Chronic thromboembolic pulmonary hypertension: epidemiology, pathogenesis and natural history

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension induced by the partial obliteration of pulmonary arteries by organized thromboemboli, usually with coexisting remodeling of still patent pulmonary arterioles. It is compatible with group 4, according to the current classification of pulmonary hypertension. CTEPH has several peculiarities. They include persistent incertainties regarding its causes and pathogenesis, but also the possibility of highly effective surgical treatment in a significant proportion of patients by means of pulmonary endarterectomy. This complex intervention may restore exercise capacity, quality of life and life expectancy, otherwise all significantly limited by CTEPH.