Hsu Chih-Hsin, Huang Wei-Chun, Chang Wei-Ting
Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan.
Department of Internal Medicine, National Cheng Kung University Hospital, Dou-Liou Branch, Yunlin.
Acta Cardiol Sin. 2022 Jul;38(4):435-442. doi: 10.6515/ACS.202207_38(4).20220331A.
Since the discovery of three major pathophysiological mechanisms of pulmonary arterial hypertension (PAH), including prostacyclin, endothelin and nitric oxide pathways, the therapeutic options for PAH have increased. Nevertheless, despite these advances, the prognosis remains unsatisfactory for many patients with PAH. With the progress of both pre-clinical and clinical research on PAH, several novel therapeutic targets have been identified for the treatment of PAH. In this study, we review updated information of novel pathophysiological pathways of pulmonary hypertension, mainly focusing on WHO Group I PAH. Drugs based on these pathways are currently under clinical or pre-clinical investigation, however they have been approved for clinical use. Large clinical trials are required to validate the clinical safety and effects of these novel therapies.
自从发现肺动脉高压（PAH）的三种主要病理生理机制，包括前列环素、内皮素和一氧化氮途径以来，PAH的治疗选择有所增加。然而，尽管取得了这些进展，许多PAH患者的预后仍然不尽人意。随着PAH临床前和临床研究的进展，已确定了几个用于治疗PAH的新治疗靶点。在本研究中，我们回顾了肺动脉高压新病理生理途径的最新信息，主要聚焦于世界卫生组织第一组PAH。基于这些途径的药物目前正在进行临床或临床前研究，但尚未获批用于临床。需要进行大规模临床试验来验证这些新疗法的临床安全性和效果。
