新生儿复杂性先天性心脏病29例的外科治疗及围术期处理

Surgical therapy and peri-operative management for complicated congenital heart disease in 29 neonates

目的总结新生儿复杂性先天性心脏病的外科治疗效果及围术期处理体会。方法2000-01～2004-01我院共收治先心病新生儿29例,其中男22例,女7例。年龄11h至28d,其中11～72h者6例,3～10d者9例,10～28d者14例;体重2.2～4.3kg,其中2.2～3kg者7例,3.1～4kg者21例,>4kg者1例。术前诊断包括完全性大动脉错位(D-TGA)14例,其中室间隔完整性9例,合并室间隔缺损者5例;完全性肺静脉异位引流(TAPVC)6例,其中心上型3例,心内型1例,心下型2例;法乐四联症合并肺动脉闭锁5例,其中Ⅰ型4例,Ⅱ型1例;永存动脉干4例,其中Ⅰ型3例,Ⅱ型1例。全组均一期根治,其中急诊手术26例,11例深低温停循环。结果本组早期(术后30d)死亡3例(10.3%),其中第一例D-TGA因重建主动脉吻合口出血于术后12h死亡,1例D-TGA和1例TAPVC术后重度低心输出量(低心排)合并肾功能衰竭分别于术后5、9d死亡。术后主要并发症包括低心排5例(17.2%),肾功能衰竭5例(17.2%),低氧血症3例(10.3%)。26例存活者随访2～48个月,发绀均消失,体重明显增加,无远期死亡及并发症。结论新生儿复杂性先天性心脏病特别是心衰严重和(或)重度低氧血症者,一经确诊应及时手术,效果满意。精心的手术设计、术后预防及积极治疗低心排和(或)肾功能衰竭是降低术后病死率的关键,包括改良超滤。

Objective To assess the effect of surgical treatment of complex congenital heart disease of neonate and summarize the experience of peri-operative management.Methods Twenty-nine neonates(male 22, female 7) who had complex congenital heart disease were admitted and operated on in our hospital from Jan, 2000 to Jan, 2004. Their ages ranged from 11 hours to 28 days(mean 11.7±7.8days), among them there were 6 aged from 11 to 72 hours, 9 aged from 3 to 10 days and 14 aged from 10 to 28 days. Their body weight ranged from 2.2 to 4.3 (mean 3.2±0.4)kg, among them there were 7 weighted from 2.2 to 3 kg, 21 weighted from 3.1 to 4 kg and 4 weighted more than 4 kg. Their preoperative diagnosis was as follows: 14 were complete transposition of the great vessels(D-TGA), among them there were 5 associated with ventricular septal defect, 9 with intact interventricular septum;6 were total anomalous pulmonary venous return(TAPVC), among them 3 were type of supercardiac, 1 was intracardiac and 2 were subcardiac; 5 neonates were pulmonary artery atresia, among them there were 4 associated with ventricular septal defect and 1 with intact interventricular septum; 4 neonates were persistent truncus arteriosus, among them 3 were typeⅠand 1 was typeⅡ. Radical operations, including emergency operations in 26 cases and selective operations in 2 cases, were performed on all these neonates. In above operations, 11 cases under deep hypothermia circulation arrest.Results Three cases death(10.3%) occurred in earlier postoperative period(within 30 days), among whom the first case with D-TGA died from reconstructive aortic stoma bleeding within 12 hours after operation, and second with D-TGA and third with TAPVC died from severe low cardiac output syndrome complicated with renal failure later 5 days and 9 days after operation, respectively. The main postoperative complications were low cardiac output(5, 17.2%), renal failure(5, 17.2%) and hypoxemia(3, 10.3%). In the follow-up duration ranged from 2 to 48 months, the 26 survivors had no cyanosis with body weight increased significantly. No long-term death and complication occurred. Conclusion Neonates who had complicated with congenital heart disease, especially with severe heart failure and/or hypoxemia, should be operated on when diagnosed definitively. The effect could be satisfactory. Exact design and prevention and treatment for low cardiac output and renal failure, including modified ultrafiltration, delay of suturing the thoracic cavity and peritoneal dialysis are key points to reduce the postoperative death rate.