Department of Internal Medicine, School of Medicine, University of Louisville, Louisville, KY 40202, USA.
Division of Cardiology, School of Medicine, University of Louisville, Louisville, KY 40202, USA.
Int J Mol Sci. 2022 Sep 2;23(17):10001. doi: 10.3390/ijms231710001.
Pulmonary arterial hypertension is a multifactorial, chronic disease process that leads to pulmonary arterial endothelial dysfunction and smooth muscular hypertrophy, resulting in impaired pliability and hemodynamics of the pulmonary vascular system, and consequent right ventricular dysfunction. Existing treatments target limited pathways with only modest improvement in disease morbidity, and little or no improvement in mortality. Ongoing research has focused on the molecular basis of pulmonary arterial hypertension and is going to be important in the discovery of new treatments and genetic pathways involved. This review focuses on the molecular pathogenesis of pulmonary arterial hypertension.
肺动脉高压是一种多因素、慢性疾病过程，导致肺动脉内皮功能障碍和平滑肌肥厚，从而使肺血管系统的柔韧性和血液动力学受损，并导致右心室功能障碍。现有的治疗方法针对的是有限的途径，只能使疾病发病率得到适度改善，而对死亡率几乎没有改善。正在进行的研究集中在肺动脉高压的分子基础上，对于发现新的治疗方法和涉及的遗传途径非常重要。这篇综述重点介绍了肺动脉高压的分子发病机制。
