“绿色通道”救治新生儿室间隔完整型完全性大动脉转位

Experience in treatment of neonatal complete transposition of great arteries with intact ventricular septum through "green channel"

目的:回顾性总结经先天性心脏病(先心病)三级预防网络"绿色通道"救治新生儿室间隔完整型完全性大动脉转位的临床经验。方法:收集2010年1月至2012年12月,经"绿色通道"转诊至我院的8例室间隔完整型完全性大动脉转位新生儿临床资料,总结分析临床疗效及转归。结果:8例患儿,男性5例,女性3例。入院年龄2～12 d,平均(5.9±2.7)d,手术日龄2～14 d,平均(8.1±2.6)d,体质量2.3～7.5 kg,平均(3.5±0.6)kg。术前严重酸中毒,呼吸衰竭1例行气管插管治疗,1例合并肾衰竭,所有患儿均直接行(arterial switch operation,ASO),手术成功率100%。术后延迟关胸5例,围术期死亡1例(12.5%),死因为多脏器功能衰竭。术后3～6个月,2次随访。手术治愈的7例患儿生长发育良好,左心室心功能正常范围,平均左心室射血分数(LVEF)分别为(67.0±2.8)%和(70.3±4.0)%,左心室心肌质量指数也显著增高。结论:完全性大动脉转位患儿应尽早诊断,提高产前诊断率加强术前内科治疗,纠正代谢紊乱,维护心功能,把握手术时机,尽快根治是成功的关键。通过先心病三级预防框架实施"绿色通道"救治室间隔完整型完全性大动脉转位,对提高危重患儿存活率,减少新生儿死亡有重要意义。

Objective：Retrospective study on clinical treatment of neonatal complete transposition of great arteries with intact ventricular septum.Methods： From January 2010 to December 2012,new-born having complete transposition of great arteries with intact ventricular septum（TGA/IVS） were transferred to our hospital for arterial switch operation（ASO） through the ＂green channel＂ of tertiary prevention network.Patients＇ clinical data and echocardiographic measurements were analyzed.The effect of ASO and operative outcome were evaluated.Results： Totally 8 patients,including 5 male and 3 female received ASO in this study,with mean weight of 2.3-7.5（3.5 ± 0.6） kg.The average age on admission was 2-12（5.9±2.7）d and mean age at operation was 2-14（8.1±2.6）d.Before surgery,all had low blood oxygen saturation,with average oxygen saturation was（68.1±19.7） %.One premature baby was combined with renal failure,another baby was treated by endotracheal intubation for his severe acidosis and respiratory failure.All patients underwent one step of ASO with success rate of 100%.5 cases had postoperative delayed sternal closure for postoperative myocardial edema.One died of disseminated intravascular coagulation（DIC） and multiple organs dysfunction syndromes（MODS） 10 d after surgery,made the perioperative mortality of 12.5%.Postoperative follow-up was performed in 7 survived at 3 and 6 months interval after ASO.All had good recovery with normal oxygen saturation and good cardiac function.Average left ventricular ejection fraction（LVEF %） of the 7 survived was（67.0±2.8） % at 3 and（70.3±4.0） % at 6 months post ASO,and LVMI increased markedly.Conclusion： Complete transposition of great arteries with intact ventricular septum should be diagnosed prenatally or early after birth.Active medical treatment of low oxygen saturation,severe metabolic disorders and maintenance of heart function before surgery contribute to improvement of the survival rate in new born with TGA/IVS.