大动脉转位并室间隔完整患儿的术前治疗策略

Preoperative management strategy for patients with transposition of the great arteries and intact ventricular septum

目的:探讨大动脉转位并室间隔完整(Transposition of the great rteries-intactventriculon septum,TGA-IVS)患儿的术前诊治。方法:收集28例确诊为TGA-IVS病例的完整临床资料,分析患儿的主要临床表现,确诊依据,术前治疗方案,术前死亡率、并发症等相关资料。结果:本组病例男19例,女9例,平均妊娠年龄38+4周,平均产重3.244kg。产前诊断7例(25%),产后诊断21例(75%),产后平均确诊年龄为45h,均不伴有染色体异常及心外畸形。所有患儿均以紫绀为最突出和首要的临床表现。一旦经超声心动图检查确诊或高度疑诊,立即给予前列腺素E(2PGE2)持续滴注,其中25例(89%)有效的维持了动脉导管的开放,并于确诊后成功施行了球囊房间隔造口术(BAS),BAS时患儿的平均年龄为21h,平均血氧浓度从BAS前的(56±26)%提高至BAS后的(82±19)%(P<0.05),术前死亡及严重并发症各1例(3.5%),其中9例(32%)病情稳定的患儿回家观察,等待手术。结论:本组病例术前100%采用了PGE2及BAS治疗,其应用安全、有效,能显著提高血氧浓度,降低术前并发症及死亡率,及时挽救患儿的生命,为进一步手术治疗的宝贵时机。

Objective：To assess the preoperative management strategy for patients with transposition of the great arteries and intact ventricular septum（TGA-IVS）. Methods：Twenty eight patients with TGA-IVS were reviewed. We analyzed their clinical characteristics, method of diagnosis, preoperative management and outcome, mortality rate and the complication incidence before surgery. Results： There were 19 boys and 9 girls in this group, with the mean gestational age of 38^＋4 weeks and birth weight of 3.244 kg. 7 cases （25%） were prenatal diagnosis, while the remaining 21 cases were postnatal diagnosis at the mean age of 45 hours. The karyotype was normal in those tested and no extracardiac anomalies were found. Cyanosis was the most common and critical characterization been noticed. Diagnosis generally could be confirmed by echocardiography. All patients received prostaglandin E2 （PGE2） infusion once highly suspected of or confirmed with TGA-IVS, 25 of whom （89%） effectively maintained the patency of the ductus arteriosus preoperatively and was stopped PGE2 after Balloon atrial septostomy（ BAS ）. BAS was performed successfully in all cases at the mean age of 21 hours, as judged by oxygen saturation increased from （56± 26 ）% before BAS to （82 ±19）% after BAS （P〈0.05）. Both preoperative mortality and serious morbidity were 35%. Nine patients （32%） went home during BAS and surgery. Conclusions：All cases in our group managed with PGE2 and BAS preoperatively, which proves to be safe and effective, may dramatically improve in oxygenation, reduce preoperative mortality and morbidity, support life until definitive surgery can be performed.