摘要目的 总结73例小儿主动脉弓阻塞性病变合并心内畸形的手术方法和临床经验.方法 2010年3月至2014年3月,手术治疗小儿主动脉弓阻塞性病变合并心内畸形73例.男48例,女25例;年龄ld至6.5岁,平均(10.5±7.3)个月;体质量3.0～14.0(7.0 ±3.2)kg;主动脉缩窄(CoA) 68例,主动脉弓中断(IAA)3例,双主动脉弓合并CoA 2例.合并的心内畸形包括:室间隔缺损(VSD) 65例、房间隔缺损(ASD) 22例、动脉导管未闭(PDA) 36例、完全性大血管转位(TGA)1例、法洛氏四联症(TOF)2例、完全性肺静脉异位引流(TAPVC)1例、右肺动脉狭窄1例、右心室双出口(DORV)1例、二尖瓣狭窄+主动脉瓣狭窄(即Shone's综合征)1例.经胸骨正中切口一期手术矫治72例,分期手术1例,一期手术者均应用深低温低流量选择性脑灌注体外循环技术.行降主动脉与主动脉弓端端吻合42例,自体肺动脉补片扩大成形术9例,降主动脉与升主动脉端侧吻合术22例.根据不同的心内合并畸形也予以相应矫治.结果 手术死亡2例,手术死亡率2.7％;术后因肾功能不全行腹膜透析2例;存活患儿随访3 ～36个月,生长发育良好,无神经系统并发症;吻合口再狭窄1例,经再次手术治愈.结论 小儿主动脉弓阻塞性病变合并心内畸形宜及早手术,经正中切口一期矫治手术安全、有效.

abstractsObjective To summarize the clinical experience in repair of aortic arch obstruction associated with intracardiac anomalies in children retrospectively.Methods From March 2010 to March 2014,73 children diagnosed as coarctation of the aorta (CoA,n =68),interrupted aortic arch (IAA,n =3),and double aortic arch with CoA (n =2) underwent surgical management.Six of them were complicated with complex intracardiac anmalies,including tetralogy of Fallot (TOF,n =2),transposition of great arteries (TGA,n =1),total anomalous pulmonary venous connection (TAPVC,n =1),double outlet of right ventricle (DORV,n =1),and Shone's syndrome (n =1) ; the rest 67 patients were associated with ventricular septal defect (VSD) and other simple anomalies.Twenty eight cases had hypoplasia of the aortic arch.All the patients had one-stage repair except for one.The aortic arch reconstruction was end to end anastomosis between the descending aorta and the arch in 42 patients,end to side anastomosis in 22,and the aortic arch were enlarged using autologous pulmonary artery patch in 9.The associated intracardiac anomalies were repaired in the same stage.Results There were 2 deaths.The operative mortality was 2.7％.Renal failure was occurred in 2 cases who were cured afterwards by peritoneal dialysis.All survivors were followed up for 3 ～ 36 months,anastomotic restenosis was found in 1 case who underwent reoperation 14 months after the first operation.No neurological complications were occurred.Conclusions One-stage complete correction of CoA and IAA with intracardiac anomalies through median sternotomy can achieve excellent short-and mid-term surgical results.