[Etiopathogenesis and physiopathology in chronic thromboembolic pulmonary hypertension]

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by the organization of thrombotic material within the pulmonary arteries. The lack of correlation between the proportion of obliterated pulmonary arteries and pulmonary hypertension values suggest that an exclusively mechanical theory could be too simplistic. Acute pulmonary embolism could be the initial event, but disease progression probably results from progressive vascular remodelling of the small vessels. Unresolved pulmonary arterial thrombosis may be a decisive factor in initiating endothelial-to-mesenchymal transition. The present article analyzes the genetic features, inflammatory mechanisms and clinical factors influencing the physiopathological mosaic of this complex entity.