Pathogenic Mechanisms of Pulmonary Arterial Hypertension

AbstractPulmonary arterial hypertension (PAH) is a progressive and fatal disease. Sustained pulmonary vasoconstriction and concentric pulmonary vascular remodeling contribute to the elevated pulmonary vascular resistance and pulmonary artery pressure in PAH. Endothelial cells regulate vascular tensi...

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Published in:JACC. Asia 2022-12, Vol.2 (7), p.787-802
Main Authors: Zhu, Jinsheng, PhD, Yang, Lei, MS, Jia, Yangfan, BS, Balistrieri, Angela, BS, Fraidenburg, Dustin R., MD, Wang, Jian, MD, Tang, Haiyang, PhD, Yuan, Jason X-J, MD, PhD
Format: Article
Language:English
Subjects:
Cardiovascular
endothelial dysfunction
endothelium-derived relaxing factor
pulmonary arterial hypertension
vascular homeostasis
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Summary:AbstractPulmonary arterial hypertension (PAH) is a progressive and fatal disease. Sustained pulmonary vasoconstriction and concentric pulmonary vascular remodeling contribute to the elevated pulmonary vascular resistance and pulmonary artery pressure in PAH. Endothelial cells regulate vascular tension by producing endothelium-derived relaxing factors (EDRFs) and endothelium-derived contracting factors (EDCFs). Homeostasis of EDRF and EDCF production has been identified as a marker of the endothelium integrity. Impaired synthesis or release of EDRFs induces persistent vascular contraction and pulmonary artery remodeling, which subsequently leads to the development and progression of PAH. In this review, the authors summarize how EDRFs and EDCFs affect pulmonary vascular homeostasis, with special attention to the recently published novel mechanisms related to endothelial dysfunction in PAH and drugs associated with EDRFs and EDCFs.
ISSN:2772-3747
2772-3747
DOI:10.1016/j.jacasi.2022.09.010