目的：探讨先天性冠状动脉瘘的诊断及手术治疗方法。方法：全组22例术前均经心电图、X线、选择性冠状动脉造影、B超检查确诊，并施行外科手术治疗。结果：本组19例术前行选择性冠状动脉造影，17例行B超检查，对照符合率 94.2%。选择性冠状动脉造影与手术对照符合率 99.8%。21例术后恢复顺利，出院时心前区杂音消失。术后(19/21例)随访15月至23年，心功能I级。死亡1例，死于术后低心排综合征。结论：近年来随着超声心动图检查经验的积累及技术提高，冠状动脉瘘的诊断并不困难，但确诊目前仍依赖于选择性冠状动脉造影或核磁共振心脏扫描。手术难度不大，但应注意修补瘘口，避免残余分流，如冠状动脉瘘远端仍有血管分支时，应注意冠状动脉远端的血液供应。

OBJECTIVE: To study the diagnosis and surgical treatment of congenital coronary artery fistula. METHODS: Twenty two children with this disorder confirmed by X ray, selective angiography and echocardiography, were treated surgically.RESULTS: The coincidence rate of diagnosis by selective angiography (n=19) and echocardiography (n=17) was 94.2% , while that by selective angiography and operation was 99.8% . The operation was successful in 21 patients, and only one died from the low cardiac output syndrome. Of the 21 cases, 19 kept cardiac functional class I during a 15 month to 23 year follow up. CONCLUSIONS: With technical skills and experience of echocardiography, diagnosis of congenital artery fistula is easy. Nevertheless definite diagnosis has to be confirmed by selective angiography or MRI. Surgical treatment is very effective and safe for those who have a definitive diagnosis. In the operation, one should ensure closure of the coronary artery fistula outlet to avoid residual shunting and ensure adequate blood supply to the distal coronary artery.