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目的:总结去顶术式治疗冠状动脉主动脉异常起源(AAOCA)壁内走行患者的效果。方法:回顾性分析2013年6月至2019年4月15例AAOCA患者的资料总结，其中2例因无明显症状及SCD高危因素而未行手术治疗，最终13例行去顶手术治疗的AAOCA患者纳入本研究并分析术后近中期结果。结果:全组患者(16.3±8.2)岁(7～35岁)，术前评估均为壁内走行解剖异常，其中左冠状动脉起源于右冠状动脉窦的患者8例(61.5%)，右冠状动脉起源于左冠状动脉窦5例(38.5%)。所有患者术前均有临床症状，其中首发症状为胸痛7例(53.8%)，晕厥5例(38.5%)，胸闷1例(7.7%)。主动脉阻断时间、中位体外循环时间及呼吸机辅助通气时间分别为(51.0±16.4)min (37～99 min)、82(74.0，97.5)min和6(4，15) h。围手术期及随访过程中无死亡病例。中位随访时间为22(10.0，39.5)个月，心功能Ⅲ～Ⅳ级的患者从术前5例减少为1例。结论:去顶术式是治疗AAOCA安全可靠的手术方式，可纠正AAOCA患者冠状动脉壁内走行的解剖异常，从而改善冠状动脉供血和心功能，有利于消除临床症状及降低潜在的心源性猝死风险。“,”Objective:Anomalous aortic origin of the coronary artery (AAOCA) has been associated with coronary ischemia, myocardial infarction, and sudden cardiac death (SCD). We review our experience for AAOCA patients with intramural course.Methods:This is a retrospective review of 15 patients from June 2010 to April 2019, 2 of them were asymptomatic and lack of evidence of increased risk of SCD and therefore excluded, the other 13 underwent unroof procedure were enrolled and analyzed for the early-mid surgical outcomes.Results:The mean age of the patients in this group was (16.3±8.2) years (7-35 years), all were intramural course subtype. Eight patients (61.5%) were anomalous left coronary artery (ALCA) and 5 (38.5%) were anomalous right coronary artery (ARCA). All patients were presented with clinical symptoms before surgery. Among them, 7 (53.8%) had onset symptom of chest pain, 5 (38.5%) had syncope, and 1 (7.7%) had chest tightness. The mean crossclamping time, median extracorporeal circulation time, and median mechanical assisted ventilation time were (51.0±16.4)min (37-99 min), 82(74.0, 97.5)min and 6(4, 15)h , respectively. There were no deaths during the perioperative period and during follow-up. The median follow-up time was 22 months (10.0, 39.5), and the number of patients with grade Ⅲ-Ⅳ cardiac function decreased from 5 cases to 1 case.Conclusion:Unroofing technique is a safe and reliable surgical method for AAOCA. It can correct the anatomical abnormalities of intracoronary artery wall in AAOCA patients, thereby improving coronary blood supply and cardiac function, which is conducive to eliminating clinical symptoms and reducing the risk of SCD.