摘要目的 总结大动脉调转术(ASO)根治>6月龄伴室间隔缺损(VSD)并重度肺动脉高压(PH)心室大动脉连接异常者手术疗效,探讨ASO适应证.方法 2000年5月至2008年10月治疗86例VSD并PH的心室大动脉连接异常者.男51例,女35例;年龄7月龄～19岁,平均(24±22)月龄;体重2.6～48.0 kg,平均(9.0±7.0)kg.肺动脉平均压50.0～97.0(64.9±13.0)mm Hg,肺血管阻力46.0～1261.9(324.0±249.0)dyn·s·cm ~(-5).手术在全麻低温(18～22℃)低流量(50 ml·kg~(-1)·min~(-1)体外循环下完成,同期矫治合并畸形.结果 手术死亡率7.0%(6/86例),均为2005年12月以前病例(40例),其中仅1例7岁病儿死因与PH有关;2006年1月以后连续46例无死亡.随访1～84个月,80例生存.2例(2岁及13岁)分别于术后2个月和1年半不明原因猝死,余78例心功能明显改善,无再手术及并发症.结论 年龄>6月龄并重度PH的TGA/VSD或TBA,如一般状况尚好,经皮血氧饱和度>0.60,X线胸片示两肺血尚多,肺血管阻力<1200 dyn·8·cm~(-5),ASO根治仍可取得满意效果,扩大了根治性ASO适应证.

abstractsObjective To summarize the experience in radical arterial switch operation (ASO) for discordant arterioventricular connection with severe pulmonary hypertension in children older than 6 months and investigate its indication. Methods 86 consecutive patients suffering from transposition of great arteries (TGA) or Taussing-Bing anomaly, with severe pulmonary arterial hypertension,51 male and 35 female,aged 7 months to 19 years[(24 ±22)]months (among 86 patients,28patients aged 7-12 months, 33 patients aged 1-3 years, 17 patients aged 4-6 years, 4 patients aged 7-12 years, 4 patients aged 13-19 years), with body weight of 2.6-48.0 (9.0 ±7.0)kg, mean pulmonary pressure of 50.0-97.0 (64.9 ± 13.0)mm-Hg, pulmonary vascular resistance of 46.0-1261.9 dyn · s · cm~(-5), underwent ASO under general anesthesia and extracorpo-real circulation with low temperature (18-22t )and low volume blood flow (50 ml · kg~(-1) · min~(-1). The complicated anomalies were corrected simultaneously. Results The total operative mortality was 7.0% (6/86). From May 2000 through December 2005, 40 ASO were performed with operative mortality of 15.0% (6/40). While from January 2006 through October 2008, consecutive 46 ASO were performed with no death. 1 patient died of pulmonary hypertension crises, and 5 patients died of causes un-related to PH. Follow-up of 1 to 84 months showed that 2 patients died of sudden death with unknown reason 2 months and 18 months postoperatively respectively, and the other 78 patients survived with significantly improved cardiac function , with no complication and reoperation. Conclusion Radical ASO can still realize satisfactory early and mid-term results for patients older than 6 months suffering from discordant arterioventricular connection with severe PH provided they are in a rather good condition without severe cyanosis with transcutaneous oxygen saturation >0.60 and X-ray chest film shows acceptable blood flow and pulmonary vascular resistance is less than 1200 dyn · s · cm~(-5). Our study broadens the indication of radical ASO.