摘要
目的总结马方综合征合并Stanford B型主动脉夹层患者的外科治疗方案以及经验体会,探讨治疗策略选择。方法回顾性分析2013年1月至2019年6月于我院心脏大血管外科接受治疗的马方综合征合并胸腹主动脉疾病27例患者的临床资料,其中男13例、女14例,平均年龄(32.2±8.6)岁。根据患者病情,19例行单泵辅助输血联合全胸腹主动脉置换术(TAAAR),8例急危重症及妊娠患者行胸主动脉腔内修复术(TEVAR)。患者术后通过门诊随访,于术后3个月、6个月、12个月及每年复查胸腹主动脉CT血管造影。研究患者手术结果、中期心血管不良事件发生率和中期生存率,其中心血管不良事件定义为患者因为主动脉或者心脏病变再次接受手术治疗。结果27例患者均顺利完成手术,手术时间60~852(395.10±222.60)min,脊髓缺血时间14~26(19.33±3.44)min,腹腔脏器缺血时间16~23(19.83±1.94)min。8例TEVAR均为急性期手术,19例TAAAR均为慢性期手术。术后早期死亡2例,均发生在TEVAR患者中,1例同期剖宫产术后,产褥期感染并多器官功能障碍死亡;1例行TEVAR术后再发A型夹层,家属放弃治疗,行剖宫产术后夹层破裂死亡。平均随访(47.6±36.7)个月后,1例患者死于脑血管意外。9例因不良心血管事件再次手术,其中TEVAR 4例,TAAAR 5例。结论TAAAR治疗马方综合征合并胸腹主动脉疾病近中期治疗效果良好,是首选治疗方式。TEVAR手术操作简单,早期死亡率和并发症发生率低,但有内漏和逆撕的风险,中期再手术率高,可应用于高危、高龄、妊娠等不适合开放手术的患者,或者作为急诊患者开放手术前的桥接治疗。
Objective To summarize the surgical treatment plan and experience of patients with Marfan syndrome complicated with Stanford type B aortic dissection,and to explore the treatment strategy selection.Methods A retrospective analysis was conducted on 27 patients with Marfan syndrome complicated with thoracoabdominal aortic diseases who were treated in the department of cardiovascular surgery of our hospital from January 2013 to June 2019,including 13 males and 14 females,with an average age of 32.2±8.6 years.According to the patients’conditions,19 of them received single pump-assisted blood transfusion combined with total thoracoabdominal aortic replacement(TAAAR),and8 received thoracic endovascular aortic repair(TEVAR)in critically ill and pregnant patients.The patients were followed up in the outpatient clinic,and the thoracoabdominal aortic CT angiography was reexamined at 3 months,6 months,12 months and annually.The outcome of surgery,the incidence of intermediate cardiovascular adverse events,defined as the reoperation due to aortic or cardiac diseases,and intermediate survival rate were studied.Results All 27 patients successfully completed the operation,the operation time was 60-852(395.10±222.60)min,the spinal cord ischemia time was 14-26(19.33±3.44)min,and the abdominal viscera ischemia time was 16-23(19.83±1.94)min.Eight patients of TEVAR were all operated in acute phase and 19 patients of TAAAR in chronic phase.Two early postoperative deaths occurred in TEVAR patients.One died of puerperal infection and multiple organ dysfunction after cesarean section at the same time.After TEVAR,type A dissection re-ocurred in one patient.The family member gave up the treatment,and the patient died of the dissection ruptured after cesarean section.During the average follow-up of 47.6±36.7 months,1 patient died of cerebrovascular accident and 9 patients were reoperated for adverse cardiovascular events,including 4 in TEVAR and 5 in TAAAR.Conclusion TAAAR is the first choice for the treatment of Marfan syndrome com
作者
石娟
马名嘉
魏翔
SHI Juan;MA Mingjia;WEI Xiang(Department of Cardiovascular Surgery,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan,453300,P.R.China)
出处
《中国胸心血管外科临床杂志》
CAS
CSCD
2020年第7期742-748,共7页
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery
