马凡综合征合并胸腹主动脉瘤的手术治疗及早中期结果

目的 探讨手术治疗马凡综合征(MFS)合并胸腹主动脉瘤的早中期结果。方法 回顾性分析2009年1月至2017年12月在阜外医院接受胸腹主动脉替换术的156例患者的临床资料,分为MFS组(n=58)和非MFS组(n=98),比较两组患者的一般情况、术后早期结果和中期随访结果。结果 MFS组患者较非MFS组更年轻(32比45岁,t=9.603,P=0.000),有家族史者更多(19%比0,χ ²=19.996,P=0.000),男性和吸烟者比例更低(55.2% 比 80.6%,χ ²=11.489,P=0.001;13.8% 比 46.9%,χ ²=17.686,P=0.001)。两组在急诊手术比例、预防性脑脊液引流、手术时间、术中循环管理方式、术中用血量方面差异均无统计学意义(P均>0.05)。MFS组的术后30 d死亡率明显低于非MFS组(0比9.2%, {\chi }_{\log ?\mathrm{rank}}^2 =5.616,P=0.018)。两组在脊髓损伤、肾功能衰竭、呼吸并发症方面差异均无统计学意义(P均>0.05)。MFS组平均随访时间43个月(3~114 个月),5年生存率(89.2±4.6)%,非MFS组平均随访时间50个月(0~111个月),5年生存率(83.8±4.1)%,两组5年生存率差异无统计学意义( {{\chi }_{\log ?\mathrm{rank}}^2}_{} =2.719,P=0.099)。MFS组5年免于再干预率为(72.7±8.4)%,非MFS组为(95.2±2.3)%,MFS组再干预率明显高于非MFS组( {\chi }_{\log ?\mathrm{rank}}^2 =5.034,P=0.025)。 结论 MFS患者术后早期死亡率低于非MFS患者,中期生存情况与非MFS患者相似,但再干预率明显高于非MFS患者,术后密切随访非常重要。

Early and Midterm Results of Thoracoabdominal Aortic Aneurysm Repair in Patients with Marfan Syndrome

Objective To evaluate the early and midterm results of surgical repair of thoracoabdominal aortic aneurysm(TAAA)in patients with Marfan syndrome(MFS). Methods The clinical data of patients with MFS undergoing TAAA repair in Fuwai Hospital between January 2009 and December 2017 were retrospectively analyzed.These patients were divided into two groups:MFS group(n=58)and non-MFS group(n=98).The baseline data,early postoperative results,and midterm follow-up outcomes were compared between these two groups. Results MFS patients were significantly younger(32 years old vs. 45 years old,t=9.603,P=0.000)and more frequently had a history of aortic aneurysm or dissection(19% vs. 0,χ ²=19.996,P=0.000)than non-MFS patients.However,the proportions of males and smokers were significantly lower when compared with non-MFS patients(55.2% vs. 80.6%,χ ²=11.489,P=0.001;13.8% vs. 46.9%,χ ²=17.686,P=0.001).There was no significant difference in proportion of emergency operation,prophylactic cerebrospinal fluid drainage,operation time,intra-operative circulation management,and intra-operative blood transfusion(all P>0.05).The 30-day mortality rate was significantly lower in MFS group than in non-MFS group(0 vs. 9.2%, {\chi }_{\log -\mathrm{rank}}^2 =5.616,P=0.018).There was no significant difference in spinal cord injury,renal failure,and respiratory complications between the two groups(all P>0.05).The mean follow-up period was 43 months(3-114 months)in MFS group and 50 months(0-111 months)in non-MFS group.The 5-year survivals rate did not significantly differ between these two groups (89.2±4.6)% for MFS group and(83.8±4.1)% for non-MFS group, {\chi }_{\log -\mathrm{rank}}^2 =2.719,P=0.099].Actuarial rates of free from re-interventions at 5 years in MFS group and non-MFS group were(72.7±8.4)% and(95.2±2.3)%,respectively.The MFS group had significantly higher re-intervention rate than the non-MFS group( {\chi }_{\log -\mathrm{rank}}^2 =5.034,P=0.025). Conclusions For patients with MFS,TAAA repair provides lower 30-day mortality and comparative middle-term survival.However,the re-intervention rate is higher among MFS patients,highlighting the importance of close follow-up.