The Pathobiology of Pulmonary Arterial Hypertension

Sudarshan Rajagopal; Yen-Rei A Yu

doi:10.1016/j.ccl.2021.08.001

The Pathobiology of Pulmonary Arterial Hypertension

Cardiol Clin. 2022 Feb;40(1):1-12. doi: 10.1016/j.ccl.2021.08.001.

Authors

Sudarshan Rajagopal¹, Yen-Rei A Yu²

Affiliations

¹ Division of Cardiology, Department of Medicine, Duke University Medical Center, Room 128A Hanes House, 330 Trent Drive, Durham, NC 27710, USA. Electronic address: sudarshan.rajagopal@duke.edu.
² Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado, 12605 E. 16th Avenue, Aurora, CO 80045, USA.

PMID: 34809910
DOI: 10.1016/j.ccl.2021.08.001

Abstract

Pulmonary arterial hypertension is characterized by obliteration and obstruction of the pulmonary arterioles that in turn results in high right ventricular afterload and right heart failure. The pathobiology of pulmonary arterial hypertension is complex, with contributions from multiple pathophysiologic processes that are regulated by a variety of molecular mechanisms. This nature likely explains the limited efficacy of our current therapies, which only target a small portion of the pathobiological mechanisms that underlie advanced disease. Here we review the pathobiology of pulmonary arterial hypertension, focusing on the systemic, cellular, and molecular mechanisms that underlie the disease.

Keywords: Endothelial cell; Fibroblast; Inflammation; Pulmonary arterial hypertension; Smooth muscle cell.

Publication types

Review

MeSH terms

Familial Primary Pulmonary Hypertension
Heart Failure*
Heart Ventricles
Humans
Hypertension, Pulmonary*
Pulmonary Arterial Hypertension*
Pulmonary Artery