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目的：探讨18例少见冠状动脉(冠脉)畸形特征及评价选择性冠脉造影在其诊断中的作用。方法：对17例患者进行冠脉造影，13例患者进行手术纠正治疗。另1例二维超声心动图误诊为动脉导管未闭，术中证实为右冠脉-右室瘘。结果：18例患者中共发现有9种冠脉畸形，包括冠脉起源异常2例；左冠脉回旋支缺如1例；左、右冠脉-肺动脉瘘3例；左冠脉-肺动脉瘘3例；右冠脉-右心房瘘3例；右冠脉-右室瘘2例；右冠脉-静脉瘘1例；左冠脉-右室瘘1例；左冠脉-左心室瘘2例。其中13例患者经手术治疗得到再证实。结论：冠脉畸形是少见的先天性血管畸形，选择性冠脉造影是确诊的重要手段，需与先天性心脏病和冠心病心绞痛鉴别，术后效果良好。“,”Objective:To determine the characteristics of eighteen cases withrare coronary artery malformations（CAM） and evaluate the role of selective coronary angiography in the final diagnoses of CAM. Method:Seventeen of eighteen cases with rare coronary artery malformation underwent selective coronary angiography. Fourteen cases were treated by cardiothoracic operation，of which one was misregarded as patent ductus arteriosus by echocardiography before procedure and was confirmed was right cornary artery-right ventricle fistula during procedure. Result:Nine kinds of rare coronary aretry malfromation were found in the 18 cases, including 2 cases with anomalous origin of coronary artery, 1 case with absence of left circumflex coronary artery, 3 cases with left and right coronary-pulmonary fistula, 3 cases with left coronary-pulmonary fistula, 3 cases with right coronary artery-right atrium fistula, 2 cases with right coronary artery-right ventricle fistula, 1 case with right coronary artery-venous fistula, 1 case with left coronary artery-right ventricle fistula and 2 cases with left coronary artery-left ventricle fistula, of which 13 cases with rare coronary artery malformation were confirmed during surgery procedure. Conclusion:CAM are rare congenital cardiovascular malformation and should be told from other congenital heart diseases or coronary heart diseases. Selective coronary angiography is an important measure for final diagnoses of CAM.