic pressure 70 mmHg. FVC is 88% predicted, TLCO is 32% predicted, HRCT shows minimal ILD and NT-proBNP is raised. The pulmonary hypertension MDT is consulted. What is the most appropriate investigation?

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Correct answer: CRight-heart catheterisation

The best answer is “Right-heart catheterisation”. Right-heart catheterisation is the investigation that most directly resolves the remaining diagnostic or staging uncertainty at this point in the pathway. The alternatives address different questions, have lower expected yield, or would be premature before this result. The remaining choices—“Exercise spirometry alone”, “Empirical sildenafil in the absence of haemodynamic confirmation”, “Bronchoscopy with lavage, after specialist assessment”, “Repeat echocardiogram in two years”—are credible in related respiratory presentations, but each addresses a different diagnostic, staging or management decision and does not fit the decisive findings and pathway position in this stem.

Reference: British Thoracic Society respiratory guidance library: https://www.brit-thoracic.org.uk/quality-improvement/guidelines/