Recent Advances in Chronic Thromboembolic Pulmonary Hypertension: Expanding the Disease Concept and Treatment Options.

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive form of pulmonary hypertension characterized by unresolved thromboembolic occlusion of pulmonary arteries, leading to increased pulmonary arterial pressure and right heart failure. This review examines recent advances in the pathophysiology, diagnosis, and management of CTEPH, focusing on expanding disease concepts and evolving therapeutic approaches. The incidence of CTEPH has been revised upward with improved diagnostic techniques revealing a higher prevalence than previously recognized. Advances in surgical and interventional therapies, particularly pulmonary endarterectomy and balloon pulmonary angioplasty, have significantly improved outcomes. Emerging medical therapies, including pulmonary vasodilators like riociguat, have offered new hope for inoperable cases. The understanding of CTEPH has broadened, leading to better diagnostic strategies and more comprehensive treatment options that significantly enhance patient outcomes. Multidisciplinary team approaches are crucial in managing the disease effectively.