Zhang Hui-Li, Li Shou-Jun, Hu Sheng-Shou, Liu Ying-Long, Shen Xiang-Dong, Yan Jun
Department of Cardiac Surgery, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China.
Zhonghua Er Ke Za Zhi. 2009 Apr;47(4):250-4.
To summarize and review the result of surgical repair of congenital heart disease in infants under 6 months of age.
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.
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 cases with TGA (23.5%, 23/98). Some PA/VSD patients had second operation.
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.
总结并回顾6个月以下婴儿先天性心脏病的手术修复结果。
1997年1月至2007年12月期间，对1387例6个月以下的先天性心脏病婴儿进行了手术。其中室间隔缺损合并肺动脉高压（VSD/PH）675例，完全性大动脉转位（TGA）138例，法洛四联症（TOF）155例，完全性肺静脉异位连接（TAPVC）111例，主动脉缩窄或主动脉弓中断合并室间隔缺损[CoA（IAA）/VSD]54例，右心室双出口（DORV）46例，室间隔缺损合并肺动脉闭锁（PA/VSD）25例，室间隔完整的肺动脉闭锁（PA/IVS）24例等。手术方式取决于不同疾病。对部分复杂先天性心脏病患者进行了随访。
在最近11年中，6个月以下包括新生儿在内的先天性心脏病婴儿的手术修复数量有所增加。与早期以室间隔缺损为主要手术治疗疾病相比，现在复杂先天性心脏病婴儿占所有手术治疗病例的一半。同时，手术死亡率逐年下降。本组有110例死亡，总死亡率为7.9%（110/1387）。随着手术方法的改进，死亡率从1997 - 2003年的11.5% - 14.4%降至2004 - 2005年的8.6% - 8.9%，再降至2006 - 2007年的3.3% - 3.8%。有98例TGA患者（83.8%，98/117）、79例TAPVC患者（87.8%，79/90）、68例TOF患者（48.2%，68/141）、13例PA/VSD患者（65%，13/20）和19例PAA/IVS患者（95%，19/20）有随访数据。随访时间为3至86个月。有16例晚期死亡，TGA患者4例，TAPVC患者10例，PA/VSD患者2例。大多数患者随访时无症状。4例TAPVC患者有轻度残余梗阻。5例TAPVC患者有肺动脉高压。23例TGA患者（23.5%，23/98）有轻度主动脉瓣反流、肺动脉瓣反流和三尖瓣反流。部分PA/VSD患者进行了二次手术。
大多数有症状的6个月以下新生儿和婴儿严重先天性心脏缺陷可在可接受的风险下进行矫正手术。由于围手术期、麻醉、手术和术后护理的改善，当代医院死亡率可降至3.3% - 3.8%。姑息性手术在6个月以下新生儿和婴儿严重复杂心脏缺陷的分期治疗中仍发挥重要作用。
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