艾森曼格综合征曾被认为是心脏外科矫治手术的禁忌证，需要进行肺移植或心肺联合移植治疗，但由于供体缺乏，大多数患儿只能通过保守治疗延长生存时间。近年来一些临床研究提示肺动脉高压靶向药物治疗ES有一定效果。现就ES治疗的最新进展作一综述。“,”Eisenmenger syndrome (ES) was considered as a surgical contraindication in the past time. Only a few patients got lung transplantations or heart-lung transplantations due to lack of donors. Most of the patients had to choose conservative treatment to prolong their survival time. Recently, some clinical trials indicated targeted therapies in pulmonary arterial hypertension (PAH) were effective for the treatment of ES. This article reviewed the recent advances in this area.