BACKGROUND Intraseptal anomalous aortic origin of a coronary artery is considered a benign condition. However, there have been case reports of patients with myocardial ischemia, arrhythmia, and sudden cardiac death. The purpose of this study was to determine the clinical presentation, myocardial perfusion on provocative stress testing, and management of children with anomalous aortic origin of a coronary artery with an intraseptal course in a prospective cohort. METHODS Patients with anomalous aortic origin of a coronary artery and intraseptal course were prospectively enrolled from December 2012 to May 2019, evaluated, and managed following a standardized algorithm. Myocardial perfusion was assessed using stress imaging. Fractional flow reserve was performed in patients with myocardial hypoperfusion on noninvasive testing. Exercise restriction, β-blockers, and surgical intervention were discussed with the families. RESULTS Eighteen patients (female 6, 33.3%), who presented with no symptoms (10, 55.6%), nonexertional (4, 22.2%), and exertional symptoms (4, 22.2%), were enrolled at a median age of 12.4 years (0.3-15.9). Perfusion imaging was performed in 14/18 (77.8%) and was abnormal in 7/14 (50%); fractional flow reserve was positive in 5/8 (62.5%). All 4 patients with exertional symptoms and 3/10 (30%) with no or nonexertional symptoms had myocardial hypoperfusion. Coronary artery bypass grafting was performed in a 4-year-old patient; β-blocker and exercise restriction were recommended in 4 patients not suitable for surgery. One patient had nonexertional chest pain and 17 were symptom-free at median follow-up of 2.5 years (0.2-7.1). CONCLUSIONS Up to 50% of patients with intraseptal anomalous aortic origin of a coronary artery had inducible myocardial hypoperfusion during noninvasive provocative testing. Long-term follow-up is necessary to understand the natural history of this rare anomaly.

中文翻译：

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患有冠状动脉内隔过程的主动脉异常的患儿的心肌缺血。

背景技术冠状动脉的房间隔内主动脉起源被认为是良性疾病。但是，有病例报告有心肌缺血，心律不齐和心源性猝死。这项研究的目的是确定前瞻性队列研究的临床表现，在刺激性压力测试中进行的心肌灌注以及对患有冠状动脉主动脉异常的儿童的治疗。方法从2012年12月至2019年5月前瞻性入选具有冠状动脉主动脉异常和间隔隔过程的患者，按照标准算法进行评估和管理。使用压力显像评估心肌灌注。在无创检查中对心肌灌注不足的患者进行分数血流储备。运动限制 与家人讨论了β受体阻滞剂和手术干预。结果18例患者（无症状（10％，55.6％），无劳累性（4％，22.2％）和劳累性症状（4％，22.2％）无症状（10％，55.6％），平均年龄为12.4岁。 （0.3-15.9）。14/18（77.8％）进行灌注成像，7/14（50％）异常。流量储备分数为5/8（62.5％）为正。所有4名具有运动症状的患者和3/10（30％）没有或没有运动症状的患者均出现了心肌灌注不足。一名4岁患者进行了冠状动脉旁路移植术；建议4例不适合手术的患者使用β受体阻滞剂和运动限制。在中位随访2.5年（0.2-7.1）时，一名患者出现了非运动性胸痛，其中17例无症状。结论在无创性激发试验中，多达50％的患有冠状动脉间隔内异常的主动脉起源的患者具有可诱导的心肌灌注不足。要了解这种罕见异常的自然历史，必须进行长期随访。