摘要目的 总结法洛四联症(TOF)合并冠状动脉畸形的解剖类型和手术方案的选择,评价术后效果.方法 2008年1月到2014年8月,1 142例患者接受TOF矫治手术,其中38例合并异常冠状动脉.年龄1个月～ 27岁,体质量4.5～51.0 kg.单支冠状动脉15例,双重前降支15例,右冠状动发出单一前降支3例,其他5例.根据异常冠状动脉位置和走行、右心室流出道狭窄部位及程度,制定手术策略.结果 手术死亡1例,37例长期生存.单片法15例,其中7例未跨环补片均残留流出道梗阻,1例需再手术;双片法6例,3例术中因残留梗阻改用双通道法;双通道法6例,随访无外管道狭窄;肺动脉下拉法11例,4例术后右肺动脉狭窄;异常冠状动脉结扎切断法3例.结论 TOF合并异常冠状动脉不是一期根治的禁忌证.冠状动脉畸形的解剖变异多样,需个体化制定手术方案.

abstractsObjective The results of repair for TOF with anomalous coronary artery(ACA) were studied to determine the incidence of coronary anomalies and evaluate surgical strategy choicesas well as postoperative outcomes.Methods From January 2008 to August 2014,1142 consecutive patients underwent repair of TOF including 44 patients with TOF and ACA:single coronary artery in 15,dual anterior descending coronary in 15,single left anterior descending coronary arising from the rightcoronary artery in 3 and the other ACA in 5.The median age was 5.7 years (range,1 month-27 years),and the median weight was 16.0 kg(range,4.5-51.0 kg).Surgical procedure was selected according to the extent of right ventricular outflow tract (RVOT) obstruction and distribution of the ACA.Results There was one operative death.No deaths during the follow-up period in the other 37 patients.Single patch techniquewasperformed in 15.RVOT residual obstruction detected in 7 who without transannular patch,and one need reoperation;Two patch technique was performed in 6,and 3 of them required an additional RV-PA(pulmonary artery) tube because of RVOT residual obstruction during the operation;Double oullet technique was in 6.No tube stenosis occurred in follow-up period time;PA translocation technique was in 11.The right PA stenosis was detected in 4;ACA was ligated and divided in 3,then RVOT reconstruction was performed.Conclusion The combination of ACA is not a contraindication to primary repair of TOF.But there are many anatomiacal variations of ACA,and the accuracy of preoperative diagnosis is low.So proper selection of surgical approach should be individualized based on the careful intraoperative identification of the distribution of the ACA as well as the location and degree of the RVOT obstruction.