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CTEPH Operability Assessment — EECC MCQ

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ModeratePulmonary Vascular DiseaseCTEPH Operability AssessmentEECC

A 65-year-old woman with CTEPH (chronic thromboembolic pulmonary hypertension) is assessed by the PH multidisciplinary team. Pulmonary endarterectomy (PEA) is considered. She has segmental and lobar perfusion defects on V/Q scan and mPAP 45 mmHg. What determines operability?

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Correct answer: E — Operability depends on the location of organised thrombus (surgically accessible in main, lobar, and segmental vessels), PVR, and assessment by an expert PEA surgical centre; PEA is the treatment of choice and potentially curative for CTEPH

PEA (pulmonary endarterectomy) is the treatment of choice for CTEPH and is potentially curative, removing organised fibrotic thrombus from the pulmonary arteries under deep hypothermic circulatory arrest. The 2022 ESC/ERS PH Guidelines recommend assessment at an expert CTEPH centre for all patients. Operability depends on: (1) location — thrombus must be surgically accessible (main, lobar, proximal segmental arteries); distal/subsegmental disease is less amenable; (2) PVR relative to accessible disease (high PVR with minimal proximal disease suggests significant distal small-vessel disease — higher surgical risk); (3) patient comorbidities. For inoperable or residual PH post-PEA: balloon pulmonary angioplasty (BPA) and medical therapy (riociguat — the only approved PAH drug for CTEPH, per the CHEST trials).

Reference: ESC/ERS (2022): Guidelines for Pulmonary Hypertension