波生坦治疗成人肺动脉高压研究进展

Research progress on bosentan in treating patients with pulmonary arterial hypertension

肺动脉高压是临床上常见的一种致残率和致死率较高的疾病,内皮素受体拮抗剂是目前治疗肺动脉高压的常用靶向药物之一。波生坦作为第1个研发并应用于临床的内皮素受体拮抗剂,目前广泛应用于肺动脉高压的治疗。单药治疗方面,波生坦可以改善特发性肺动脉高压、结缔组织病相关性肺动脉高压患者的运动耐量、血流动力学状态和长期生存率;但其应用于先心病相关肺动脉高压患者的长期生存获益及慢性血栓栓塞性肺动脉高压患者的疗效仍缺乏证据。对于心功能Ⅲ~Ⅳ级的肺动脉高压患者,初始应用波生坦联合另一种靶向药物治疗可能优于单药治疗,而对于心功能Ⅱ~Ⅲ级的肺动脉高压患者,目前仍缺少联合用药优于单药治疗的证据。

Pulmonary arterial hypertension （PAH） is a life-threatening disease which may cause right heart dysfunction and even death.As the first developed and clinically applied endothelin receptor antagonist, Bosentan has been widely used in patients with PAH. Monotherapy of bosentan may have short-time benefits associated with improved functional capacity and hemodynamic parameters in idiopathic PAH patients and patients with PAH secondary to connective tissue diseases （CTD） or congenital heart diseases （CHD）. Long-time benefits of survival have been observed in all of the above-mentioned patients except CHD-PAH. In addition, the efficacy is not clear in patients with chronic thromboembolic pulmonary hypertension （CTEPH） at present. Furthermore, initial combination therapy of bosentan and other categories of targeted drugs may have better outcomes than monotherapy in PAH patients with World Health Organization （WHO） functional class Ⅲ-Ⅳ , yet there is a lack of evidence in patients with WHO functional class Ⅱ-Ⅲ .