摘要目的 评价伴随不同种类解剖畸形的完全性大动脉转位通过不同术式取得的治疗效果.方法 通过大动脉调转术、双向腔肺吻合术、Rastelli矫治术、Fontan术、Senning术等治疗伴随室间隔缺损、肺动脉狭窄、房间隔缺损、动脉导管未闭、下腔静脉中断、永存左上腔静脉、冠状动脉畸形、主动脉弓缩窄等畸形的完全性大动脉转位,并评价术后生存率.结果 全组275例,早期死亡14例(5.09％):大动脉调转术后死亡10例(10/243,4.12％),其余术式术后死亡4例(4/32,12.50％);生存261例,其中双向Glenn术、改良Fontan和全腔肺动脉吻合术后患儿发绀明显缓解,大动脉调转术和改良Senning术后患儿发绀消失,生长发育正常.结论 完全性大动脉转位可通过多种术式治疗,新生儿通过大动脉调转术治疗取得较好效果.

abstractsObjective To summarize the results of surgical management of different types of transposition of great arteries with different procedures.Methods Two hundred and seventy-five patients with transposition of great arteries and other congenital heart defects (ventricular septal defect,pulmonary artery stenosis,atrial septal defect,patent ductus arteriosus,interrupted inferior vana cava,persistent left superior vena cava,coronary artery anomalies and coarctation of the aortic arch) were surgically corrected.Different procedures,such as arterial switch operation,bidirectional cavo-pulmonary anastomosis,Rastelli procedure,Fontan operation and Senning operation,were adopted according to different malformations.Clinical data was reviewed and postoperative survival was evaluated.Results There were 14 cases of early deaths (5.09％) in this group with 10 cases occurred after arterial switch operation (10/243,4.12％) and 4 cases following other procedures (4/32,12.50％).Surviving patients following Glenn/modified Fontan procedures had significant improvement in oxygen saturation.Patients who undertook arterial switch operation and Senning operations could grow and develop normally.Conclusions Complete transposition of great arteries can be treated with a variety of procedures according to the combined anomalies.Satisfactory results can be achieved in neonates with arterial switch operation.