Konno-Rastan手术治疗儿童复杂性多水平左心室流出道梗阻

Konno-Rastan procedure in children with complex multilevel left ventricular outflow tract obstruction

目的总结Konno-Rastan手术治疗复杂多水平左心室流出道梗阻的疗效、并发症和常见失误及预防。方法1996年1月至2012年8月，13例患儿行主动脉根部及左心室流出道扩大、人工机械瓣膜主动瓣置换（Konno-Rastan术）。男8例，女5例；年龄5-13岁；体质量12-51k，中位值21kg。诊断先天性主动脉瓣狭窄8例，先天性主动脉瓣狭窄合并主动脉瓣上狭窄3例，先天性主动脉瓣狭窄合并室间隔缺损、主动脉缩窄及右心室流出道狭窄1例，先天性主动脉瓣狭窄、主动脉瓣球囊扩张术后再狭窄1例。患儿均合并继发性室间隔增厚、左心室流出道梗阻。主动脉瓣环直径12．0-16．4mm，术前跨主动脉瓣压差90-151mmHg（1mmHg=0．133kPa）。置入St．Jude AG19号机械瓣膜8例，AGl7号5例。结果无手术死亡。呼吸机辅助4-74h，中位时间6h；ICU停留1-6天。1例术后第3天出现突发性晕厥，确诊为Ⅲ度房室传导阻滞，植入永久性心外膜起搏器，术后7天恢复窦性心律，传导功能正常，将起搏器设定为60次／min，VVI模式备用。2例术后3个月复查发现新生心室水平残余分流，其中1例因反复心功能衰竭再次手术，修补残余分流，术后心功能状态良好，无残余心内畸形；1例因无明显症状，门诊随访，术后3年患类风湿，感染性心内膜炎及肾病综合征，拟择期行残余分流修补术。其余患儿无不适症状。全部患儿随访1-78个月，终生服用华法林抗凝，控制国际标准比值（INR）1．8-2．5。随访期内无死亡，未发现人工瓣膜相关性并发症发生。完全性房室传导阻滞发生率为7．7％；残余分流发生率为15．4％；术后早期感染性心内膜炎发生率为0，远期发生率为7．7％。结论Konno-Rastan手术可以有效地治疗患儿复杂的多水平左心室流出道梗阻，但由于手术操作复杂，可导致较高比例的手术并发症；完善手术操作是提高手术成功率的关键因素之一。

Objective Konno-Rastan procedure is one option to cope with complex multilevel left ventricular outflow tract obstruction （LVOTO）, which continues to pose a serious challenge to cardiac surgeons. The aim of this study is to retro- spectively analyse indications tor Konno-Rastan procedure, and to review the safeguards and pitfalls. Methods Between Janu- ary 1996 and August 2012, totally 13 children with multilevel LVOTO underwent Konno-Rastan procedure. There were 8 boys and 5 girls. Age at surgeries ranged from 5 to 13 years, and weight from 12 to 51 kg with median of 21 kg. The pathology of this cohort includes： 8 cases of congenital aortic valvular stenosis, 3 cases of congenital aortic valvular stenosis combined with supravalvular stenosis, 1 case of congenital aortic stenosis combined with VSD, coarctation and RVOT obstruction, 1 case of aortic stenosis s/p percutaneous balloon aortic valvuloplasty. All patients have secondary diffuse tunnel LVOTO. Diameter of aortic ring ranges from 12.0 to 16.4 mm, and pressure gradient across the stenotic region ranges from 90 - 151 mm Hg. 8 cases were implanted with St. Jude AGI9 while 5 cases implanted with St. Jude AG17. Results All 13 cases survived. The 4th pa- tient was implanted permanent epicardial pacemaker for transient Ⅲ AVB. The 4th and 5th patients were found residual ven- tricular septal repture at the nadir of ventricular incision, one underwent redo procedure while another is being followed up. All cases take cumadine to sustain INR at 1.8 - 2.5. No death emerges during follow-up period. The morality is 0%, the inci- dence rate of m AVB is 7.7% , residual VSD 15.4% and endocarditis 7.7%. Conclusion Konno-Rastan procedure is a promising technique to relieve LVOTO. However, this complex procedure may lead to several fetal complications. Success of the surgery demands perfect operations.