Marfan综合征的外科治疗

SURGICAL TREATMENT OF MARFAN SYNDROME

目的 :总结 31例马凡综合征的外科治疗经验。方法 :自 1991年 10月至 1999年 5月收治Marfan综合征 31例 ,所有病例均有升主动脉瘤合并主动脉瓣关闭不全 ,1例同时合并明显主动脉瓣狭窄。2 6例施行Bentall手术 (带瓣人造血管升主动脉置换术和冠状动脉移植术 )。结果 :5例未手术患者均发生猝死。前期 9例手术中 ,死于术后出血 2例和低心排综合征 1例 ,6例痊愈。 1995年以来 17例手术均获成功 ,无住院死亡。其中 1例在出院 1年后因脑血管意外晚期死亡。结论 :Marfan综合征预后凶险 ,如升主动脉最大内径超过 55～ 60mm ,应及早施行Bentall手术 。

Objective:To review the surgical experience in the treatment of Marfan syndrome.Methods:From Oct.1991 to May,1999,31patients with Marfan syndrome were admitted to our hospital,all of whom had aneurysm of ascending aorta with associated aortic insufficiency,and one also had marked aortic stenosis.26 of all patients underwent aortic composite graft with valve replacement and reimplantation of coronary arteries(Bentall operation).Result:All of the 5 patients not treated surgically met with sudden deaths.Of our first 9 surgically treated patients,2 died of postoperative hemorrhage,1 of low cardiac output syndrome,and 6 recovered.From 1995,17 patients received successful operations without mortality during hospitalization,but one of them died of stroke later on.Conclusion:Since the prognosis is extremely dangerous,patients with Marfan syndrome should undergo Bentall operation as early as possible when the greatest diameter of the ascending aorta exceeds 55～60mm, and our experience suggests that the result of Bentall operation is satisfactory.