Citation: Cardiovascular Revascularization Medicine. 2020 Jul 10.Journal: Cardiovascular revascularization medicine : including molecular interventions.Published: ; 2020ISSN: 1878-0938.Full author list: Ali L; Ghazzal A; Radwan S; Barnett C.UI/PMID: 33608240.Subject(s): IN PROCESS -- NOT YET INDEXEDInstitution(s): MedStar Washington Hospital Center | MedStar Heart & Vascular InstituteDepartment(s): Medicine/General Internal MedicineActivity type: Journal Article.Medline article type(s): Case ReportsOnline resources: Click here to access onlineDigital Object Identifier: https://dx.doi.org/10.1016/j.carrev.2020.07.012 (Click here)Abbreviated citation: Cardiovasc Revasc Med. 2020 Jul 10.Local Holdings: Available in print through MWHC library: 2002 - present.Abstract: 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 (TTE), right heart catheterization (RHC), NT pro-B-type natriuretic peptide (NT Pro-BNP) 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 (BPA) may be useful, however, further studies are required. Several pulmonary artery hypertension (PAH) 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 association for this indication. Copyright (c) 2020 Elsevier Inc. All rights reserved.