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Chronic thromboembolic pulmonary hypertension — ABIM Board MCQ

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HardCardiovascularChronic thromboembolic pulmonary hypertensionABIM Board

A 53-year-old woman has progressive exertional dyspnea 18 months after an unprovoked pulmonary embolism. She has remained adherent to therapeutic anticoagulation. Echocardiography shows right ventricular enlargement and an estimated pulmonary artery systolic pressure of 62 mm Hg; left ventricular systolic and valvular function are normal. Pulmonary function tests show no significant obstructive or restrictive disease. CT pulmonary angiography is nondiagnostic because of motion artifact. Which diagnostic study should be obtained next to determine whether chronic thromboembolic obstruction is the cause of her pulmonary hypertension?

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Correct answer: E — Ventilation-perfusion SPECT lung scanning

The correct answer is E. Chronic thromboembolic pulmonary hypertension should be considered in a patient with persistent dyspnea and pulmonary hypertension more than 3 months after pulmonary embolism. Ventilation-perfusion scanning, preferably with SPECT, is the preferred screening study because it sensitively detects the characteristic segmental mismatched perfusion defects; a normal scan essentially excludes CTEPH. Repeat conventional CT pulmonary angiography can miss distal chronic webs or stenoses and is not the preferred exclusion test. Right heart catheterization confirms and characterizes pulmonary hypertension but does not establish its thromboembolic cause. Catheter pulmonary angiography is generally used after abnormal screening to define vascular anatomy and treatment feasibility. Cardiopulmonary exercise testing can characterize unexplained exercise limitation but cannot reliably exclude CTEPH.

Reference: Ha S, Han S. The Role of Lung Ventilation/Perfusion Scan in the Management of Chronic Thromboembolic Pulmonary Hypertension, Diagnostic Work-up and Diagnostic Performance of V/Q Scintigraphy sections, 2024. https://pmc.ncbi.nlm.nih.gov/articles/PMC11612049/