马方综合征合并Stanford B型主动脉夹层患者临床特征与疗效的单中心回顾性研究  

A single-center retrospective study on the clinical characteristics and therapeutic effects of patients with Marfan syndrome complicated with Stanford type B aortic dissection

作　　者：贾崧淏 贡鸣[1] 孙立忠[2] 朱俊明[2] 刘永民[2] 刘愚勇[1] 张宏家[1] JIA Songhao;GONG Ming;SUN Lizhong;ZHU Junming;LIU Yongmin;LIU Yuyong;ZHANG Hongjia(Department of Cardiac Surgery,Beijing Anzhen Hospital,Capital Medical University,Beijing Institute of Heart,Lung and Blood Vessel Diseases,Beijing 100029,China;不详)

机构地区：[1]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、心脏外科、心血管疾病精准医学北京实验室、北京大血管疾病诊疗中心,北京市大血管外科植入式人工材料工程技术研究中心,100029 [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、心脏外科、心血管疾病精准医学北京实验室、北京大血管疾病诊疗中心,100029

出　　处：《心肺血管病杂志》2023年第2期142-148,共7页Journal of Cardiovascular and Pulmonary Diseases

基　　金：国家自然科学基金(82241205);科技部国家重点项目经费(2017YFC1308000);北京市医院管理局“登峰”计划专项经费资助(DFL20180602)。

摘　　要：目的:回顾性分析了我中心马方综合征(MFS)合并Stanford B型主动脉夹层患者的临床特征和中期随访结果。方法:回顾性分析2003年1月至2021年12月,在北京安贞医院就诊的61例MFS合并Stanford B型主动脉夹层患者的临床特征和结局。按治疗方案分为手术治疗组(n=50)和药物治疗组(n=11),比较两组基线特征和30d死亡率。对手术治疗组患者,按既往是否接受过主动脉根部手术分为两组,比较两组基线特征、手术方式、围手术期并发症、30d死亡率、中期死亡率和再次手术发生率。结果:患者平均年龄(35.2±8.72)岁,30例(49.2%)患者既往接受过主动脉根部手术。50例(82.0%)患者接受了手术治疗,11例(18.0%)患者接受了药物治疗。药物治疗组患者更年轻(P<0.001),发病时间更短(P=0.02),两组患者的30d死亡率差异无统计学意义(P=0.90)。在手术治疗组,27例(54%)患者接受了胸腹主动脉置换术,6例(12%)患者接受了Bentall+孙氏手术,4例(8%)患者接受了Bentall+降主动脉术中支架置入术,3例(6%)接受了降主动脉腔内修复术。在5.91年的中位随访时间内,共有9例(22.5%)患者死亡,6例(15%)患者接受再次手术,Kaplan-Meier生存分析表明显示既往有主动脉根部手术组患者的中期生存高于无主动脉根部手术组(Log-rank P=0.049),免于再手术率两组差异无统计学意义(Log-rank P=0.29)。单因素和多因素COX回归分析发现术后神经系统并发症(HR=32.41;P=0.00)和术前有主动脉瓣反流(HR=3.91;P=0.00)是患者中期死亡的危险因素。结论:MFS合并Stanford B型主动脉夹层患者更年轻,再手术风险更高,既往接受过主动脉根部手术的患者有更高的中期生存率,但不会影响其再手术率。未来应更加细致的探索治疗策略,降低MFS合并Stanford B型主动脉夹层患者死亡率和再手术率。Objective:There are currently no reports on patients with Marfan syndrome and Stanford type B aortic dissection.We retrospectively analyzed the clinical characteristics and mid-term follow-up results of patients with Marfan syndrome and Stanford type B aortic dissection in our center.Methods:The clinical characteristics and outcomes of 61 patients with Marfan syndrome and Stanford type B aortic dissection who were treated in Beijing Anzhen Hospital from January 2003 to December 2021 were retrospectively analyzed.They were divided into surgical treatment group(n=50)and medical treatment group(n=11)according to the treatment plan,and the baseline characteristics and 30-day mortality were compared between the two groups.The patients in the surgical treatment group were divided into two groups according to whether they had received aortic root surgery in the past.Baseline characteristics,surgical methods,perioperative complications,30-day mortality,mid-term mortality,and reoperation rates were compared between the two groups.Results:The mean age of the patients was(35.2±8.7)years,and 30(49.2%)patients had previously undergone aortic root surgery.Fifty(82.0%)patients received surgery and 11(18.0%)patients received medical therapy.Patients in the drug treatment group were younger(P<0.001)and had a shorter time to onset(P=0.02),and there was no significant difference in 30-day mortality between the two groups(P=0.90).In the surgical treatment group,27(54%)patients underwent thoracoabdominal aortic replacement,6(12%)patients underwent Bentall+Sun procedure,and 4(8%)patients underwent Bentall+FET,3(6%)underwent endovascular repair of the descending aorta.During a median follow-up of 5.91 years,9(22.5%)patients died and 6(15%)patients underwent reoperation,and Kaplan-Meier survival analysis showed that patients with prior aortic root surgery had higher medium-term survival In the group without aortic root surgery(Logrank P=0.049),there was no significant difference in the rate of freedom from reoperation between the two g

关 键 词：马方综合征 Stanford B型主动脉夹层 治疗 

分 类 号：R54[医药卫生—心血管疾病]