右心室双出口无肺动脉狭窄的外科治疗

目的回顾性总结右心室双出口(DORV)无肺动脉狭窄(PS)病人的手术治疗经验。方法1975年1月至2003年6月，38例DORV无PS病人均采用双心室修复，建立左心室至主动脉心内隧道，其中2例DORV肺动脉下室间隔缺损的婴儿行大动脉调转术。结果全组早期死亡5例(13．2％)，其中2例死于肺动脉高压危象、3例死于低心输出量综合征。33例生存者中29例(87．9％)随访1个月。23年，晚期死亡1例(2．6％)，术后95d死于室间隔残余漏所致右心衰竭。随访者心功能恢复至Ⅰ级19例，Ⅱ级9例，Ⅲ级1例。结论DORV无PS的手术治疗早、晚期效果良好。一旦诊断明确，应尽早手术治疗，以避免产生不可逆的肺血管病变，影响治疗效果。

Surgical treatment of double-outlet right ventricle without pulmonary stenosis

Objective: To investigate the result of biventricular repair for double outlet right ventricle without pulmonary stenosis. Methods: From January 1975 to June 2003, 38 consecutive patients underwent biventricular repair for double outlet right ventricle without pulmonary stenosis. At surgery, the median age of this group was 5.8 years. Two types of repair techniques were applied: intraventricular baffle repair (n=36) and arterial switch operation with VSD to pulmonary artery baffle (n=2). Results: There were 5 hospital deaths, with mortality rate of 13 2%. Two of these patients died of pulmonary hypertension crisis and the other 3 died of low cardiac output syndrome. One case (2 6%) died of right heart failure on 95th day postoperatively. The follow up periods in 29 patients (76%) ranged from 1 month to 23 years. 19 cases (65.5%) of these patients were in NYHA class I, 9 (31.0%) in NYHA class II, and the 1 (3.5%) in NYHA class III. Conclusion: The biventricular repair might be an effective treatment for double outlet right ventricle without pulmonary stenosis is good. Operation should be performed as early as possible before the development of progressive pulmonary vascular disease.