婴儿主动脉弓阻塞病变的外科治疗

目的 总结8年来56例婴儿主动脉弓阻塞病变的外科治疗经验.方法 2001年5月至2009年6月,本院采取经正中切口一期矫治婴儿主动脉弓阻塞病变56例,其中男32例,女24例;年龄16 d至11个月;体重2.3～7.3 kg;主动脉弓中断A型22例,B型3例;主动脉缩窄31例.合并室间隔缺损（VSD）及动脉导管未闭（PDA）55例,房间隔缺损（ASD）6例,右室双出口（DORV）1例,完全性大动脉转位（TGA）1例.均采取经胸骨正中切口深低温停循环下一期矫治手术,采用降主动脉与主动脉弓端端吻合41例,端侧吻合前壁自体心包片加宽弓成形15例.对合并圆锥部VSD者,为避免远期左室流出道梗阻采用0.4 mm Gore-Tex补片Luciani法修补VSD.结果 围术期死亡2例,病死率3.57%;出现喉返神经损伤2例,跨主动脉弓压差3例,均＜20 mmHg.54例存活患儿随访4个月至8年,生长发育良好,无远期主动脉狭窄及左室流出道梗阻,无神经系统并发症.多普勒超声测定跨修复点压力阶差（1.5±0.7）kPa,较术前（6.7±2.3）kPa,显著降低（t=2.51,P＜0.05）.结论 婴儿主动脉弓阻塞合并心内畸形宜早期手术治疗,经胸骨正中切口一期矫治手术安全有效.

Surgical management of aortic arch obstruction associated with cardiac anomalies in infants

Objective To summarize the experience of one-stage repair of aortic arch obstruction associated with cardiac anomalies. Methods Between January 2001 and June 2009, 56 infant cardiac patients underwent one-stage correction of interrupted aortic arch （ AA, 25 cases） and eoarctation of the aorta （ COA, 31 cases）. All procedures were median sternotomy under eardiopulmonary bypass. The aortic arch reconstruction were end-to-end anastomosis between the descending aorta and the arch in 41, end-to-side anastomosis in 15, and ventricular septal defect was repaired with Gore-Tex patch. Results There were ：2 death （ overall mortality 3.57% ） . The recurrent laryngeal nerves injuries in 2, the peak-to-peak systolic pressure of aortic arch in 3（ all lower than 20mmHg）. All survivors were followed up for 4 month to 8 year. They were asymptomatic and developing normally. No later re-stenosis of anastomosis occurred and severe neurological complications were found. Conclusion One-stage complete correction of IAA and COA with cardiac anomalies through median sternotomy yields excellent intermediate surgical results.