阜外医院71例Rastelli手术的近中期结果

目的总结分析阜外医院Rastelli手术的近中期结果。方法2010年5月至2017年3月阜外医院完成71例Rastelli手术治疗大动脉转位(TGA)或者右心室双出口(DORV)合并室间隔缺损(VSD)和肺动脉狭窄患儿,男48例,女23例;手术时年龄(4.7±2.7)岁,其中10例完全型大动脉转位;27例DORV,34例矫正型TGA(CTGA)。30例有手术史,其中Blalock-Taussig分流13例,双向Glenn手术17例。31例同时扩大室间隔缺损。外管道使用同种带瓣管道9例,牛颈静脉管道56例,自制Gore-Tex外管道6例;管道直径(17.9±3.3)mm。结果本组主动脉阻断(132.0±71.1)min,体外循环(209.0±83.4)min。机械通气时间(102.6±81.7)h。术后住院(13.6±12.8)天。早期死亡1例(1.4%)。早期并发症12例(16.9%),其中因Ⅲ度房室传导阻滞安装永久起搏器4例,心包积液开窗3例,延迟关胸3例,二次开胸2例。术后随访4个月~6.8年。1、5年生存率分别为97.2%,97.2%;1、5年免除右心室流出道狭窄(RVOTO)率分别为98.6%,84.1%;1、5年免除干预率分别为98.6%,90.0%。随访期间置换外管道1例,介入球囊扩张7例10次。所有患儿最后一次随访均未见左心室流出道狭窄,左心室到主动脉压差(10.5±8.8)mmHg。结论Rastelli手术适用于部分DORV、CTGA、TGA合并VSD和严重的肺动脉狭窄或肺动脉闭锁患儿,早期死亡比例低,中期效果好,但是远期常需要再手术,尤其是置换外管道。

Early and midterm results of the Rastelli operation in Fuwai hospital

Objective To describe eraly and midterm outcome of the Rastelli repair in Fuwai hospital Patients.Methods From May 2010 to March 2017,71 patients with transposition of the great arteries(TGA)with ventricular septal defect(VSD)and right ventricular outflow tract obstruction(RVOTO)or double outlet right ventricle(DORV)with VSD and RVOTO underwent Rastelli repair.48 cases male,23 cases female.Age at operation is(4.7±2.7)years.There are 10 TGA cases,27 DORV cases,34 CTGA cases in this group.30 patients(42.3%,30/71)received palliative operation prior to the Rastelli procedure,including 13 BT shunt and 17 bi-Glenn operation.31 patients(43.7%,31/71)underwent the Rastelli procedure with VSD enlargement.Right ventricle-to-pulmonary artery connection were created with the use of 9 homografts,56 valved bovine jugular vein,6 man-made valved Gore-Tex conduit.The overall mean right ventricle-to-pulmonary artery conduit size was(17.9±3.3)mm.Results CPB time was(209.0±83.4)minutes,aortic crossclamping time was(132.0±71.1)minutes,mechanical ventilation time was(102.6±81.7)h.Early mortality was 1.4%(1/71).morbidity in hospital was 16.9%,4 patients withⅢAVB implanted permanent pacemaker,Subxiphoid pericardial window drainage in 3 cases,delayed sternal closure in 3 and re-thoratomy for hemaostsis in 2.Follow up is from 4 months to 6.8 years.Overall survival was 97.2%and 97.2%at 1 and 5 years,respectively.Freedom from RVOTO was 98.6%and 84.1%at 1 and 5 years,respectively.Freedom from reintervention was 98.6%and 90.0%at 1 and 5years,respectively.1 patients performed a conduit replacement.Seven patients performed 10 times balloon dilatation.Time-related freedom from recurrent LVOTO on echocardiogram in all patients,and the pressure gradient of the LV to the aorta was(10.5±8.8)mmHg at the most recent follow-up.Conclusion The Rastelli operation remains the preferred procedure for part of the DORV,CTGA,TGA with VSD and severe fixed valvular or subvalvular PS.The Rastelli procedure can be performed with low early mortality.There is frequent need for late reoperation,especially for conduit replacement.