BACKGROUND Anomalous aortic origin of a coronary artery (CA) is the second leading cause of sudden cardiac death in young athletes. Management is controversial and longitudinal follow-up data are sparse. We aim to evaluate outcomes in a prospective study of anomalous aortic origin of CA patients following a standardized algorithm. METHODS Patients with anomalous aortic origin of a CA were followed prospectively from December 2012 to April 2017. All patients were evaluated following a standardized algorithm, and data were reviewed by a dedicated multidisciplinary team. Assessment of myocardial perfusion was performed using stress imaging. High-risk patients (high-risk anatomy-anomalous left CA from the opposite sinus, presence of intramurality, abnormal ostium-and symptoms or evidence of myocardial ischemia) were offered surgery or exercise restriction (if deemed high risk for surgical intervention). Univariate and multivariable analyses were used to determine predictors of high risk. RESULTS Of 201 patients evaluated, 163 met inclusion criteria: 116 anomalous right CA (71%), 25 anomalous left CA (15%), 17 single CA (10%), and 5 anomalous circumflex CA (3%). Patients presented as an incidental finding (n=80, 49%), with exertional (n=31, 21%) and nonexertional (n=32, 20%) symptoms and following sudden cardiac arrest/shock (n=5, 3%). Eighty-two patients (50.3%) were considered high risk. Predictors of high risk were older age at diagnosis, black race, intramural course, and exertional syncope. Most patients (82%) are allowed unrestrictive sports activities. Forty-seven patients had surgery (11 anomalous left CA and 36 anomalous right CA), 3 (6.4%) remained restricted from sports activities. All patients are alive at a median follow-up of 1.6 (interquartile range, 0.7-2.8) years. CONCLUSIONS In this prospective cohort of patients with anomalous aortic origin of a CA, most have remained free of exercise restrictions. Development of a multidisciplinary team has allowed a consistent approach and may have implications in risk stratification and long-term prognosis.

中文翻译：

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遵循标准化方法的冠状动脉主动脉异常起源的结果。

背景技术冠状动脉（CA）的主动脉异常起源是年轻运动员心脏猝死的第二个主要原因。管理存在争议，纵向随访数据稀疏。我们旨在根据标准化算法对CA患者主动脉异常起源的前瞻性研究评估结果。方法从2012年12月至2017年4月，对CA主动脉异常的患者进行前瞻性随访。所有患者均按照标准化算法进行评估，并由专门的多学科团队对数据进行审查。使用压力显像评估心肌灌注。高危患者（高危解剖结构-异常窦窦左CA，存在壁内壁，为手术或运动受限（如果认为手术干预的高风险）提供了异常的口和症状或心肌缺血的证据。使用单变量和多变量分析来确定高风险的预测因子。结果在评估的201例患者中，有163例符合纳入标准：右CA异常116例（71％），左CA异常25例（15％），单CA 17例异常（10％）和5个反旋支CA异常（3％）。表现为偶然发现（n = 80，49％），有劳累性（n = 31，21％）和非劳累性（n = 32，20％）症状且在心脏骤停/休克后出现的患者（n = 5，3％） ）。八十二名患者（50.3％）被认为是高危人群。高风险的预测因素是诊断时年龄较大，黑人种族，壁内病程和劳累性晕厥。大多数患者（82％）被允许进行无限制运动。47例患者接受了手术（左CA 11异常，右CA 36异常），其中3例（6.4％）不能参加运动。所有患者均存活1.6年（四分位间距为0.7-2.8）年。结论在这个CA主动脉异常起源患者的前瞻性队列中，大多数患者仍然没有运动限制。多学科团队的发展允许采取一致的方法，并且可能对风险分层和长期预后产生影响。