医学
肺动脉高压
肺动脉
病态的
发病机制
表观遗传学
血流动力学
心室重构
心脏病学
心力衰竭
内科学
胚胎血管重塑
生物
生物化学
基因
作者
A. A. Roger Thompson,Allan Lawrie
标识
DOI:10.1016/j.molmed.2016.11.005
摘要
Pulmonary arterial hypertension (PAH) describes a group of conditions with a common hemodynamic phenotype of increased pulmonary artery pressure, driven by progressive remodeling of small pulmonary arteries, leading to right heart failure and death. Vascular remodeling is the key pathological feature of PAH, but treatments targeting this process are lacking. In this review, we summarize important advances in our understanding of PAH pathogenesis from novel genetic and epigenetic factors, to cell metabolism and DNA damage. We show how these processes may integrate and highlight exploitable targets that could alter the relentless vascular remodeling in PAH.
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