6例年轻马方综合征合并胸腹主动脉瘤的外科治疗  

Surgical treatment of 6 cases of young Marfan syndrome with thoracoabdominal aortic aneurysm

作　　者：闵心平 夏军[1] 徐鹏[1] 昌金星 王志维[1] 朱俊明[2] MIN Xinping;XIA Jun;XU Peng;CHANG Jinxing;WANG Zhiwei;ZHU Junming(Department of Cardiovascular Surgery,People's Hospital of Wuhan University,Wuhan 430060,China)

机构地区：[1]武汉大学人民医院心血管外科,430060 [2]首都医科大学附属北京安贞医院-北京市心肺血管疾病研究所心血管外科

出　　处：《心肺血管病杂志》2020年第8期958-962,978,共6页Journal of Cardiovascular and Pulmonary Diseases

摘　　要：目的:马方综合征(MFS)合并胸腹主动脉夹层动脉瘤患者常需再次及多次手术治疗,本研究在于探讨年轻MFS合并胸腹主动脉瘤外科治疗(TAAAR)的特点及经验总结。方法:随访2008年至今,共6例MFS合并胸腹主动脉瘤行外科手术患者,分析弓部及分支血管处理、脊髓动脉及内脏动脉重建方式对再次手术和脊髓保护的影响。结果:随访时间(7.4±3.4)年,6例男性患者,平均年龄25.5岁,共进行13次手术,围手术期无患者死亡,术后截瘫1例,双下肢肌力减退1例;5例为MFS合并Stanford A型主动脉夹层,其中1例一期行Bentall及右半弓替换,二期行CrawfordⅡ型TAAAR,1例一期行孙氏手术,二期行CrawfordⅡ型TAAAR,另外有3例患者一期行Bentall及全弓替换(术中支架左锁骨下动脉"开窗"术),二期行CrawfordⅡ型TAAAR,主动脉根部瘤合并CrawfordⅡ型胸腹主动脉瘤1例,一期行TAAAR,再次手术间隔时间为(5.0±1.8)年。结论:MFS合并主动脉病变,外科同期弓部重建,为二期手术准备,术中支架慎重"开窗",为二期手术"减压";脊髓动脉重建对预防术后截瘫具有重要意义;内脏动脉功能重建,具有多样性,依据术中情况而定,不宜残留病变血管;手术方案个体化即解决"当务之急"同时兼顾后期外科治疗。Objective:The purpose of this study is to explore the characteristics and experience of surgical treatment of young Marfan syndrome(MFS)with thoracic abdominal aortic aneurysm(TAAA).Methods:Six patients with MFS and TAAA were followed up from 2008 to now.The effects of the treatment of arch and branch vessels,the reconstruction of spinal artery and splanchnic artery on reoperation and spinal cord protection were analyzed.Results:The follow-up time was(7.4±3.4)years.There were 6 male patients underwent 13 operations,with an average age of 25.5 years.One case with paraplegia and one with hypo-muscularity of lower limbs occurred postoperatively without death.5 cases were MSF with Stanford Type A aortic dissection,in which 1 case underwent Bentall and right hemiarch replacement in the one-stage,thoracoabdominal aortic aneurysm replacement(TAAAR)in the second procedure,1 case underwent sun's operation in the one-stage,and TAAAR in the second stage.In addition,3 cases underwent Bentall and full arch replacement first(stent"fenestration"of left subclavian artery in operation),TAAAR secondly.The time interval between operations was(5.0±1.8)years.Conclusions:For MFS with aortic dissection,the reconstruction of the arch should be carried out simultaneously,and stent"fenestration"also should be avoided preparing for the reoperation.The reconstruction of spinal artery is of great significance to prevent the occur of postoperative paraplegia;it is not suitable to retain the diseased additional vascular tissue,and the individualization of surgical plan is to solve the"urgent task"and consider the later surgery.

关 键 词：马方综合征 胸腹主动脉瘤替换 再次手术 术中支架开窗 

分 类 号：R54[医药卫生—心血管疾病]