Abstract: 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.