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Chronic thromboembolic pulmonary hypertension — SCE Respiratory MCQ

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HardRespiratory MedicineChronic thromboembolic pulmonary hypertensionSCE Respiratory

A 58-year-old woman remains breathless 10 months after an unprovoked pulmonary embolism despite continuous anticoagulation. Ventilation–perfusion scanning shows bilateral mismatched segmental perfusion defects. Right-heart catheterisation demonstrates a mean pulmonary arterial pressure of 38 mmHg, pulmonary arterial wedge pressure of 9 mmHg and pulmonary vascular resistance of 6.2 Wood units. Pulmonary angiography shows predominantly segmental and subsegmental webs. A national chronic thromboembolic pulmonary hypertension multidisciplinary team, including an experienced pulmonary endarterectomy surgeon, considers the disease technically inoperable but amenable to balloon pulmonary angioplasty. She is in WHO functional class III. Triple-positive antiphospholipid syndrome has been confirmed on samples taken more than 12 weeks apart. She currently takes apixaban and sildenafil, initiated before specialist referral. Her blood pressure is 118/70 mmHg and renal and hepatic function are normal. Which management plan is most appropriate?

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Correct answer: B — Change apixaban to warfarin; discontinue sildenafil; start riociguat after 24 hours; proceed to staged balloon pulmonary angioplasty after medical pretreatment

This is symptomatic, haemodynamically confirmed CTEPH that has undergone expert operability assessment. Because the obstructive disease is technically inoperable but amenable to BPA, staged BPA is appropriate. Her PVR is greater than 4 Wood units, a setting in which medical treatment before BPA is advised to reduce interventional complications. Riociguat is the guideline-recommended drug for symptomatic inoperable CTEPH. Triple-positive antiphospholipid syndrome changes the anticoagulant choice: lifelong anticoagulation is required, and a vitamin K antagonist is recommended rather than continued apixaban. Sildenafil cannot be combined with riociguat because concurrent PDE5 inhibition and soluble guanylate cyclase stimulation is contraindicated owing to excess hypotension. The UK SmPC requires sildenafil to be stopped at least 24 hours before riociguat begins. A omits indicated medical pretreatment despite PVR 6.2 Wood units. B retains off-label sildenafil rather than using recommended riociguat. C combines sildenafil and riociguat, which is contraindicated. D correctly addresses pulmonary vasodilator sequencing and BPA but incorrectly retains a direct oral anticoagulant despite confirmed antiphospholipid syndrome. Thus E integrates the required anticoagulant, drug transition and multimodal CTEPH sequence.

Reference: 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension (2022) — https://publications.ersnet.org/content/erj/61/1/2200879 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: CTEPH treatment algorithm (2022) — https://publications.ersnet.org/index.php/highwire_display/entity_view/node/597221/full Adempas 1.0 mg film-coated tablets: Summary of Product Characteristics (12 December 2025) — https://www.medicines.org.uk/emc/product/7926/smpc