Rastelli手术治疗大动脉转位伴室间隔缺损

目的介绍Rastelli手术治疗大动脉错位伴室间隔缺损的经验.方法全组49例中男29例,女20例.平均年龄5.6岁;平均体重17.2kg.完全性大动脉转位31例,纠正性大动脉转位18例;伴肺动脉狭窄45例,伴肺动脉高压4例.均在低温体外循环下行Rastelli手术.二期根治5例.体外循环灌注(178.5±52.5)min;主动脉阻断(109.2±38.3) min.结果手术早期死亡6例,死亡率12.2%.死因为肺动脉高压危象、肾衰、心律紊乱和严重低心输出量综合征.术后并发症有心律紊乱、肺动脉高压危象、蛛网膜下腔出血、脑功能紊乱、肾衰及多脏器功能衰竭、心包或胸腔积液、感染等.CICU平均监护7.3 d.随访中因同种带瓣大动脉(VHC)感染死亡1例.结论完全性大动脉转位手术中,右室流出道直切口有利于心内隧道修补室间隔缺损;纠正性大动脉转位手术中,解剖右室径路显露缺损较好且易避开传导系统,但不利于术后心功能恢复.而解剖左室径路修补室间隔缺损的房室传导阻滞发生率高;大于4岁者手术宜选择大号VHC可减少再次手术几率;对无长段左室流出道狭窄的完全性大动脉转位病婴,可在动脉换位术基础上行肺动脉瓣叶交界切开或Konno术以解除左室流出道梗阻.为防止术后功能性二尖瓣反流,对伴肺动脉狭窄的纠正性大动脉转位病儿,提倡心房-大动脉双换位手术.

Rastelli repair for transposition of great arteries with ventricular septal defect

Objective: To review the experiences of Rastelli repair for transposition of great arteries with ventricular septal defect (TGA-VSD). Methods: 49 patients with TGA-VSD underwent Rastelli operation at our department between September 1991 and April 2002. The mean age was 5.6 years and mean weight 17.2 kg. 5 patients had history of prior palliative operations (BT Shunt in 3 and PA banding in 2). Results: There were 6 postoperative deaths with a hospital mortality of 12.2%. The causes of early death were pulmonary hypertension crisis, renal failure, arrhythmia and severe low cardiac output syndrome. The postoperative complications included arrhythmia, pulmonary hypertension crisis, renal and multi-organ failure, pericardial and pleural effusion and pulmonary infection. Mean CICU stay was 7.3 days. Postoperative VHC infection was the factor contributing to 1 late death. Conclusion: For D-TGA patients, a right ventricular incision can provide excellent exposure of VSD. For C-TGA patients, exposure of VSD by a right ventriculotomy could be a good approach in avoiding damage to conduction system but not good for later cardiac function. While repair VSD with exposure through the left ventricle may be benefices to cardiac function but increases the risk of damage of conduction system. Large size of VHC (>15 mm) should be used in patients over the age of 4 and later reoperation might be avoided. For D-TGA without long segment of LVOTO, we can choose arterial switch plus pulmonary valvotomy or Konno procedure to relief the LVOTO. To prevent postoperative functional mitral regurgitation, in C-TGA-VSD-PS patients the best surgical procedure is double switch operation.