Pathogenesis, Diagnosis, and Management of Chronic Thromboembolic Pulmonary Hypertension

Document Type

Article

Publication Date

7-1-2023

Publication Title

Interv Cardiol Clin

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.

Medical Subject Headings

Hypertension, Pulmonary; Chronic Disease; Endarterectomy; Pyrazoles; Pyrimidines

PubMed ID

38964822

Volume

12

Issue

3S

First Page

37

Last Page

37

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