儿童及青少年冠状动脉异常起源于对侧冠状窦的诊断与治疗

Diagnosis and treatment of anomalous aortic origin of a coronary artery from the opposite sinus in children and adolescents

目的探讨冠状动脉异常起源于对侧冠状窦的临床特点、诊断及处理方法，提高临床医师对冠状动脉异常起源于对侧冠状窦的认识。方法选择2012年1月至2016年12月广东省人民医院心儿科收治的冠状动脉异常起源于对侧冠状窦患儿11例，男8例，女3例；年龄（10．1±5．6）岁。所有患儿均行肌酸激酶同工酶（CK—MB）、肌钙蛋白I、体表心电图、经胸超声心动图、心脏多层螺旋CT检查；大于6岁病例行平板运动心电图检查。结果11例病例经超声心动图及心脏多层螺旋CT检查确诊，左冠状动脉起源于右冠状窦5例，右冠状动脉起源于左冠状窦6例。11例中5例有运动性晕厥，其中左冠状动脉起源于右冠状窦4例，右冠状动脉起源于左冠状窦1例，病变冠状动脉均为主动脉壁内走行；晕厥后24h内CK—MB、肌钙蛋白I均有明显增高，心电图提示心肌缺血。该5例晕厥病例全部行冠状动脉去顶手术，其余6例未手术处理冠脉病变，予限制体育活动，严密随访。随访期间，所有患儿未再出现运动后晕厥及胸痛表现，无提示心肌缺血的检查结果出现。结论部分冠状动脉异常起源于对侧冠状窦的病例可导致运动性心源性晕厥，对运动后晕厥或胸痛的青少年患者，超声心动图检查时应仔细观察冠状动脉的起源及走行情况。一旦确诊应避免剧烈活动，对于合并冠状动脉壁内走行及有症状的患者应及时手术治疗。

Objective To observe the clinical characteristics and improve the diagnosis and treatment of anomalous aortic origin of a coronary artery from the opposite sinus （AAOCA） in children and adolescents. Methods Between January 2012 and December 2016,11 children/adolescents [ 8 were boys,3 were girls, age was （10.1 ± 5.6） years old] diagnosed with AAOCA were retrospectively reviewed at Department Pediatric Cordiology, Guangdong General Hospital. The clinical features, transthoracic echocardiography （ TTE）, computed tomography images, electro- cardiogram （ECG） ,cardinc troponin I（CTnI） and creatinine kinase - MB（ CK - MB） were analyzed. Results The di- agnosis of AAOCA was confirmed by TTE and computed tomography images in all 11 cases. Left coronary artery origina- ted from the right sinus in 5 patients, and right artery coronary originated from the left sinus in 6 cases. Five patients with intramural course had the history of exercise - related syncope. Of these 5 cases with exercise - related syncope ,4 cases were diagnosed as left coronary artery originated from the right sinus and 1 case was diagnosed as right artery coronary originated from the left sinus. ECG, CTnI and CK - MB revealed acute myocardial ischemia in these patients with exercise - related syncope. Unroofing procedures were undergone in these 5 patients. The remaining 6 patients had no syncope and symptoms of cardia ischemia, sports activities were restricted and follow - ups were counseled in these 6 patients. There was no sudden death events later, and none of the patients demonstrated any evidence of new myocardial ischemia in the follow -up study. Conclusions AAOCA can be associated with syncope and myocardial ischemia in children and adolescents. The correct diagnosis of AAOCA requires a very high index of suspicion. Sports activities restriction and follow - up should be counseled in cases with AAOCA. Unroofing procedures should be performed in symptomatic cases with AAOCA and an intramural course.