房室连接不一致的心室双出口外科治疗

Surgical treatment for double outlet of ventricle with atrioventricular discordance

目的 报道房室连接不一致的心室双出口外科治疗经验。 方法 从 1985年 1月至 2 0 0 2年 9月 ,共进行房室连接不一致的心室双出口外科治疗 11例 (年龄 3～ 2 5岁 ) ,其中右心室双出口 (DORV) 10例 ,左心室双出口(DOL V) 1例 ,行双心室修复术 9例 ,采用心内补片、心外带瓣管道或冷冻同种带瓣管道修复 ;行 Fontan类手术 2例。结果 双心室修复术后早期死亡 2例 ,死亡原因为严重低心排血量综合征 ;Fontan类手术修复后血氧饱和度和活动耐力明显改善 ,无早期死亡 ;双心室修复术后长期随访 4例 ,随访时间 1～ 11.5年 ,NYHA心功能 级或 级 3例 , 级 1例。 结论 房室连接不一致的心室双出口根据心室、肺动脉发育和合并心内畸形决定手术方式 ,完整修复室间隔缺损 ,保证心室大动脉流出道通畅 ,修复房室瓣和防止损伤传导束是传统心内修复术的关键。

Objective To report the surgical treatment for double outlet of ventricle with atrioventricular discordance. Methods 11 patients aged from 3 to 25 years underwent surgical treatment for double outlet of ventricle with atrioventricular discordance. 10 of them were double outlet right ventricle and the other one was double outlet left ventricle. The surgical treatment included biventricular repair (n=9) and single ventricular repair (n=2). The biventricular repair was performed by intraventricular patch repair and extracardiac valved conduit or homograft valved conduit. The single ventricular repair included bidirected Glenn operation and total cavopulmonary connect. Results There were two early deaths in biventricular repair and no death in single ventricular repair. The cause of death was severe low cardiac output syndrome. Four survivors in biventricular repair were followed up from 1 to 11.5 years, three of them were in NYHA class Ⅰ or Ⅱ and one in NYHA class Ⅲ. Conclusions Double outlet of ventricle with atrioventricular discordance can be treated by biventricular repair or single ventricular repair according to the development of ventricle and pulmonary arteries. Closure of ventricular septal defect , no obstruction from ventricle to great artery and no harm of conduction system are the keys of conventional management to achieve good results.