复杂畸形伴肌部多发室间隔缺损的直视镶嵌封堵

目的 介绍复杂畸形伴肌部多发室间隔缺损心内直视镶嵌封堵的经验.方法 全组10例,男7例,女3例.复杂心内外畸形包括右心室双出口Taussig-Bing畸形2例、右心室双出口主动脉瓣下室缺2例、完全性大动脉错位伴室间隔缺损肺动脉高压2例、法乐四联症1例、室间隔缺损伴重度二尖瓣反流2例、室间隔缺损伴主动脉重度缩窄1例.其他伴发畸形有:二尖瓣反流、单支右冠状动脉畸形、房间隔缺损、冠状窦隔缺损、主动脉弓发育不良、动脉导管未闭等.全组均解剖纠治镶嵌心内直视封堵肌部多发室间隔缺损,平均手术年龄(8.8±5.4)个月,平均手术体重(6.3±2.0)kg.结果 手术无死亡.无心内直视镶嵌封堵相关并发症.3例术后并发肺动脉高压危象;1例术后3 d延迟关胸;1例拔肺动脉测压管出血,心包填塞,床边开胸止血.随访无死亡,2例封堵器下缘残留少量分流.结论 心内直视封堵肌部多发室间隔缺损,可避免传统手术纠治需心室切口、心室内肌束切开,对心功能影响大的缺点;可缩短体外循环时间,同时镶嵌复杂畸形Ⅰ期解剖纠治,使手术简化、安全、尤其适用于婴幼儿,可获得较佳中长期疗效.

Abstract：

Objective To present our experience using hybrid approaches to repair muscular ventricular septal defects (mVSDs) associated with additional congenital heart diseases in children.Methods From April 2006 to September 2010, 10 patients with mVSDs associated with additional congenital heart diseases underwent corrective surgery at this center. Their mean age was 8. 8 month old, and their mean body weight was 6. 3 kg. The additional congenital heart defects included 2 patients with D-transposition of great arteries, 2 with double outlet right ventricle-Taussig-Bing anomaly, 1 with Tetralogy of Fallot, 2 with double outlet right ventricle, 1 with severe coarctation of aorta,and 2 with severe mitral valve regurgitation. Results All patients underwent the single stage hybrid approach repair under direct vision and all recovered from surgery. After surgery, 3 patients developed pulmonary hypertension crisis among whom 1 had delayed chest closure and 1 underwent emergence surgery to relief cardiac tapenade. The patients were followed up for 3 months to 3 years No late deaths were noted. Complete closure of muscular ventricular septal defects was achieved on 5 patients.Clinically insignificant residual shunt was observed on 2 patients. Conclusions It is safe and effective using hybrid approaches to repair muscular ventricular septal defects (mVSDs) associated with additional congenital heart diseases in children.

Hybrid approaches for the treatment of muscular ventricular septal defects associated with additional congenital heart diseases

Objective To present our experience using hybrid approaches to repair muscular ventricular septal defects (mVSDs) associated with additional congenital heart diseases in children.Methods From April 2006 to September 2010, 10 patients with mVSDs associated with additional congenital heart diseases underwent corrective surgery at this center. Their mean age was 8. 8 month old, and their mean body weight was 6. 3 kg. The additional congenital heart defects included 2 patients with D-transposition of great arteries, 2 with double outlet right ventricle-Taussig-Bing anomaly, 1 with Tetralogy of Fallot, 2 with double outlet right ventricle, 1 with severe coarctation of aorta,and 2 with severe mitral valve regurgitation. Results All patients underwent the single stage hybrid approach repair under direct vision and all recovered from surgery. After surgery, 3 patients developed pulmonary hypertension crisis among whom 1 had delayed chest closure and 1 underwent emergence surgery to relief cardiac tapenade. The patients were followed up for 3 months to 3 years No late deaths were noted. Complete closure of muscular ventricular septal defects was achieved on 5 patients.Clinically insignificant residual shunt was observed on 2 patients. Conclusions It is safe and effective using hybrid approaches to repair muscular ventricular septal defects (mVSDs) associated with additional congenital heart diseases in children.