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CTEPH — RACP Adult Medicine MCQ

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HardRespiratoryCTEPHRACP Adult Medicine

A 55-year-old woman with a history of recurrent PE (5 years ago) presents with progressive exertional dyspnoea. V/Q scan shows multiple segmental mismatched perfusion defects. Right heart catheterisation confirms PH (mPAP 38 mmHg). CT angiography shows organised thrombus with webs and bands. What is the most likely diagnosis?

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Correct answer: E — Chronic thromboembolic pulmonary hypertension

Multiple mismatched perfusion defects on V/Q scan with organised thrombus on CTPA and confirmed PH on RHC defines CTEPH (Group 4 PH). Unlike acute PE, the thrombus is organised with webs and bands. Treatment of choice is pulmonary endarterectomy (PEA) at an experienced centre. Balloon pulmonary angioplasty or riociguat are options for inoperable CTEPH.

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