• Benoit Lechartier, Nihel Berrebeh, Alice Huertas, Marc Humbert, Christophe Guignabert, and Ly Tu.
• Pulmonary Division, Lausanne University Hospital, Lausanne, Switzerland; School of Medicine, Université Paris-Saclay, Le Kremlin-Bicêtre, France; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis-Robinson, France; AP-HP, Department of Respiratory and Intensive Care Medicine, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
• Chest. 2022 Jan 1; 161 (1): 219-231.

AbstractPulmonary arterial hypertension (PAH) is a progressive incurable condition that is characterized by extensive remodeling of the pulmonary circulation, leading to severe right-sided heart failure and death. Similar to other vascular contractile cells, pulmonary arterial smooth muscle cells play central roles in physiological and pathologic vascular remodeling because of their remarkable ability to dynamically modulate their phenotype to ensure contractile and synthetic functions. The dysfunction and molecular mechanisms underlying their contribution to the various pulmonary vascular lesions associated with PAH have been a major focus of research. The aim of this review is to describe the medial and nonmedial origins of contractile cells in the pulmonary vascular wall and present evidence of how they contribute to the onset and progression of PAH. We also highlight specific potential target molecules and discuss future directions that are being explored to widen the therapeutic options for the treatment of PAH.Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.

21 keywords...

hide…