双调转术治疗矫正型大动脉转位合并心内畸形

目的总结双调转手术治疗矫正型大动脉转位（cTGA）合并心内畸形的经验。方法2002年4月至2004年6月，行双调转手术治疗cTGA合并心内畸形病儿7例，其中男6例，女1例；年龄4—15岁，平均8．4岁。SLL型6例，IDD型1例。合并室间隔缺损6例，右室双出口1例，继发孔房间隔缺损2例，肺动脉狭窄6例；3例SLL型为右旋心，1例IDD型为左旋心。手术方式包括改良Senning＋Rastelli 4例，Mustard＋Rastelli＋双向Glenn手术、Senning＋Rastelli手术、改良Senning＋Switch手术各1例。结果术后早期死亡1例，为cTGA合并室间隔缺损、肺动脉高压，行改良Senning＋Switch手术者，死因为左心功能衰竭。早期主要并发症有低心排血量综合征、房室分离各1例，胸腔积液和低蛋白血症2例。随访2—24个月，恢复良好，复查多普勒超声心动图、心电图和X线胸片显示，窦性心律5例，结性心律1例，心功能均为Ⅰ级。结论双调转手术可以达到解剖矫治cTGA合并心内畸形，早期手术死亡率低，中、远期心功能效果良好。SLL型病例采用改良Senning心房内调转手术效果优于Senning和Mustard手术。

Double switch operation for congenital corrected transposition of great arteries with heart anomaly

Objective To report the outcomes of double switch operation as the primary approach for congenital corrected transposition of great arteries (cTGA) with heart anomaly. Methods From April 2002 to June 2004, seven patients ranged 4 to 15 years with cTGA underwent double switch operation. Six patients were situs solitus (SLL segmental anatomy) while one patient was situs inversus (IDD segmental anatomy). The heart defects included ventricular septal defect in 6 cases, secondary atrial septal defect in, double outlet of right ventricle in 1, pulmonary stenosis in 6, dextrocardia in 3 and levocardia in 1. The operative procedures comprised of 4 modified Senning+Rastelli, Mustard +Rastelli +bidirectional Glenn, 1 Senning +Rastelli, 1 modified Senning+switch. Results There was one early operative death after modified Senning+switch operation. The cause of death was left ventricular failure. The postoperative complications included severe low cardiac output syndrome in 1, temporary atrioventricular block in 1, pleurisy and low plasma protein in 2. The survivors were followed up from 2 to 24 months, all of them were in sinus cardiac rhythm except one case with junction cardiac rhythm. All were in NYHA I class. Conclusion Anatomic correction of cGTA by double switch operation can be performed with lower operative mortality and good medial-term outcome. In the SLL cases, modified Senning operation may yield better outcomes than Mustard operation.