Pulmonary Vascular Research Group, Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.
Pulmonary Vascular Research Group, Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.
Trends Mol Med. 2017 Jan;23(1):31-45. doi: 10.1016/j.molmed.2016.11.005. Epub 2016 Dec 16.
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.
肺动脉高压（PAH）描述了一组具有共同的血流动力学表型的病症，即肺动脉压力升高，由小肺动脉的进行性重塑驱动，导致右心衰竭和死亡。血管重塑是 PAH 的关键病理特征，但针对这一过程的治疗方法却很缺乏。在这篇综述中，我们总结了对 PAH 发病机制的重要认识进展，包括新的遗传和表观遗传因素、细胞代谢和 DNA 损伤。我们展示了这些过程如何整合，并强调了可改变 PAH 中不可逆转的血管重塑的可利用靶点。
