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,χ2=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%,χ2=11.489,P=0.001;13.8% vs. 46.9%,χ2=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%, [Formula: see text]=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.