合并壁内型冠状动脉畸形患儿的动脉调转手术疗效

摘要

Objective To evaluated the early and mid-term results of arterial switch operation (ASO) for patients with intramural coronary artery.Methods From September 2008 to March 2012,75 patients underwent ASO at Guangzhou Women and Children Medical Center for repair of transposition of the great arteries and Taussig-Bing anomaly.Among these patients,7patients (9.3％) had an intramural coronary artery.Mean age at operation was 2.4 months (2 days to 1 year) and mean body weight was(4.3 ±2.2) kg.The TGA and VSD in 3 cases,TGA/IVS in 2,and Taussig-Bing anomaly in 2.Among them 3 patients had an aortic arch anomaly,interruption of the aortic arch in 1 and coarctation of the aorta in 1.The individual coronary button technique was used in coronary transfer in 7 patients,of whom one patient required to unroof the intramural segment,an-other one required to unroof the intramural segment and enlarge with autologous pericardium the because of myocardial ischemia.There was 1 operative death because of low cardiac output syndrome.This patient underwent a coronary transfer combining aortic arch repair but without unroofing the stenotic intramural segment.The mortality was 14.2％.In the same period the mortality for 68 patients without an intramural coronary artery was 4.4％ (3/68).There was no statistical difference in mortalitv between the patients with and without an intramural coronary artery (P ＞ 0.05).Results 6 patients follow-up 4 to 47months.There was no late death.No intramural coronary artery obstruction was identified by cardiac computerize temography.All patients had normal ventricular function and were in NYHA class Ⅰ during follow-up.The intramural coronary artery is well known as a risk factor of ASO.Conclusion The technique of coronary transfer should be individually adapted to each anatomical situation.Individual technique for coronary transfer has excellent results.%目的 总结合并壁内型冠状动脉畸形患儿行动脉调转手术的即刻疗效及近、中期随访结果,探讨合并壁内型冠状动脉畸形行动脉调转手术中冠状动脉移植的有效方法.方法 2008年9月至2012年3月,成功完成动脉调转手术75例,其中术中发现合并壁内型冠状动脉7例.7例患儿年龄2天～1岁,平均2.4个月；体质量(4.3 ±2.2)kg.完全型大动脉转位合并室间隔缺损3例,室间隔完整型大动脉转位2例,Taussig-Bing畸形2例,合并主动脉弓离断1例,主动脉缩窄2例.5例采用双钮扣法,1例采用双钮扣法+壁内冠状动脉去顶,1例采用双钮扣法+壁内冠状动脉去顶化+心包补片扩大法.结果 1例同期深低温停循环下行主动脉弓离断矫治,壁内型左冠状动脉狭窄未去顶化,术后第1天死于低心排血量综合征.6例顺利出院.术后随访4 ～ 47个月,平均(17.3±16.5)个月.随访期间无死亡,患儿心脏CT均未见冠状动脉狭窄,末次心脏超声心动图均提示心功能正常,心功能Ⅰ级.结论合并壁内型冠状动脉动脉畸行患儿行调转手术风险较大,但选择合适的方法,采用个体化处理仍可以取得良好的效果.