AAOCA is the second leading cause of sudden cardiac death in the young. Many questions remain regarding the morphologic and physiologic mechanisms leading to myocardial ischemia and a possible sudden event. Patients with AAOCA present as an incidental finding at least half of the time, advanced imaging is essential to define anatomic characteristics of this lesion, assessment of myocardial perfusion with stress cardiac magnetic resonance imaging is feasible and contributes greatly to risk stratification. Specific subsets of this population require invasive assessment of coronary flow with measurement of fractional flow reserve. Surgical intervention can be safely performed though long term data on impact to prevent sudden events remains to be determined. Our institution developed one of the largest Registries holding data in over 500 patients with prospective enrollment and follow up. Data demonstrates that assessment of myocardial ischemia is essential for risk stratification, certain anatomic types previously thought benign can lead to ischemia and require intervention, and most patients continue to exercise with no restrictions at medium term follow up. Newest strategies for noninvasive assessment of FFR and impact of morphologic variables utilizing advanced CTA imaging, 3D printing, and computational fluids dynamics are promising tools to continue to unravel risk factors in this lesion. Optimal risk stratification in AAOCA is yet to be defined, though substantial strides are being made with a standardized approach to the evaluation and management of these patients. Continued collaboration among centers and the scientific community will positively impact patients and families living with AAOCA

Silvana Molossi, MD, PhD, graduated from Federal University of Pelotas, Brazil and completed a clinical and research fellowship in pediatric cardiology at the Hospital For Sick Children in Toronto, Canada. She received a doctorate in Pathology and Cardiovascular Sciences from the University of Toronto. She served as a Professor of Pediatrics on the School of Medicine of the Federal University of Rio Grande do Sul, Brazil. She served for seven years as the Chief of the Pediatric Cardiology Division and Cardiac Pediatric Intensive Care Unit at Complexo Hospitalar Santa Casa, Porto Alegre, Brazil. She joined the Faculty at Baylor College of Medicine as Associate Professor, Department of Pediatrics, Section of Cardiology, Texas Children’s Hospital, in March 2011.  She has been involved in screening young athletes for conditions known to be high risk for sudden cardiac death for over 15 years and holds the Vice President of Science and Research position for the Championship Hearts Foundation, also being on the Medical Advisory Board for the National Coronary Anomalies Foundation, both non-profit organizations. She has served as the Co-Chair of the Sports and Exercise Cardiology Council and Section for the American College of Cardiology from 2013-2016. In the past 9 years, she has developed the first Coronary Artery Anomalies Program in the US dedicated to the care of young individuals with coronary artery anomalies, particularly anomalous aortic origin of a coronary artery, the second leading cause of sudden death in the youth. She serves as the Medical Director of the Coronary Anomalies Program and Associate Chief for the Section of Cardiology at Texas Children’s Hospital.