摘要目的 回顾性分析我院近11年来外科治疗的≤6个月小婴儿先天性心脏病(先心病)1387例,探讨其病种、手术时机和治疗观念的变迁,以期进一步提高小婴儿先心病的就诊和救治率.方法 1997年1月至2007年12月,在我院行手术治疗的≤6个月先心病患儿1387例,主要病种包括:室间隔缺损合并肺动脉高压(VSD/PH)、法洛四联症(TOF)、完全性大动脉转位(TGA)、完全性肺静脉异位引流(TAPVC)、主动脉缩窄或主动脉弓中断合并室间隔缺损[CoA(IAA)/VSD]、右心室双出口(DORV)、合并室间隔缺损的肺动脉闭锁(PA/VSD)、室间隔完整的肺动脉闭锁(PA/IVS)等,根据病情采取相应的手术方法矫治,部分复杂型先心病进行了随访.结果 手术死亡110例,总手术死亡率7.9%.从历年手术治疗分析,手术死亡率1997至2003年为11.5%～14.4%,2004至2005年降至8.6%～&9%,2006至2007年降至3.3%～3.8%.对TGA、TAPVC、TOF、PA/VSD、PA/IVS患儿进行了随访,随访率分别为83.8%(98/117)、87.8%(79/90)、48.2%(68/141)、65%(13/20)和95%(19/20),随访期限为3～86个月.晚期死亡16例.随访中绝大多数患儿无症状,心功能和生长发育正常.结论 绝大部分早期出现症状的危重先心病可以在小婴儿期进行矫治,手术效果接近国际水平.不能进行一期矫治的可以先做姑息手术,改善缺氧、促进肺动脉发育,为以后的根治手术创造条件.

abstractsObjective To summarize and review the result of surgical repair of congenital heart disease in infants under 6 months of age.Methods Between January 1997 and December 2007,1387 infants under 6 months of age with congenital heart disease were operated on.There were 675 cases with ventricular septal defect with pulmonary hypertension (VSD/PH),138 with complete transposition of the great arteries (TGA),155 with tetralogy of Fallot (TOF),111 with totally abnormal pulmonary venous connection (TAPVC),54 with coarctation of aorta or interrupted aortic arch with ventricular septal defect [CoA(IAA)/VSD],46 with double outlet right ventricle (DORV),25 with pulmonary atresia with ventricular septal defect (PA/VSD),24 with pulmonary atresia with intact interventricular septum (PA/IVS) and so on.The operative procedure was dependent on different diseases.Follow-up has been conducted in patients with some complex congenital heart diseases.Results In the recent 11 years,the number of surgical repair in infants under 6 months of age,including neonates,with congenital heart disease has been increased.In contrast to the early phase when ventricular septal defect was the major disease treated with surgery,infants with complex congenital heart disease account for half of all cases treated with surgery now.In the meantime,the surgical mortality has been decreased year after year.There were 110 deaths in our group and the total mortality was 7.9% (110/1387).With improvement of surgical procedure,the mortality was decreased from 11.5%-14.4% in 1997 -2003 to 8.6%-8.9% in 2004-2005 to 3.3%-3.8% in 2006-2007.Follow-up data were available for 98 patients in TGA (83.8%,98/117),79 in TAPVC (87.8%,79/90),68 in TOF (48.2%,68/141),13 in PA/VSD (65%,13/20) and 19 in PAA/IVS (95%,19/20).The duration of follow-up ranged from 3 to 86 months.There were 16 late deaths,4 in TGA,10 in TAPVC and 2 in PA/VSD patients.The majority were asymptomatic on follow-up.Mild residual obstruction was seen in 4 cases with TAPVC.Pulmonary hypertension was seen in 5 cases with TAPVC.Mild aortal valve regurgitation,pulmonary valve regurgitation and tricuspid valve regurgitation were seen in 23 eases with TGA (23.5%,23/98).Some PA/VSD patients had second operation.Conclusions Most symptomatic neonates and infants younger than 6 months with critical congenital heart defects can undergo corrective operation under acceptable risk.Due to improvements in perioperative,anaesthetic,surgical,and postoperative care,contemporary hospital mortality can be reduced to 3.3%-3.8%.Palliative procedures still play an important role in the staged treatment of severe complex heart defects in neonates and infants younger than 6 months of age.