体外循环下小儿肌部室间隔缺损的镶嵌治疗

Hybrid procedure with cardiopulmonary bypass for muscular ventricular septul defects in children

目的探讨镶嵌模式（hybrid procedure）治疗小儿肌部室间隔缺损（mVSD）的手术方法及临床应用。方法2006年1月至2010年6月，在体外循环心内直视手术下采用手术及封堵相结合的镶嵌技术矫治小儿mVSD45例，其中男20例，女25例；年龄52天～12岁；体重3～32kg。7例为单个mVSD，38例为多发性VSD。同时合并大血管错位（D—TGA）1例、法洛四联症（TOF）2例、肺动脉狭窄（PS）3例、动脉导管未闭（PDA）6例、房间隔缺损（ASD）6例、主动脉缩窄1例。均在心脏停跳后直视下将导引钢丝经三尖瓣孔自心脏右室面穿过VSD至左室面，直视下置入导引器，然后送入封堵器，完成mVSD封堵。多发性VSD38例，予自体心包片修补膜周部等较大的VSD，心内其他畸形同期完成矫治。结果42例置入单枚封堵器（直径4—10mm）、3例置入双枚封堵器（直径4—7mm）。手术经过顺利，术前左室射血分数（EF）均在正常范围，术后1天小于8月龄组EF均值低于正常，大于8月龄组EF正常，两者差异有统计学意义。术后常规每天给予5mg／kg肠溶阿司匹林3～6个月。术后随访超声检查示封堵器位置无偏移，无残余分流，无二尖瓣、主动脉瓣反流、Ⅲ度传导阻滞及新发心律失常等。术后因重症感染放弃治疗1例，无远期死亡病例。结论体外循环下镶嵌技术治疗小儿mVSD明显降低了围手术期并发症及病死率，简化了手术过程，降低了手术风险，是一种安全、有效的方法。

Objective To summarize the technique and clinical experience of hybrid procedure under cardiopulmonary bypass （CPB） in children with muscular ventricular septal defect （mVSD）. Methods From January 2006 to June 2010, 45 cases of mVSD underwent hybrid procedure with CPB. mVSDs were closed with devices under direct vision in 45 cases. Of them, there were 20 males and25 females. They ranged from 52 days to 12 years[mean （2.05 ±2.48）year] in age and from 3 to 30 kg [ （ 11.93 ± 7.70） kg3 in body weight. Preoperatively, most of children were highly susceptible to respiratory tract infections. The hybrid approach was used in all patients with CPB under the guidance of transesophageal echocardiography （TEE）. The diameter of mVSDs ranged from 2 to 7 mm under TEE. Of 45 cases, 40 patients had increased rates of pulmonary blood flow. 29 patients had left axis deviation and 12 cases had sinus arrhythmia on electrocardiography （ECG）. 19 had other congenital heart lesions, including transposition of great arteries in 1 ease, tetralogy of Fallot in 2, pulmonary artery stenosis in 3, patent ductus arteriosus in 6, atrial septal defects in 6） and aorta coactation in 1. The quantity of VSDs were from 1 to 7 （ single, in 7 ; two, in 24 case ; three, in 8 case ; four, in 5 case and seven, in 1 case. 37 patients were combined with pulmo- nary hypertension in our cohort. 38 patients with another large VSD and 19 with other congenital heart lesions were required surgical repair at sometime. Results The hybrid procedures were undertaken in all 45 cases of this cohort. All cases were successful and no deaths occurred during operation. A total of 48 devices were implanted in 45 patients, including single devices in 42 cases （ device size ranged from 4 to 10 mm） and two devices in 3 cases （ device size ranged from 4 - 7 mm）. The average time on CPB was （58.28 ± 20.70） min , while aortic crossclamp time was （34.94 ± 14.75 ） min. In addition, the time on mechanical ventilation postoperatively ranged from 2 hours to 6 days. Compared to the older children, 20 infants aged less than