摘要目的 探讨马方综合征合并Stanford A型主动脉夹层累及主动脉弓病变的患者行主动脉全弓替换结合支架象鼻手术(孙氏手术),术后支架象鼻远端主动脉的变化及中期预后.方法 回顾分析2009年2月至2014年2月,47例Stanford A型主动脉夹层累及弓部病变且术前远端主动脉均无明显扩张的马方综合征患者资料,男38例,女9例;年龄19～ 50岁,平均(32.43±7.96)岁.;均行孙氏手术治疗,其中急诊手术30例(63.8％,30/47).依据孙氏术后1年支架象鼻远端主动脉扩张速度是否大于5 mm,分为远端主动脉残余夹层病变好转组(29例)和进展组(18例).观察术前和术后随访CTA影像学表现,对比分析术后远端主动脉假腔愈合、血栓化程度和主动脉重塑等预后情况.结果 47例患者手术均成功.平均随访1年,随访期间远端主动脉夹层破裂死亡1例(2.1％,1/47);再次行全胸腹主动脉替换4例(8.5％,4/47),再次手术时间间隔6～12个月,平均(9.88±2.84)个月.术后随访CTA显示:支架象鼻段降主动脉假腔完全血栓化率达85.1％ (40/47);远端主动脉痊愈率25.5％(12/47).好转组远端主动脉直径年增长率低,支架象鼻远端降主动脉、膈肌主动脉裂孔水平和肾动脉水平分别为(0.00±3.41) mm、(1.14±2.20) mm和(0.97 ±2.15) mm.孙氏术后,远端主动脉残余夹层病变进展组和好转组相比,支架象鼻段降主动脉假腔完全血栓化率降低(72.2％对93.1％,P=0.089),再次全胸腹主动脉替换手术发生率增加(22.2％对0,P=0.017).主动脉破裂风险事件发生率增加(5.6％对0,P =0.383);术后1年远端主动脉直径明显扩张:支架象鼻末端降主动脉段(40.17±7.09) mm对(27.86±6.77) mm(P＜0.001),膈肌主动脉裂孔水平(42.17 ±9.91) mm对(27.48±7.14) mm(P＜0.001),肾动脉水平(38.22 ±6.90) mm对(24.00 ±6.18) mm(P ＜0.001).结论 采用孙氏手术治疗马方综合征合并StanfordA型主动脉夹层累及弓部病变患者,有利于促进支架象鼻段假腔血栓化形成,全主动脉痊愈和主动脉重塑,并延长了再次手术时间间隔.孙氏手术后,当远端主动脉扩张速度大于5 mm/年时,应积极监测远端主动脉直径变化,防止主动脉破裂风险事件的发生.

abstractsObjective The purpose of the study was to assess the distal aorta changes and prognosis after total arch replacement combined with stented elephant trunk implantation(Sun's procedure) for Marfan patients with Stanford type A aortic dissection involving the aortic arch.Methods Between February 2009 and February 2014,47 Marfan patients (38 males,9 females) with Stanford type A aortic dissection involving the aortic arch underwent Sun's procedure.Mean ages(32.43 ± 7.96) years(ranged from 19 to 50 years).According to whether the growth rate of the distal aortic diameter is more than 5 mm/year or not after the first year,the data it was divided into the improve group(29 cases) and the progressive group(18 cases).The residual false lumen thrombosis and the diameter of the distal aorta were evaluated by CT date.Results All patients were survived and discharged after Sun's procedure.The mean follow-up period was 1 years.The survival rate was 97.9％ (46/47) and 1 patients died.The total recover of the distal aorta was achieved in12 patients(25.5％) after procedure.The reoperation of total thoracoabdominal aortic replacement rate of the distal aorta was 8.5％ (4/47) and the reoperation interval was(9.88 ±2.84) month(6-12 month).Complete thrombus formation around the stented elephant trunk was observed in 85.1％ (40 of 47).The annual rate of growth of the distal aorta were:the descending aorta segment of stented elephant trunk (0.00 s3.41) mm,the diaphragm level(1.14 ±2.20)mm,the renal artery level(0.97 ±2.15)mm.Complete thrombus formation around the stented elephant trunk of theprogressive group was lower thanthe improvegroup(72.2％ vs 93.1％,P =0.089).The reoperation rate of total thoracoabdominal aortic replacement surgery of the progressive group was higher than the improve group(22.2％ vs 0,P =0.017).The incidence of aortic rupture risk of the progressive group was higher than the improve group (5.6％ vs 0,P =0.383).The diameter of the distal aorta after Sun's procedure of the progressive group was higher than the improve group after 1 year:the descending aorta segment of stented elephant trunk end(40.17 ±7.09) mm vs (27.86 ±6.77)mm(P ＜0.001),the diaphragm level(42.17 ±9.91)mm vs(27.48 ±7.14) mm(P ＜0.001),the renal artery level (38.22 ± 6.90) mm vs(24.00 ± 6.18) mm (P ＜ 0.001),the difference was statistically significant.Conclusion Using Sun's procedure for Marfan patients with Stanford type A aortic dissection involving the aortic arch would promote false lumen thrombosis of stented elephant trunk and aortic remodeling and delay the time interval of the reoperation.The Marfan patients in progressive group which the diameter of the distal aortic growth rate was more than 5mm/year,should be actively carry out rigorous monitoring of the distal aorta and prevention of aortic rupture risk events.