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CTEPH After PE Recognition — EECC MCQ

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ModeratePulmonary Vascular DiseaseCTEPH After PE RecognitionEECC

A 45-year-old man with recurrent unprovoked PE has a V/Q scan showing mismatched perfusion defects despite 6 months of therapeutic anticoagulation. His CTPA shows organised thrombus. mPAP on RHC is 38 mmHg. What diagnosis should be considered and what is the first step?

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Correct answer: D — Chronic thromboembolic pulmonary hypertension (CTEPH) should be considered — referral to an expert CTEPH centre for multidisciplinary assessment of operability (PEA vs BPA vs medical therapy) is the essential first step

CTEPH develops in 3-4% of patients after acute PE, when organised fibrotic thrombus persists in the pulmonary arteries despite adequate anticoagulation, causing mechanical obstruction and secondary small vessel arteriopathy. The ESC 2022 PH Guidelines recommend: (1) V/Q scintigraphy as the screening test for CTEPH (more sensitive than CTPA); (2) RHC to confirm PH; (3) CTPA/MRA and pulmonary angiography for anatomical assessment; (4) referral to an expert CTEPH centre for multidisciplinary assessment. Treatment: PEA (curative, first-line if operable), BPA (for inoperable/residual disease), riociguat (medical therapy — CHEST trials). Lifelong anticoagulation is required. CTEPH should be considered in any patient with persistent dyspnoea or exercise intolerance after adequate PE treatment.

Reference: ESC/ERS (2022): PH Guidelines