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CTEPH — EECC MCQ

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ModeratePulmonary Vascular DiseaseCTEPHEECC

A 55-year-old woman with unexplained exertional dyspnoea and a history of pulmonary embolism 3 years ago has persistent perfusion defects on V/Q scintigraphy despite 3 years of anticoagulation. Right heart catheterisation shows mPAP 38 mmHg, PAWP 10 mmHg, PVR 6 Wood units, and CO 4.2 L/min. CT pulmonary angiography shows organised thrombus in the lobar and segmental arteries. What is the recommended first-line treatment?

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Correct answer: C — Surgical pulmonary endarterectomy (PEA) at an experienced centre

This patient has chronic thromboembolic pulmonary hypertension (CTEPH — Group 4 PH). The 2022 ESC/ERS PH Guidelines recommend surgical pulmonary endarterectomy (PEA) as the treatment of choice for operable CTEPH (Class I, LOE C), as it is the only potentially curative treatment. Operability assessment should be performed at expert CTEPH centres. For inoperable CTEPH or persistent/recurrent PH after PEA, riociguat is recommended (Class I, LOE B). BPA is recommended for technically inoperable patients or residual PH post-PEA (Class I, LOE B). All CTEPH patients require lifelong anticoagulation in addition to any interventional treatment. A multimodal approach (PEA + BPA + medical therapy) is now recognised.

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