肺动脉高压(PAH)是一种以肺血管阻力增加、肺动脉压(PAP)升高为主要特征的疾病。患者出现PAH压后可以诱发进行性右心衰竭和肺血管疾病,大部分患者诊断PAH后预期寿命仅有2～3年。近年来随着对PAH发病机制的认识不断深入,治疗水平不断提高,大大改善了患者的生存质量。以下就近年 Pulmonary hypertension (PAH) is a disease characterized by increased pulmonary vascular resistance and elevated pulmonary arterial pressure (PAP). Patients with PAH can induce progressive right heart failure and pulmonary vascular disease, most patients with PAH life expectancy after only 2 to 3 years. In recent years, with the understanding of the pathogenesis of PAH deepening, the continuous improvement of treatment, greatly improving the quality of life of patients. The following is recent years