目的　探讨电子束CT(EBT)对先天性心脏病 (先心病 )房室大血管连接畸形的诊断价值。方法　经手术及造影证实的 16例房室大血管连接畸形先心病 ,均经EBT和超声心动图及彩色多普勒血流检查。 9例经心血管造影检查 ,8例有手术结果。以手术、造影结果的诊断为标准 ,分析EBT对房、室与大血管连接畸形的诊断价值。结果　 16例房室大血管连接畸形先心病中肺动脉闭锁 7例 ,完全性大动脉转位 1例 ,部分型大动脉转位 3例 ,右室双出口 1例 ,部分型肺静脉异位引流 3例 ,完全性肺静脉异位引流 1例。EBT正确诊断 15例 ,误诊 1例 ,诊断准确率为 93 .8%,另将别的畸形过多诊断成此类疾病 3例。超声心动图正确诊断 11例 ,准确率 68.8%。EBT优于超声心动图 ,但二者无统计学差异 ( χ2 =3 .2 82 ,Ρ >0 .0 5 )。结论　EBT对先心病房室大血管连接畸形的诊断较超声心动图有一定优势 ,对于部分型肺静脉异位引流等病例甚至优于心血管造影检查 Objective To investigate the diagnostic value of electron beam computed tomography (EBT) in diagnosing congenital heart disease (CHD). Methods Sixteen cases of atrioventricular anomalies congenital atrioventricular anastomosis were confirmed by surgery and angiography. All of them were examined by EBT, echocardiography and color Doppler flow imaging. Nine patients underwent angiography and eight had surgical findings. With the diagnosis of surgery and contrast as the standard, the diagnostic value of EBT for the abnormality of the connection between the room and the room and the large blood vessel was analyzed. Results In 16 cases of atrioventricular anomalies, 16 cases had pulmonary atresia in congenital heart disease, 1 case of complete aortic transposition, 3 cases of partial aortic transposition, 1 case of right ventricular double outlet and 3 cases of partial anomalous pulmonary venous drainage. Anomalous pulmonary venous drainage in 1 case. EBT correct diagnosis of 15 cases, 1 case of misdiagnosis, diagnostic accuracy of 93.8%, while the other malformations too much diagnosis of such diseases in 3 cases. Echocardiography correct diagnosis of 11 cases, the accuracy rate of 68.8%. EBT was superior to echocardiography, but there was no significant difference (χ2 = 3.222, P> 0.05). Conclusions EBT has some advantages over echocardiography in the diagnosis of major vascular malformations in congenital atrioventricular compartments. It is even superior to cardiovascular angiography in some cases of ectopic drainage of pulmonary veins