右心室双出口的诊断与治疗

右心室双出口的诊断与治疗

作者：李伯君,高长青,盛炜

作者单位：中国人民解放军总医院心血管外科，北京100853 【摘要】目的回顾性分析47例右心室双出口(double outlet right ventricle, DORV)病例的病理解剖特点及其手术方式，探讨DORV的个案化手术设计治疗。方法自1997年1月至2006年1月对47例DORV患者行手术治疗，其中男性31例，女性16例；年龄2～26（13.6±12.1）岁。诊断为单纯DORV者36例，DORV合并镜面右位心、内脏转位5例，1例合并下腔静脉闭锁、左上腔静脉畸形引流及无顶冠状静脉窦畸形，2例Taussing-Bing心脏畸形，2例伴有完全型房室隔缺损（其中1例伴有大血管转位），46例患者均伴有肺动脉狭窄（PS），临床表现为紫绀和杵状指。另外1例17岁男性患者术前仅由超声心动图诊断为右位心、室间隔缺损（VSD），无紫绀，术中探查为DORV且无PS，肺动脉压不高。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36例；(2) 内管道连接VSD 与主动脉，同时作补片加宽右室流出道或切断（开）肺动脉，封闭其近端、远端与右室切口之间用外管道连接(Rastelli 手术) 8例；(3) 全腔静脉-肺动脉连接术2例；(4) Glenn（格林）手术1例。结果平均带气管导管时间（30.6 ±18.40）h，平均住ICU时间（2.8 ±1.5）d，平均住院日（25.4 ±18.0）d。术后出现并发症6例，其中二次插管1例，胸水2例，腹水2例，气胸1例，低心排1例。手术后早

期死亡3例，死亡率6.4 %。远期随访28例，行超声心动图检查均无残余梗阻和残余分流。结论明确DORV病理解剖关系，根据不同病变采取不同手术方式予以矫治，是取得良好治疗效果的保证。

【关键词】先天性心脏病；右室双出口；紫绀；外科治疗；

Diagnosis and Treatment of Double Outlet Right Ventricle

LI Bo-jun，GAO Chang-qing，SHENG Wei，ZHU Lang-biao，LI Gong-song，YU Yi-fei，W ANG Dong-qing

Department of Cardiovascular Surgery,General Hospital of PLA, Beijing 100853, China

Abstract: OBJECTIVE To analyze pathoanatomy and modus operandi of 47 patients with Double Outlet Right Ventricle（DORV）, and evaluate individual operation design of DORV.METHODS47 patients (31male, 16 female) aged from 2 to 26 (mean 13.6±12.1) with DORV were operated from Jan 1997 to Jan 2006, 46 of these patients had pulmonary artery stenosis. The modus operandi included: (1) took "inner tunnel" to repair VSD and widen outlet of right ventricle with patch for 36 cases. (2) took "inner tube" to connect VSD and aorta, then widened outlet or cut off pulmonary artery, blocked proximal end and took outer tunnel to connect remote end and incision of right ventricle (Rastelli method) for 8 cases. (3) Total Cavopulmonary Connection for 2 cases. (4) Glenn shunt for 1 case. RESULTSFor all cases, the everage time of tracheal intubation maintaining, ICU and hospital staying was 30.6±18.4

hours, 2.8±1.95 days and 25.4±18.0 days, respectively. Postoperative complications occured on 6 cases, including reintubation (1 cases), pleural fluid (2), ascites (2), pneumothorax (1) and low heart output (1) .

3 cases (6.4%) died postoperatively. Long-term follow of 28 cases found there was no residual obstruction and residual shunt with echocardiography for all of them.CONCLUSIONOnly understand the pathoanatomy of DORV and take the homologus operandi to operate, can it ensure a good therapeutic efficacy.

Key words：Congenital heart disease; Cyanosis; Double outlet right ventricle；Operation

右室双出口(double outlet right ventricle,DORV）临床无自愈可能，在诊断建立后均应行手术治疗。自1961年McGoon等首次成功矫治1例DORV以来［1］，许多更为复杂的DORV通过外科手术得以矫治。近年来，随着对其病理解剖认识的提高，手术治疗该疾病取得较大进展。本文旨在通过回顾性分析47例DORV患者的病理解剖特点及其手术方法，探讨DORV的个案化手术设计治疗。

1资料与方法

1.1临床资料

研究对象为1997年1月至2006年1月在本院接受手术治疗的47例DORV患者，其中男性31例，女性16例；年龄2～26（13.6±12.1）岁。DORV的诊断标准为一条大动脉的全部及另一条大动脉的50%以上起自解剖右心室(50% 规则)；室间隔缺损(ventricular