摘要目的 探讨双调转手术和传统修复术治疗矫正型大动脉转位合并心内畸形的效果.方法 2002年4月至2006年12月,19例矫正型大动脉转位合并心内畸形患者接受手术治疗,男性14例,女性5例;年龄2～22岁,平均8.6岁.合并的心内畸形包括:室间隔缺损18例,右心室双出口1例,肺动脉狭窄17例,房间隔缺损4例,肺动脉高压2例.手术方式包括双调转手术解剖矫正修复15例,传统修复术治疗心内畸形4例.结果 双调转术后死亡1例,发生严重低心排血量综合征2例,一过性房室传导阻滞1例,反复胸腔积液2例.传统心内修复术后无死亡病例,发生严重低心排血量综合征1例.双调转手术存活者随访6个月～4年,均为窦性心律,心功能NYHA分级Ⅰ～Ⅱ级,射血分数51%～68%.传统心内修复术存活者随访1年,3例心功能达Ⅰ～Ⅱ级,射血分数52%～61%;1例心功能Ⅲ级,射血分数40%.结论 双调转手术解剖修复矫正型大动脉转位合并心内畸形早中期效果良好,严格选择手术适应证和手术时机是提高手术效果的关键.对于右心室功能较好,无三尖瓣畸形的患者,如果存在影响双调转效果的因素,仍应选择传统心内修复手术.

abstractsObjective To determine the outcome of anatomically corrective repair and traditional repair of corrected transposition of great arteries(c-TGA) with heart anomaly. Methods From April 2002 to December 2006, nineteen patients including fourteen male and five female with c-TGA,underwent operations,age ranged from 2 to 22 years old and weight ranged from 10 to 48 kg. Fifteen of them received anatomically corrective repair and the other four received traditional repair. Eighteen patients were referred to SLL (segmental anatomy)in situs solitus while fifteen of them with levocardia and three with dextrocardia.One patient was referred to IDD(segmental anatomy)in situs inversus with levocardia. Associated cardiac lesions included ventricular defect in eighteen patients, double outlet of right ventricle in one patient,pulmonary stenosis in seventeen patients and pulmonary hypertension in two patients. The operative procedures to anatomically correct atrioventricular discordance included an atrial switch plus a ventriclearterial switch. The atrial switch was performed using the modified Senning procedure(n=13),Senning procedure(n=1) and Mustard procedure(n=1). The ventricle-arterial switch was performed using a Rastelli procedure(n=13) or an arterial switch(n=2). The patients underwent Mustard and Rastelli procedure had received bidirect Gleen shunt due to postoperative high pressure of superior vena cava. Three patients underwent traditional cardiac repair because of small ventricular septal defect and one patient was reoperated to undergo traditional cardiac repair because of left ventricular failure after received anatomically corrective repair. Results In the patients received anatomically corrective repair,there was one early operative death received a modified Senning atrial switch and an arterial switch. The cause of death was acute myocardial failure due to imperfect coronary transfer. The postoperative complications included severe low cardiac output syndrome(n=1),temporary atrioventricular block(n=1) and thorax cavity fluidify ( n=1). The survivors were followed up for 6 months to 4 years. All were sinus cardiac rhythm and in NYHA class Ⅰ or Ⅱ. There was no death in the patients received traditional repair. Four patients were followed up for 1 year. Three patients were in NYHA Ⅰ or Ⅱ class and one patient in class Ⅲ. Conclusions Anatomically corrective repair of c-TGA can be performed with good operative survival and intermediate-term outcome. The patients with good right ventricular function and well developed tricuspid valve who were difficult to undergo anatomically corrective repair might be fit to receive traditional repair.