右心室双出口的手术治疗

目的　总结右心室双出口手术治疗的早期和远期结果及经验。　方法　 1985年 5月～ 2 0 0 1年 12月 4 1例右心室双出口患者接受了外科治疗 ,其中男 2 6例、女 15例 ,年龄 10个月～ 2 1岁 ,平均 (10± 6 )岁 ,体重 6 5～ 6 5 0kg ,平均 (2 6± 16 )kg。 4 1例中 ,14例行经典的Rastelli手术 (内隧道外管道手术 ) ;11例行经内隧道成形左心室流出道、右心室流出道直接疏通或用心包片加宽 ;11例行改良Rastelli手术 ;2例行全腔静脉肺动脉吻合术 ;2例行双向格林手术 ;1例行大动脉调转术。结果　早期 (术后 1个月 )死亡 1例 ,出院 4 0例。随访 36例 ,随访 5个月～ 17年 ,中位数为 7年 ,无死亡 ,二次手术 3例。所有患者紫绀消失 ,症状明显改善 ,心功能Ⅰ级 34例 ,心功能Ⅱ级 2例 ,能够进行正常的生活、学习和工作。　结论　绝大多数右心室双出口患者经双心室矫治可以取得很好的早期及远期结果 ;对于合并其他严重畸形的患者可行右心室旁路手术。

Surgical treatment for double outlet of the right ventricle

OBJECTIVE: To summarize the experience and results of surgical treatment for double outlet of the right ventricle (DORV). METHODS: From May 1985 to December 2001, 41 patients with DORV with age ranging from 10 months to 21 (mean age 10 +/- 6) years underwent surgical treatment. There were 26 male and 15 female patients. 14 patients underwent the classical Rastelli procedure (an intraventricular tunnel and a extra cardiac conduit repair); intraventricular tunnel and relive right ventricle outflow tract directly with and with out pericardial patch in 11; and modified Rastelli procedure in 11; total cavopulmonary connection (TCPC) in 2; Bidirection cavopulmnary connection in 2; Arterial switch operation in 1. All patients underwent the procedure by cardiopulmonary bypass. RESULTS: There were 1 early death, 40 patients were discharged without any complication. Thirty-six patients were followed up from 5 months to 17 years. Reoperation in 3, because of residual ventricular septal defect in 1 and left ventricle outflow tract stenosis in 2. All patients were asymptomatic and did well in ordinary life. The cardiac function were in NYHA class I in 34 and class II in 2. The outcome was satisfactory. CONCLUSIONS: Biventricular repair can be achieved in most patients with DORV with satisfactory early and late results, and the late survival was exciting. In complex DORV, a right heart bypass procedure is related to a lower surgical mortality.