关键词: 动脉性肺动脉高压, 分子通路, 配受体, 靶向治疗, 药物研发

Abstract: Pulmonary arterial hypertension (PAH) is a complex pulmonary vascular disease characterized by progressive elevation of mean pulmonary artery pressure resulted from the pathological feature of pulmonary vascular remodeling. Without medical intervention, PAH can eventually lead to right heart failure and death of patients. Up to the present, there are few treatment options for PAH are still mainly function through pulmonary vasodilation. Although these treatments can alleviate symptoms, the prognosis remains poor. In recent years, breakthroughs have been made in understanding the pathogenesis of PAH, thus support the development of new treatment strategies targeting at dysregulation of signaling pathways in PAH. This review focuses on five critical pathways and the relevant drugs those entered phase Ⅱ clinical trials and discusses their therapeutic potential, so to provide a basis for future research on targeting therapies for PAH patients.

Key words: pulmonary arterial hypertension, molecular pathways, ligands and receptors, targeted therapy, drug development