Objective:To retrospectively analyze the effects of anatomical morphology on surgical method selection and prognosis in double outlet right ventricle (DORV),and provide individual operation design for DORV with different pathological anatomy.Methods:From Jan 2010 to June 2015,67 patients with DORV (41male,26 female) were operated,aged from 5 months to 27years and body mass from 3.4 kg to 65.0 kg.All patients with preoperative echocardiography and heart computed tomography angiography (CTA).DORV were confirmed by open-heart surgery.The malformation classified by Steward on the location of ventricular septal defect(VSD),including 39 patients of subaortic VSD,9 of subpulmonary VSD (Taussig-Bing malformation),12 of subarterial VSD and 7 of non-subarterial VSD.Patients with severe pulmonary hypertension,complete atrioventricular septal defect,atrioventricular connections discording,left ventricular dysplasia have been ruled out.The surgical procedures included:1Took inner tunnel to repair VSD and widen outlet of right ventricle with patch for 51 cases.2Took inner tube to connect VSD and aorta,then widened outlet of right ventricle with monocusp patch or cut off pulmonary artery,blocked proximal end and took outer tube with valve to connect pulmonary remote end and incision of right ventricle (Rastelli procedure) for 9 cases.3Took inner tunnel to connect VSD and pulmonary artery,followed by arterial switch operation (ASO) in 4 cases.4Total cavopulmonary connection (TCPC) procedure in 1 case,bidirectional Glenn shunt in 1 case,and B-T shunt in 1 cases.Results:For all cases,average time of tracheal intubation maintaining,ICU and hospital staying was (29.5 ± 13.7) hours,(2.1 ± 1.9)days and (14.5 ±7.2) days,respectively.Postoperative complications occurred on 18 cases,including reintubatiorn in 3 cases and 5 cases of pleural effusion,ascites in 2 cases,1 case of pneumothorax,and acute renal injury in 2 cases,low cardiac output syndrome in 5 cases.5 cases of early death after surgery,mortality is 7.5％.Long term follow-up of 60 cases,from 1to 6 years,6 cases of left ventricular outflow tract obstruction and 4 cases of right ventricular outflow tract stenosis after Rastelli operation were found by echocardiography examination.There were no obstruction and obviously residual shunt in the others.Conclusion:Only clearly understood the pathological anatomy of DORV and took the individual surgical procedure,could it ensure a good therapeutic efficacy.%目的:回顾性分析右心室双出口(DORV)的解剖形态特点对手术方式选择及患者预后的影响,为不同病理解剖形态的右心室双出口患者提供个案化的外科治疗方案.方法:自2010年1月至2015年6月,对67例DORV患者行手术治疗,其中男性41例,女性26例,年龄5个月～27岁,体质量3.4～65.0 kg.全部患者术前均做超声心动图和心脏CT血管造影(CTA).心内直视手术证实为DORV.按Steward对该畸形的室间隔缺损(VSD)位置分类:主动脉开口下39例,肺动脉开口下(Taussig-Bing畸形)9例,两大动脉开口下12例,远离两大动脉开口7例.合并重度肺动脉高压、完全性心内膜垫缺、房室连接不一致、左心发育不良患者已排除.手术方式:①心内隧道修补VSD、心外补片加宽流出道和肺动脉51例;②内管道连接VSD与主动脉,同时作带瓣补片加宽右心室流出道或切断肺动脉,封闭其近端、远端与右心室切口之间用带瓣外管道连接(Rastelli手术)9例;③心内隧道修补VSD连接左心室与肺动脉,再行大动脉调转(ASO)术4例.④全腔静脉-肺动脉连接术1例、双向Glenn手术1例、B-T分流术1例.结果:平均带气管导管时间(29.5±13.7)h,平均住ICU时间(2.1±1.9)d,平均住院日(14.5 ±7.2)d.术后出现并发症18例,其中二次插管3例,胸腔积液5例,腹腔积液2例,气胸1例,急性肾损伤2例,低心排5例.手术后早期死亡5例,病死率7.5％.远期随访60例,随访时间1～6年,超声心动图检查示术后左心室流出道梗阻(LVOTO)6例,Rastelli术后右心室流出道狭窄4例,其余均无残余梗阻和明显残余分流.结论:明确DORV病理解剖关系,根据不同病变采取个案化手术方案,是取得良好治疗效果的保证.
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