Serraf A, Bruniaux J, Lacour-Gayet F, Sidi D, Kachaner J, Bouchart F, Planche C
Department of Pediatric Cardiac Surgery, Marie-Lannelongue Hospital, Le Plessis Robinson, France.
J Thorac Cardiovasc Surg. 1991 Jul;102(1):140-7.
One hundred eighteen patients, 100 with transposition of the great arteries plus ventricular septal defect and 18 with double-outlet right ventricle and subpulmonary ventricular septal defect have undergone arterial switch and patch closure of the ventricular septal defect since February 1983. In transposition of the great arteries the ventricular septal defect was perimembranous in 70 cases, trabecular in 28, and infundibular in 10. Eleven patients had multiple ventricular septal defects. In addition to 18 patients with double-outlet right ventricle, malalignment of the conal septum was present in 19 cases. Coronary type A distribution was recognized in 79 cases, type C in one, type D in 24, and type E in 14. Great arteries were side by side in 19% of cases. Aortic coarctation was present in 31 cases, and subaortic obstruction in 9. Age at operation ranged from 4 days to 4 years (mean, 3.5 +/- 8.3 months), and mean weight was 4.0 +/- 1.6 kg. Thirty-seven infants were younger than age 1 month. Thirty-six patients underwent previous operations: pulmonary artery banding alone (n = 13), pulmonary artery banding and coarctation repair (n = 13), and coarctation repair alone (n = 10). Mean time between the first procedure and the switch was 2.2 months. Six patients with aortic coarctation underwent one-stage repair, through median sternotomy, aortic reconstruction, closure of the ventricular septal defect, and arterial switch. Perioperative mortality was 13.5% (70% confidence limit 10% to 17.6%, n = 16). It was directly related to coronary artery kinking in 50% of deaths and to anatomy and size discrepancy of the great arteries in the remaining deaths. Univariate analysis could not find any significant risk factor of in-hospital mortality. Mean follow-up of 30.3 +/- 23.5 months was achieved in all but 2 survivors. There was one late death. Ten patients underwent 11 reoperations for recoarctation (n = 1), pulmonary stenosis (n = 7), residual ventricular septal defect (n = 2), and stenosis of superior vena cava (n = 1). Two patients needed a permanent pacemaker. Actuarial survival and freedom from reoperation at 5 years were 84.5% +/- 3.6% and 85.7% +/- 4.6%, respectively. We conclude that anatomic correction of complex transposition is a safe method that offers good early and midterm results.
自1983年2月以来，118例患者接受了动脉调转术及室间隔缺损修补术，其中100例为大动脉转位合并室间隔缺损，18例为右心室双出口合并肺动脉下室间隔缺损。在大动脉转位患者中，70例室间隔缺损为膜周部，28例为小梁部，10例为漏斗部。11例患者有多个室间隔缺损。除18例右心室双出口患者外，19例存在圆锥间隔对位不良。79例为冠状动脉A型分布，1例为C型，24例为D型，14例为E型。19%的病例中，大动脉呈并列关系。31例存在主动脉缩窄，9例存在主动脉下梗阻。手术年龄为4天至4岁（平均3.5±8.3个月），平均体重为4.0±1.6kg。37例婴儿年龄小于1个月。36例患者曾接受过手术：单纯肺动脉环缩术（n = 13）、肺动脉环缩术及主动脉缩窄修复术（n = 13）、单纯主动脉缩窄修复术（n = 10）。首次手术与动脉调转术之间的平均时间为2.2个月。6例主动脉缩窄患者通过正中胸骨切开术、主动脉重建、室间隔缺损修补及动脉调转术进行了一期修复。围手术期死亡率为13.5%（7%置信区间为10%至17.6%，n = 16）。50%的死亡与冠状动脉扭曲直接相关，其余死亡与大动脉的解剖及大小差异有关。单因素分析未发现任何与院内死亡率相关的显著危险因素。除2例幸存者外，所有患者的平均随访时间为30.3±23.5个月。有1例晚期死亡。10例患者因再发主动脉缩窄（n = 1）、肺动脉狭窄（n = 7）、残余室间隔缺损（n = 2）及上腔静脉狭窄（n = 1）接受了11次再次手术。2例患者需要植入永久性起搏器。5年时的实际生存率及免于再次手术率分别为84.5%±3.6%和85.7%±4.6%。我们得出结论，复杂型大动脉转位的解剖矫治是一种安全的方法，可提供良好的早期和中期结果。
