摘要：
目的 探讨应用主动脉全弓替换结加支架象鼻手术(孙氏手术)治疗Bentall术后未手术部位新发A型主动脉夹层累及主动脉弓病变的中远期预后.方法 回顾分析2009年2月至2016年2月141例Stanford A型主动脉夹层累及弓部病变的Mar fan综合征(MFS)患者资料,11例(7.8％)初次因主动脉根部瘤行Bental1手术,其中男10例,年龄(43.2±8.6)岁(26～ 50岁),急诊手术8例,高血压病史4例,家族发病史7例,均行孙氏手术治疗.Bentall手术至孙氏手术时间间隔(11.3±5.8)年.依据术前降主动脉近端直径是否大于＞ 40 mm分两组.收集所有患者围手术期临床资料和主动脉CTA影像学资料,分析孙氏手术后远端主动脉假腔血栓化程度、主动脉直径变化,远端主动脉扩张和再次手术等早期和中远期预后.结果 11例Bentall术后合并Stanford A型主动脉夹层MFS患者均成功行孙氏手术治疗,无脊髓损伤.术前降主动脉近端直径＞ 40 mm组患者住院死亡3例,其中1例手术时间长,肺实变合并多脏器功能衰竭;1例围手术期脑梗塞并脑出血;1例远端主动脉破裂.孙氏手术后随访(5.3±2.0)年(2.1～8.1年),随访率100％.远端主动脉假腔血栓化率在支架象鼻近端周围达100％,非支架降主动脉段达75.0％;支架近端无内漏,无远端主动脉破裂.3例远端主动脉缓慢扩张,最大直径达50 mm,拟择期行胸腹主动脉替换手术(TAAAR)术.晚期非心脏原因死亡2例,无再次手术.术前降主动脉近端直径＞ 40 mm组与≤40 mm组相比,FET支架段降主动脉重塑不明显,差异有统计学意义(P=0.01),在非支架降主动脉段,膈肌和肾动脉水平两组直径差异无统计学意义.Kaplan-Meier生存分析显示,孙氏手术后1个月、1年和6年生存率分别为81.8％,72.7％和63.6％.结论 孙氏手术治疗Bentall术后远端未手术部分新发Stanford A型主动脉夹层累及弓部病变的MFS患者早期死亡比例较高,中长期预后好.术前降主动脉近端直径大于40 mm是早期死亡、远端主动脉扩张和再次手术等主动脉不利事件的重要危险因素.%Objective Stanford type A aortic dissection(TAAD) involving aortic arch in following prior Bentall procedure in patients with Madfan syndrome(MFS) is uncommon.The purpose of the study was to assess the early and long-term outcomes of this kind of patients underwent total arch replacement and frozen elephant trunk (TAR + FET).Methods Between February 2009 and February 2016,141 patients with Marfan syndrome(confirmed by revised Ghent Criteria) underwent TAR + FET for TAAD,of those 11 (7.8 ％) patients (8 acute) following a prior Bentall procedure and without dissection in distal aorta.Mean age at FET was(43.2 ± 8.6) years and 10 were males.Hypertension was seen in 4 (36.4％) and family history was seen in 7 (63.6％).The interval from Bentall procedure to FET averaged (11.3 ± 5.8) years.Two groups were segregated by the maximal diameter of descending aorta more than 40 mm or not.The early and long-term outcomes were analyzed and risk factors identified for late adverse events.Results Operative mortality was 27.3 ％ (3/11).No spinal cord injury occurred.The cause of death was long time of surgery,multiorgan failure,stroke and cerebral hemorrhage and rupture of distal aorta.Follow-up was complete in 100％,averaging(5.3-± 2.0)years(range 2.1-8.1 years).Obliteration of the false lumen was seen in 100％ across the proximal FET and 75.0％ in the unstented descending aorta.Distal aortic dilation occurred in 3 patients which were waiting open thoracoabdominal aortic repair(TAAAR) but no reoperation yet.Of those 8 patients,2 died of non-cardiac reason.The DMax of FET segment has significant grow in DA more than 40ram group than less than 40mm group (P =0.01).Another segment of distal aorta has no significant different.Survival were 81.8％,72.7％ and 63.6％ at 1 month,1 year and 6 years after surgery.Conclusion Using Sun's procedure for Marfan patients for type A aortic dissection involving aortic arch following previous Bentall procedure was safe and technically feasible with good long-term outcomes,but higher early death.The DMax of descending aorta more than 40mm was at higher risk for early death,late distal aortic dilation,and reoperation.
