In-Depth Analysis of a Case of Persistent Severe Chronic Thromboembolic Pulmonary Hypertension

Pulmonary hypertension (PH) is a disease characterized by an increase in the pulmonary vascular resistance that typically progresses to right heart failure and death. It is classified into five groups. Management depends on the group classification. Group four PH, chronic thromboembolic pulmonary hypertension (CTEPH) is thought to be a result of acute pulmonary emboli that cause fibrosis and scarring of the pulmonary arteries with consequent obstruction. The diagnosis of CTEPH is made by identifying perfusion abnormalities on ventilation/perfusion (V/Q) scan. Other studies required for the diagnostic evaluation include transthoracic echocardiogram, right heart catheterization, NT pro-B-type natriuretic peptide and thrombophilia evaluation. Several other tests needed to exclude other causes of pulmonary hypertension include high-resolution computed tomography (HRCT), connective tissue disease evaluation, thyroid function testing, human immunodeficiency virus testing, and liver ultrasonography to exclude portal hypertension. The treatment for CTEPH is surgical pulmonary endarterectomy (PEA). In patients who are not candidates or decline PEA, pulmonary balloon angioplasty may be useful, however, further studies are required. Several pulmonary artery hypertension medications have been studied in the management of inoperable CTEPH or persistent PH following PEA including bosentan (improves hemodynamics but not exercise capacity), macitentan (improves both hemodynamics and clinical parameters), and riociguat (improves both hemodynamics and exercise capacity). However, only riociguat is approved by the Food and Drug Administration for this indication.