In-depth analysis of a case of persistent severe chronic thromboembolic pulmonary hypertension

Abstract Pulmonary hypertension (PH) is characterized by an increase in the pulmonary arterial pressure. It has been classified into five groups. Management depends on the group classification, can be medical or surgical. Chronic thromboembolic pulmonary hypertension (CTEPH) is group four PH. It is characterized by chronic thromboembolic phenomena (non-resolution of pulmonary emboli) that result in fibrosis and scarring, resulting in obstruction of the pulmonary vasculature on various levels, casing PH. CTEPH diagnosis is done by ventilation/perfusion (V/Q) scan. Work up usually includes transthoracic Echocardiogram (TEE), Right heart catheterization (RHC), BNP (or ProBNP) and hypercoagulability testing. Other work up might be needed to rule out other causes of PH, and can include high resolution CT (HRCT), rheumatological studies, thyroid function test, HIV testing and liver ultrasound. Gold standard therapy is surgical pulmonary endarterectomy (PEA). Pulmonary balloon angioplasty (BPA) is a new emerging intervention that is promising but needs to be better studied. Some medications have been studied in management of inoperable or persistent CTEPH (post PEA) include Bosentan (improved hemodynamics but not exercise capacity), Macitentan (improved both hemodynamics and clinical parameters), Riociguat (improved both hemodynamics and exercise capacity).