艾森门格综合征的研究进展

The Advance in Research on Eisenmenger's Syndrome

艾森门格综合征(Eisenmenger’s syndrome,ES)是先天性心脏病终末期的一种病理生理综合征,诊断方法明确,但早期临床表现缺乏特异性,预后不佳。近年来,随着广大学者的不懈努力,艾森门格综合征的治疗方式不断改进。新药物、改良外科手术方式、体外膜式氧合、移植和基因等治疗方式不仅可以缓解症状、纠正肺循环血流动力学异常,在一定程度上还改善了艾森门格综合征患者的预后。我们就近年来艾森门格综合征的发病机制、诊断、治疗等研究进展进行综述。

Eisenmenger＇s syndrome is a kind of pathophysiologic syndrome that occurs in the end stage of congenital heart disease.The diagnosis of Eisenmenger＇s syndrome is clear,but its early clinical presentations are nonspecific and its prognosis is poor.Recently,the therapeutic regimen is steadily improved by the endless efforts that lots of scientists have made.Treatments including new drugs,modified surgery,extracorporeal membrane oxygenation,transplantation and gene therapy can not only relieve symptoms,correct abnormal pulmonary hemodynamics,but also can improve prognosis to some extent.We will review and introduce the advance of research on Eisenmenger＇s syndrome,including its pathogenesis,diagnosis and treatment.