The role of endothelial cells in pulmonary hypertension: old concepts and new science

Abstract

Endothelial dysfunction plays a key role in the initiation and progression of pulmonary hypertension (PH), a fatal and currently incurable disease associated with increased pulmonary vascular resistance and leading to right heart failure. Recent data from genomic, transcriptomic and metabolomic studies and emergence of novel organ-on-chip technologies have provided new insights into the role of endothelium in the pathogenesis of this disease. In this review, we characterise key features of endothelial dysfunction in PH, highlighting the diversity of endothelial cell types and differences in their responses to vascular insults. We also present the current understanding of the effects of genetic mutations on endothelial function and comment on new ways of disease modelling using organ-on-chip technologies.