Extract
Pulmonary arterial hypertension (PAH) is a chronic, progressive and rapidly fatal disease with a poor prognosis, high mortality rate and lack of curative treatment options [1], defined by a mean pulmonary artery (PA) pressure greater than 20 mmHg at a rest [2, 3]. The main pathophysiological features of PAH include vasoconstriction, remodelling of small muscular PAs and perivascular inflammation [4]. PAH is a multifactorial disease. Genetic pre-disposition, epigenetic events, environmental exposure and other underlying conditions trigger endothelial cell (EC) apoptosis, leading to vascular pruning and loss of microvasculature, as well as cancer-like hyper-proliferation and survival of PA smooth muscle cells (PASMCs), PAECs and PA adventitial fibroblasts, and obstruction of small muscular PAs [1, 4, 5]. The functional and structural microvascular rarefaction results in the reduction in cross-sectional lumen area and in unresolved increase in right ventricular (RV) afterload, leading to premature death from right heart failure [6].
Abstract
Dysbalanced Wnt signalling promotes pulmonary arterial hypertension http://bit.ly/3ZvuNXf
Footnotes
Conflict of interest: T.V. Kudryashova reports funding from the National Institutes of Health (grant 1R01 HL166932). E.A. Goncharova and S.S. Pullamsetti have nothing to disclose.
Support statement: This work is supported, at least in part, by National Institutes of Health (NIH) grant 1R01 HL166932 to T.V. Kudryashova. Funding information for this article has been deposited with the Crossref Funder Registry.
- Received March 23, 2023.
- Accepted March 25, 2023.
- Copyright ©The authors 2023. For reproduction rights and permissions contact permissions{at}ersnet.org
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