大于6月龄伴室间隔缺损并重度肺动脉高压心室大动脉连接异常的大动脉调转术

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.

The arterial switch operation for discordant arterioventricular connection with severe pulmonary hypertension:experience with 86 cases aged 7 months to 19 years

Objective 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.