摘要：
目的 探讨主动脉弓中断(IAA)患者接受双心室外科治疗的早中期结果.方法 回顾性分析2009年6月至2017年6月广东省人民医院心外科收治的主要诊断包含IAA的68例双心室结构患者的临床资料.手术年龄8d至18岁,包括新生儿28例(41.2％),年龄＜1岁32例(47.1％),年龄1～15岁6例(8.8％),年龄＞15岁2例(2.9％).IAA分型A型46例(67.6％),B型22例(32.3％),无C型病例.5例不合并心内畸形的患者中,4例采用左胸后外侧切口入路行一期矫治,1例采用胸部正中切口经右股动脉-右心房插管心肺转流下完成降主动脉-主动脉弓端侧吻合术.63例合并心内畸形患者中,2例仅行经左胸后外侧切口下主动脉弓重建,2例患者实施分期手术,其余59例患者实施深低温停循环下一期IAA矫治+心内畸形矫治,其中43例行选择性脑灌注.术中弓部重建方式包括端端吻合或端侧吻合55例,自体肺动脉管道连接6例,自体肺动脉补片+主动脉弓-降主动脉吻合5例,左锁骨下动脉垂片成形1例,牛心包管道连接1例,D.K.S.术1例,左心室双通道1例.结果 随访时间1～9年(中位随访时间为6年),4例(5.9％)死于循环系统疾病:术后30 d内死亡3例,包括新生儿1例,1～12月龄患者2例;中远期死亡1例(术后8个月死于心功能不全).术后早期残余主动脉弓梗阻(压差＞30 mmHg,1 mmHg=0.133 kPa)者11例,中度以上者3例;随访至术后5年时残余梗阻3例,中度以上者2例.存在主动脉瓣及瓣下梗阻(压差＞30 mmHg)者3例;随访至5年时残余梗阻3例,中度以上2例.目前共5例患者实施6次心血管系统再干预手术:3次因肺动脉狭窄,2次因主动脉弓严重狭窄,1次因主动脉瓣下严重梗阻.结论 IAA在新生儿、婴幼儿期完成一期手术矫治是安全的,临床疗效良好,但远期仍面临再梗阻,需再次手术干预.对于部分患者需根据具体情况选择主动脉弓重建方式.%Objective To analyze the early and midterm results of surgical treatment of interrupted aortic arch (IAA) with double-ventricular procedure.Methods The data of the 68 cases with the main diagnosis of IAA with biventricular structure from June 2009 to June 2017 at Department of Cardiac Surgery,Guangdong General Hospital was collected,including 46 cases of type A,22 cases of type B.There was no type C case.Except for 5 cases without patent ductus arteriosus or other intracardiac malformations,the remaining 63 cases were combined with intracardiac deformity.The age of operation was 8 days to 18 years,including 28 cases(41.2％) of newborns,32 cases(47.1％) within 1 year old,6 cases(8.8％) from 1 to 15 years old,and 2 cases(2.9％) above 15 years old.Of the 5 patients with no intracardiac malformations,4 patients were treated with a left-posterior thoracic lateral incision for primary surgical correction with endto-side anastomosis,and 1 adult patient was treated with a median sternotomy incision to complete the anastomosis with the assist of extracorporeal circulation.Of the 63 patients with intracardiac malformation,2 underwent reconstruction of the aortic arch via posterolateral left thoracic incision merely;2 patients underwent staged surgery to correction the IAA and intracardiac malformation;the remaining 59 patients underwent the one-stage procedure of IAA and intracardiac malformation correction with deep hypothermic circulatory arrest and 43 cases of selective cerebral perfusion.A total of 55 cases of end-to-end or end-to-side anastomosis were performed in all aortic arch reconstruction.There were 6 cases of connection the arch to descending aorta with autogenous pulmonary artery conduit,and 1 case with homemade bovine pericardial conduit.And there were 5 cases of anastomosis enlargement with autogenous pulmonary artery patch,and 1 case with left subclavian artery flap patch.There were 1 case performed with D.K.S.operation for the severe hypogenetic aortic valve and ascending aorta,and 1 case with left ventricular double outlet channel operation for the severe subvalvular aortic stenosis.Results Follow-up to 9 years,4 deaths (5.9％)related to circulatory system diseases:3 cases died within 30 days after surgery (1 case was neonatal,2 cases were 1 to 12 months old);one died 8 months after surgery for low cardiac output syndrome.In the early postoperative period,11 cases (16.2％) of residual aortic arch obstruction (pressure gradient ＞30 mmHg) were observed,of which 3 cases were moderate (4.4％);follow-up to 5 years after operation was residual obstruction in 3 cases and moderate in 2 cases.There were 3 cases with aortic valve and subvalvular obstruction (differential pressure ＞30 mmHg),3 cases were followed up to 5 years after aortic valve and subvalvular obstruction,and 2 cases were moderate or above.To date,5 patients have undergone 6 reoperations of the cardiovascular system:3 cases because of pulmonary artery stenosis,2 cases because of severe aortic arch stenosis,and 1 case because of aortic subvalvular obstruction.Conclusions One-stage surgical repair of IAA is safe and clinically effective in the neonatal and infant.But some patients still requires reoperation for re-obstruction from subvalvular or aorta arch.For some patients,the choice of aortic arch reconstruction depends on the specific situation.
