目的　探讨先天性冠状动脉瘘的临床诊断特点。方法　回顾性分析有完整病史资料并经手术治疗的小儿先天性冠状动脉瘘 2 1例。结果　 2 1例中右冠状动脉瘘 1 5例占 71 4 % ,左冠状动脉瘘 6例占 2 8 6% ,1 9例引流到右心系统占 90 5% ,2例引流到左心系统占 9 5% ;合并其他畸形 3例占 1 4 3 %。结论　先天性冠状动脉瘘最重要的临床诊断特征是心前区异常位置的连续性杂音 ,杂音的性质与分流部位、压力相关。彩色多普勒血流显像仪和升主动脉或冠状动脉造影能够明确诊断 ,可显示迂曲扩张的冠状血管与其引流的心腔部位。 Objective To investigate the clinical diagnosis of congenital coronary fistula. Methods A retrospective analysis of 21 cases of pediatric congenital coronary fistula with complete history data and surgically treated. Results Fifteen 71% of the right coronary fistulas were found in 21 cases, while 6 cases of the left coronary artery fistulas accounted for 286%. Nineteen cases of drainage to the right heart system accounted for 90.5%. Two cases of drainage to the left ventricular system accounted for 9 5%; 3 cases with other malformations accounted for 14.3%. Conclusions The most important clinical diagnosis of congenital coronary artery fistula is the continuous murmur of anomalous location in precordial area. The nature of murmur is related to the location of shunt and pressure. Color Doppler flow imaging and ascending aorta or coronary angiography can confirm the diagnosis, can show tortuous dilation of the coronary vessels and drainage of the heart chamber.