Li Ning, Zhang Yu, Gao Yuan, Bai Yifan, An Zhao, Zhang Guanxin, Han Qingqi, Lu Fanglin, Li BaiLing, Han Lin, Xu Zhiyun
Department of Cardiothoracic Surgery, Changhai Hospital, Second Military Medical University, Shanghai 200433, China.
J Thorac Dis. 2019 Dec;11(12):4951-4959. doi: 10.21037/jtd.2019.12.21.
Data on outcome of Stanford type A aortic dissection (TAAD) in Marfan syndrome (MFS) patients are limited. We investigated the full spectrum of reoperation and survival after initial surgery in MFS patients who suffered TAAD.
Retrospective analysis of 85 consecutive MFS patients in one-single center during the past 15 years.
Overall, 85 MFS patients with TAAD underwent surgical repair [74% acute dissections; 80% DeBakey type I; 91% composite valved graft; 70% total arch replacement (TAR); 68% frozen elephant trunk (FET); 7% in-hospital mortality] at Changhai hospital affiliated to the Second Military Medical University over the past 15 years. Five (20.8%) patients in non-TAR group need aortic arch reintervention with resternotomy during follow-up, which is significantly higher than that in TAR group (P=0.001). Freedom from aortic arch reoperation in non-TAR group was all 78.7%±8.5% at 5, 10, and 15 years. No patient required aortic arch reoperation in TAR group (P=0.001). On the other hand, the FET was inserted into false lumen intentionally at initial surgery in 2 cases of chronic TAAD with narrowed true lumen. Scheduled thoracoabdominal aortic replacement was performed 6 months later. Both 2 patients are with well clinical outcomes. At last, we found that Debakey type and TAR at initial surgery were irrelevant to survival and reoperation for descending aorta.
TAR combined with FET is recommended in MFS patients when the aortic arch is dissected or enlarged. The FET could be inserted into the false lumen intentionally in selective case for scheduled 2-staged descending aortic repair.
关于马方综合征(MFS)患者中斯坦福A型主动脉夹层(TAAD)的预后数据有限。我们调查了发生TAAD的MFS患者初次手术后再次手术及生存的全貌。
对过去15年中同一单中心的85例连续MFS患者进行回顾性分析。
总体而言,过去15年中,85例患有TAAD的MFS患者在第二军医大学附属长海医院接受了手术修复[74%为急性夹层;80%为DeBakey I型;91%使用复合带瓣移植物;70%进行全弓置换(TAR);68%使用冰冻象鼻技术(FET);7%院内死亡率]。非TAR组中有5例(20.8%)患者在随访期间需要通过再次开胸进行主动脉弓再次干预,这显著高于TAR组(P = 0.001)。非TAR组在5年、10年和15年时无主动脉弓再次手术的生存率均为78.7%±8.5%。TAR组无患者需要进行主动脉弓再次手术(P = 0.001)。另一方面,在2例真腔狭窄的慢性TAAD患者中,初次手术时将FET有意植入假腔。6个月后进行了计划性胸腹主动脉置换。2例患者临床结局均良好。最后,我们发现初次手术时的DeBakey分型和TAR与降主动脉的生存及再次手术无关。
当主动脉弓发生夹层或扩大时,建议在MFS患者中采用TAR联合FET。在选择性病例中,可有意将FET植入假腔以进行计划性两阶段降主动脉修复。