skip to main content

Operable chronic thromboembolic pulmonary hypertension with antiphospholipid syndrome — SCE Respiratory MCQ

Instant feedback + full explanation. One question, done properly.

HardAdultsOperable chronic thromboembolic pulmonary hypertension with antiphospholipid syndromeSCE Respiratory

A 63-year-old woman has persistent exertional dyspnoea 8 months after an unprovoked pulmonary embolism. She has received apixaban continuously and is in WHO functional class III. Echocardiography shows right ventricular dilatation and impaired systolic function. Ventilation–perfusion scintigraphy demonstrates multiple bilateral segmental mismatched perfusion defects. Right heart catheterisation shows a mean pulmonary arterial pressure of 42 mmHg, pulmonary arterial wedge pressure of 9 mmHg and pulmonary vascular resistance of 7.1 Wood units. CT pulmonary angiography and catheter-based pulmonary angiography demonstrate organised webs, bands and pouch occlusions affecting the main, lobar and proximal segmental pulmonary arteries bilaterally. There is no clinically significant parenchymal lung disease or left-heart disease. A national chronic thromboembolic pulmonary hypertension multidisciplinary team considers the obstructive material surgically accessible and her perioperative risk acceptable. Testing prompted by a previous late miscarriage demonstrates lupus anticoagulant and high-titre anticardiolipin and anti-β2-glycoprotein-I antibodies, confirmed on repeat testing 12 weeks later. Which management plan is most appropriate?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: B — Switch to lifelong warfarin and proceed to pulmonary endarterectomy

This is established chronic thromboembolic pulmonary hypertension: persistent mismatched defects and organised thromboembolic lesions accompany pre-capillary pulmonary hypertension after adequate anticoagulation. The decisive anatomical finding is surgically accessible proximal disease in a patient whose operative risk has been accepted by a specialist CTEPH team. Pulmonary endarterectomy is therefore the treatment of choice and should not be displaced by medical therapy or balloon pulmonary angioplasty. Her persistently triple-positive antiphospholipid antibodies and obstetric history establish high-risk antiphospholipid syndrome. CTEPH requires lifelong therapeutic anticoagulation, and ESC/ERS guidance specifically recommends a vitamin K antagonist when antiphospholipid syndrome is present. Warfarin should therefore replace apixaban. A is inappropriate because riociguat is principally recommended for inoperable CTEPH or persistent/recurrent pulmonary hypertension after endarterectomy, and apixaban is not the preferred anticoagulant in antiphospholipid syndrome. B incorrectly substitutes balloon pulmonary angioplasty, which is primarily used for technically inoperable distal obstruction or suitable residual disease after surgery. C delays potentially curative surgery; routine pretreatment with riociguat is not required merely because pulmonary vascular resistance is raised. D selects the correct intervention but retains a direct oral anticoagulant despite confirmed antiphospholipid syndrome. Thus, lifelong warfarin combined with pulmonary endarterectomy is the single best plan.

Reference: 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension (6 January 2023) — https://publications.ersnet.org/content/erj/61/1/2200879 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension (6 January 2023) — https://publications.ersnet.org/content/erj/61/1/2200879 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension (6 January 2023) — https://publications.ersnet.org/content/erj/61/1/2200879