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Pathogenesis, Diagnosis, and Management of Chronic Thromboembolic Pulmonary Hypertension
Malika Elhage Hassan1, Jorge Vinales2, Sidney Perkins3
1Division of Cardiology, Department of Medicine, Emory University School of Medicine, 1364 Clifton Road Northeast Suite F606, Atlanta, GA 30322, USA.
Abstract:
Chronic thromboembolic pulmonary hypertension (CTEPH) is thought to occur as a sequelae of thromboembolic processes in the pulmonary vasculature. The pathophysiology of CTEPH is multifactorial, including impaired fibrinolysis, endothelial dysregulation, and hypoxic adaptations. The diagnosis of CTEPH is typically delayed considering the nonspecific nature of the symptoms, lack of screening, and relatively low incidence. Diagnostic tools include ventilation-perfusion testing, echocardiography, cardiac catheterization, and pulmonary angiography. The only potentially curative treatment for CTEPH is pulmonary endarterectomy However, approximately 40% of patients are inoperable. Currently, only Riociguat is Food and Drug Administration approved specifically for CTEPH, with additional drug trials underway.
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