Determining the Natural and “Unnatural” History of Anomalous Aortic Origin of a Coronary Artery with an Interarterial Course

Doctor's Name: 
Julie A. Brothers, M.D.
Children’s Hospital of Philadelphia

Anomalous aortic origin of a coronary artery that runs between the two great arteries 
( AAOCA ) is a rare heart anomaly that is associated with a high risk of sudden death. The increased risk of sudden death appears to be greatest during or just after exercise, among otherwise healthy children and young adults, and is the second leading cause of sudden death in children and young adults participating in sports. The greatest challenge for doctors is diagnosing AAOCA because many people do not have any complaints. When they do, they mostly complain of chest pain, palpitations, dizziness or fainting during or just after exercise. There is a debate among doctors regarding how best to treat a child or young adult with AAOCA. While most agree that surgery is necessary if the child has evidence of decreased blood flow to the heart tissue, what is unclear is how to treat children and young adults who have AAOCA but who do not have complaints or evidence of reduced blood flow to the heart.

The goal is to establish a multi-institutional registry of children and young adults with AAOCA to determine which treatment options ( i.e. surgery or medical management ) are best in order to reduce their risk of decreased blood flow to the heart while allowing the child to ultimately lead a normal quality of life. Because AAOCA is rare, this can only be accomplished through the collaboration of multiple hospitals and institutions.

This project will be carried out through participating member institutions of the Congenital Heart Surgeons Society ( CHSS ), with the data stored and analyzed at the CHSS Data Center. The CHSS is a group of approximately surgeons from 60 hospitals in the United States, Canada and South America. They all share a common interest in treatment and management of congenital heart malformations and are committed to research in this area. The CHSS Data Center collects patient-related clinical information from patient charts and contacts children and their families to obtain follow-up information. These studies are very and help doctors to improve upon their evaluation, treatment and management of their patients.

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