Patient Registry Studies Anomalous Aortic Origin of a Coronary Artery (AAOCA) in Children and Young Adults

Researchers from The Children’s Hospital of Philadelphia helped to establish the Congenital Heart Surgeons’ Society’s multi-institutional registry of children and young adults with anomalous aortic origin of a coronary artery (AAOCA). AAOCA is a rare heart anomaly that is associated with an increased risk of sudden death. The purpose of the registry is to study outcomes, course of the disease, and risk of sudden death in children with AAOCA.

Researchers are also working to determine if there are differences in anatomy and risk of sudden death between children who have symptoms and those who do not. We’re also looking to see if there is decreased morbidity or risk of sudden death over time in children who have surgery compared with those who do not. This knowledge will help us make informed treatment decisions and impact our recommendations regarding surgery versus exercise restriction versus no exercise restriction.

While relatively rare, AAOCA is the second leading cause of sudden death from heart disease in children and young adults participating in sports. Among otherwise healthy children and young adults, the increased risk of sudden death appears to be greatest during or just after exercise. The risk of death is much higher when the left coronary artery arises from the wrong location; when the right coronary arises from the wrong location, the risk is significantly lower.

Diagnosing AAOCA is very challenging because many people do not have any obvious symptoms or complaints. When they do, they most commonly complain of chest pain, palpitations, dizziness or fainting during or just after exercise. 

Because AAOCA is rare, we can only accomplish the goals of this study through the collaboration of multiple hospitals and institutions. We have over 450 youth and young adults participating to date, and we are currently enrolling additional patients.

If you’re interested in learning more about the registry or other coronary anomaly research, please contact Tonia Morrison, 267-426-5420. If you choose to participate, we will review your child’s medical records and provide a questionnaire for you to fill out every year.

Related publications

Wittlieb-Weber CA, Brothers JA. Coronary Artery Anomalies: Current Recognition and Treatment Strategies. Update on Recent Progress. Curr Cardiovasc Risk Rep. 8:395-398. July 2014. Read the abstract.

Lorber R, Srivastava S, Wilder TJ, McIntyre S, DeCampli WM, Williams WG, Frommelt P, Parness IA, Blackstone EH, Jacobs M, Mertens L, Brothers J, Herlon R, AAOCA Working Group of the Congenital Heart Surgeons Society. Anomalous Aortic Origin of Coronary Arteries in the Young: Echocardiographic Evaluation with Surgical Correlation. JACC Cardiovasc Imaging. 2015 Nov;8(11):1239-49. doi: 10.1016/j.jcmg.2015.04.027. Read the abstract.

This registry has been generously funded by: Michael H. Ludwig Memorial Foundation; Anthony’s Heroes for Hearts; Children’s Heart Foundation; Cardiac Center at The Children’s Hospital of Philadelphia; Cardiovascular and Critical Care Research Center, Children’s Hospitals and Clinics of Minnesota; Cardiac Surgery Research and Teaching Fund, Hospital for Sick Children, Toronto