肺动脉闭锁伴大型主肺动脉侧支的单源化手术治疗

摘要

Objective To evlauate the midterm outcomes of surgical repair for pulmonary atresia with ventricular septal defect (PA-VSD) and major aorto-pulmonary collateral arteries (MAPCA).Methods Between January 2006 and December 2012,twenty-seven consecutive patients undergoing surgical repair for PA/VSD/MAPCA in Shanghai Children' s Medical Center were retrospectively reviewed.There were 14 males and 13 females with a media age of 13.4 months[(0.5 ± 79.4) months] and a media weight of7.5 kg[(2.1 ± 65.0) kg].Associated lesions included atrial septal defect(ASD) in 9 and patent ductus arteriosus(PDA) in 14.Results Primary complete correction (intracardiac repair + unifocalization) was performed in 5 patients while staged operation was adopted in 22 patients including unifocalization with right ventricular-pulmonary artery reconstruction in 20,unifocalization with modified BT shunt in 1,and unifocalization with central shunt in 1.A total of 2 patients underwent a hybrid procedure.There was no death during hospital stay and follow-up.The cardiopulmonary bypass (CPB) time ranged from 76 to 179 min [(134.7 ± 43.8)minutes] and the aortic crossclamping time from 0 to 64 min [(40.0 ± 16.9)minutes].In 2 patients undergoing one-stage repair,enlargement of the ASD and fenestration (4 mm) on a VSD patch were required to relieve an increased Pp/Ps.A median sternotomy combined with lateral thoractomy was used in 4 patients.Major postoperative complications included low cardiac output syndrome in 4,Ⅱ-degree atrioventricular heart block in 1,lung infection in 4,sepsis in 1,residual right ventricular outflow tract obstruction(pressure gradient ＞40 mmHg) in 2,patient,phrenoparalysis in 1,wound infection in 2,patient,chest drainage over 15ml/kg requiring subsequent operation in 1,and residual pulmonary branch stenosis in 2.in 5 patients(20％).1 patient developed sinus bradycardia in association with sinus pauses two months after discharge.There were overall two in-hospital death.1 patient developed pericardial effusion one month after discharge.The follow-up period ranged from 3 months to 6 years[(48.52 ± 22.16) months] and there were no late death during this period.Conclusion Unifocalization provides an effectual and feasible way to treat major aorto-pulmonary collateral arteries in patients suffering from PA/VSD.It can avoid the increment of both right ventricular pressure overload and pulmonary vascular resistance(PVR) which result from the gradual stenosis of MAPCA.Besides,it is beneficial to the growth of central pulmonary artery.Preoperative unsatisfactory Nakata index and increased number of MAPCA were two significant predictors of poor survival.The choice of individualized staged surgical procedure is of importance in decreasing mortality and complications.%目的 总结单源化手术治疗肺动脉闭锁伴室间隔缺损(PA-VSD)合并大型主肺动脉侧支(MAPCA)的临床应用效果.方法 2006年1月至2012年12月,27例PA-VSD-MAPCAs的患儿行手术纠治,男14例,女13例;平均年龄13.4个月;平均体质量7.5 kg;伴房间隔缺损9例、动脉导管未闭14例,均无其他心内外畸形.结果 一期根治术(侧支单源化加心内缺损修补)5例,姑息性单源化加右心室流出道-肺动脉重建术(RV-PA reconstruction) 20例;姑息性单源化加改良Blalock-Taussig分流术(mB-T)1例,单源化加中央分流术1例.单源化手术中MAPCA与肺动脉或肺动脉分支行端侧吻合11例、侧侧吻合20例.心内、外科杂交手术2例.体外循环76 ～179 min,平均(134.7 ±43.8) min,主动脉阻断0 ～ 64 min,平均(40.0±16.9)min.2例根治术患儿行室间隔补片开窗(开窗4 mm)/扩大房间隔缺损;4例患儿采取正中切口加侧切口途径入胸.术后并发低心排综合征4例,肺部感染4例,膈肌麻痹2例,Ⅱ度房室传导阻滞1例,脓毒血症1例,伤口感染1例.1例患儿因胸引量较多(每小时＞ 15 ml/kg)二次开胸探查止血.术后右心室流出道残余梗阻2例[压差＞40 mmHg(1 mmHg=0.133 kPa)],肺动脉及分支残余梗阻2例.2例患儿住院期间因低心排血量综合征和肺部严重感染死亡.出院患儿门诊随访3个月～6年,随访期间无死亡.结论 早期单源化手术是处理肺动脉闭锁伴室间隔缺损合并MAPCA的重要方法,可避免MAPCA渐进性狭窄造成肺血管阻力增加、右心室后负荷增加,且可促进中央及末梢肺血管的发育.MAPCA数量的增加和术前较低的Nakata指数是早期死亡的危险因素.个体化选择分期手术方案可有效降低病死率和并发症.