Pulmonary Vascular Disease

Question · easy

Which of the following accounts for the majority of pulmonary hypertension (PH) cases?

Sarcoidosis and other multifactorial mechanisms Chronic thromboembolic disease Left-sided heart disease and chronic lung disease Heritable pulmonary arterial hypertension (e.g., BMPR2 mutations)

Question · medium

A 46-year-old woman is evaluated for progressive exertional dyspnea. Transthoracic echocardiography estimates a right ventricular systolic pressure of 58 mm Hg with right ventricular enlargement. Which of the following statements regarding further evaluation is correct?

The echocardiogram alone is sufficient to confirm pulmonary hypertension if estimated pressures are markedly elevated CT pulmonary angiography is more sensitive than V/Q scanning for chronic thromboembolic disease Right heart catheterization is required to confirm the diagnosis and help define its cause A ventilation/perfusion (V/Q) scan is indicated only if the patient has a documented history of pulmonary embolism

Question · hard

According to the 2022 ESC/ERS guidelines, which hemodynamic profile defines pre-capillary pulmonary hypertension?

mPAP >20 mm Hg with PVR >2 Wood units and pulmonary artery wedge pressure >15 mm Hg mPAP ≥25 mm Hg with PVR >3 Wood units and pulmonary artery wedge pressure ≤15 mm Hg mPAP >20 mm Hg with PVR >2 Wood units and pulmonary artery wedge pressure ≤15 mm Hg mPAP ≥25 mm Hg with PVR >2 Wood units and pulmonary artery wedge pressure >15 mm Hg

Question · medium

A 52-year-old man with dyspnea on exertion had a pulmonary embolism 2 years ago. V/Q scanning shows mismatched perfusion defects, and right heart catheterization confirms pre-capillary pulmonary hypertension. Imaging shows surgically accessible chronic thrombus. In addition to lifelong anticoagulation, what is the most appropriate management?

Riociguat as definitive first-line therapy in place of surgery Balloon pulmonary angioplasty as the preferred substitute for surgery in all patients Three months of anticoagulation followed by reassessment before any surgical referral Referral to an experienced center for pulmonary thromboendarterectomy (PEA) evaluation

Question · hard

Sotatercept was approved in 2024 as add-on therapy for pulmonary arterial hypertension. Which of the following best describes its mechanism?

An activin signaling inhibitor that rebalances the BMPR2/TGF-β axis toward antiproliferative signaling An oral prostacyclin (IP) receptor agonist that reduces disease progression An endothelin receptor antagonist requiring serial liver chemistry monitoring A soluble guanylate cyclase stimulator that acts independently of endogenous nitric oxide