How does pulmonary endarterectomy cure CTEPH: A clue to cure PAH?

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a hot topic in the field of pulmonary hypertension because many CTEPH patients are now curable by surgical pulmonary endarterectomy. However, there are still uncertainties regarding the pathogenesis of CTEPH, specifically how and where the small vessel arteriopathy that is indistinguishable from that in pulmonary arterial hypertension (plexogenic arteriopathy) develops and how pulmonary endarterectomy improves hemodynamics and possibly cures CTEPH. Based on our recent experimental finding that hemodynamic stress is fundamental for the development of plexogenic arteriopathy, we discuss the uncertainties of CTEPH and potential implication of the effectiveness of pulmonary endarterectomy for reversing plexogenic arteriopathy and possibly providing a novel approach to cure pulmonary arterial hypertension.