Abstract: AbstractObjectiveTo report the experience for surgical treatment of tetralogy of Fallot and double outlet right ventricle with anomalous coronary artery.MethodsTwenty patients with anomalous coronary artery underwent surgical correction from April 1989 to May 2004.The coronary abnormalities included single left or right coronary artery in 11,left anterior decending coronary artery originating from right coronary artery in 6,fistula between right coronary artery and pulmonary artery in 2 and vascular plexus on right outflow tract in 1.One stage correction in 19 cases;palliative procedure underwent in 1 case.ResultsThere was one postoperative death,2 cases with residual leak and 3 with residual obstruction.ConclusionFor the closures of VSD and dredge of the outflow tract of right ventricle,incision on right ventricle depended on coronary artery distribution.Damage of coronary artery must be avoided.More attention must be paid to the patients with abnormalous coronary distribution.

Key words: Doubleoutlet right ventricle, Abnormal coronary artery

摘要： 目的总结法洛四联症和右室双出口合并冠状动脉畸形手术治疗经验，探讨冠状动脉畸形对右室流出道重建的影响。方法1989年4月至2004年5月治疗合并冠状动脉畸形的法洛四联症和右室双出口患儿20例，其中单支冠状动脉畸形11例，左前降支起自右冠状动脉6例，右冠状动脉肺动脉瘘2例，左右冠状动脉交通支形成蔓状血管丛1例。行姑息手术1例，根治术19例。结果根治术早期死亡1例，18例随访10个月至8年，残余漏2例，残余梗阻3例，余结果满意。结论根据畸形冠状动脉走行特点，选择手术切口，避免损伤异常冠状动脉。有些术中被迫使用异体材料或无法行根治术，影响了中远期效果，应引起足够重视。

关键词: 法洛四联症, 右室双出口, 冠状动脉畸形