• Anusha Jegatheeswaran, Paul J Devlin, Brian W McCrindle, William G Williams, Marshall L Jacobs, Eugene H Blackstone, William M DeCampli, Christopher A Caldarone, J William Gaynor, James K Kirklin, Richard O Lorber, Carlos M Mery, James D St Louis, Silvana Molossi, and Julie A Brothers.
• Division of Cardiac Surgery, Department of Surgery, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address: anusha.jegatheeswaran@utoronto.ca.
• J. Thorac. Cardiovasc. Surg. 2019 Sep 1; 158 (3): 822-834.e3.

ObjectivesWe sought to determine anatomic features associated with evidence of myocardial ischemia and sudden cardiac events (arrest or death) for patients with anomalous aortic origin of a coronary artery.MethodsWe enrolled 560 patients, less than or equal to 30 years, at diagnosis from 40 institutions. Ischemia was defined as the presence of exertional syncope, a sudden cardiac event (arrest/death), or abnormal investigation results. Data on detailed anatomic features were abstracted from echocardiography, computed tomography, magnetic resonance imaging, operative, and/or surgeon-completed reports.ResultsThere were 236 patients with negative ischemia test results, and 49 with evidence of ischemia (including 18 who presented with a sudden cardiac event); 275 asymptomatic patients who had not undergone provocative ischemia testing were excluded from primary analyses. Patients with ischemia (vs without), were more likely to have left anomalous coronary arteries (28/49 vs 46/236; P < .0001). Of patients with ischemia (vs without), those with anomalous left coronary arteries were more likely to have an intramural coronary artery course, or a high or slit-like coronary artery orifice. Of patients with ischemia (vs without), those with anomalous right coronary arteries were more likely to have a longer intramural course. Among patients with ischemia, the occurrence of sudden cardiac events was not shown to have any associated anatomic features.ConclusionsAnatomic features including coronary artery involved, intramural course and length, and orifice anomalies were associated with evidence of myocardial ischemia for patients with anomalous aortic origin of a coronary artery. These features might importantly inform risk stratification and decisions regarding surgical management.Copyright © 2019. Published by Elsevier Inc.

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