马凡综合征合并主动脉夹层患者的外科治疗  

Surgical treatment of patients with Marfan syndrome and aortic dissection

作　　者：王湘 李刚 马琼 庄贤勉 王尧 张锐 WANG Xiang;LI Gang;MA Qiong;ZHUANG Xian-mian;WANG Yao;ZHANG Rui(Department of Big Vascular Surgery,Shenzhen Hospital,Fuwai Hospital,Chinese Academy of Medical Sciences(Shenzhen Sun Yat-sen Cardiovascular Hospital),Shenzhen,Guangdong 518000,China)

机构地区：[1]中国医学科学院阜外医院深圳医院(深圳市孙逸仙心血管医院)心脏大血管外科,广东深圳518000

出　　处：《岭南心血管病杂志》2022年第5期423-426,共4页South China Journal of Cardiovascular Diseases

基　　金：深圳市医学三名工程项目(项目编号:SZSM201911019)。

摘　　要：目的探讨马凡综合征(Marfan syndrome,MFS)合并主动脉夹层(aortic dissection,AD)患者的外科治疗方案、手术效果。方法回顾性收集2009年4月至2019年12月在中国医学科学院阜外医院深圳医院(深圳市孙逸仙心血管医院)行外科手术治疗的MFS合并AD患者的临床资料,并进行分析。结果42例纳入分析的MFS合并AD患者中,住院死亡3例,出院39例患者,随访(59±11)个月,其中5例患者实施了二次胸腹主动脉替换术(thoracic and abdominal aortic replacement,TAAR)手术。结论MFS合并AD患者情况多变,外科手术方案应以Bentall+Sun为主,合并胸腹主动脉瘤时应同期或分期行TAAR;MFS合并A型AD患者在接受Bentall+Sun手术治疗后,降主动脉易扩张形成胸腹主动脉瘤,部分患者需要行再次TAAR。Objectives To investigate the surgical treatment of patients with Marfan syndrome(MFS)complicated with aortic dissection(AD).Methods Clinical data of patients with MFS complicated with AD who underwent surgery in Fuwai Hospital,Chinese Academy of Medical Sciences(Shenzhen Sun Yat-sen Cardiovascular Hospital)from April 2009 to December 2019 were retrospectively collected and analyzed.Results Among the 42 MFS patients with AD included in the analysis,3 patients died in hospital and 39 patients were discharged from hospital.The patients were followed up for(59±11)months.Five patients were performed thoracic and abdominal aortic replacement(TAAR)during the following-up.Conclusions Patients with MFS combined with AD are changeable,and Bentall+Sun should be the main surgical plan.When combined with thoracic and abdominal aortic aneurysms,TAAR should be performed simulta⁃neously or by stages.After Bentall+Sun surgery,the descending aorta of MFS patients with type A AD is easy to dilate and form thoracic and abdominal aortic aneurysms,and some patients needed TAAR surgery again.

关 键 词：马凡综合征 主动脉夹层 Sun手术 

分 类 号：R654[医药卫生—外科学]