Determining Risk of Sudden Cardiac Death in Children and Young Adults with Heart Disease

Girl Doing a Cardiac Stress TestSudden cardiac death (SCD) is a tragic event, especially when it occurs in young athletes. One of the leading causes of SCD in young athletes is congenital anomalies of the coronary arteries.

Coronary arteries are the blood vessels that supply blood and nutrients to the heart muscle. Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital anomaly associated with a risk of decreased blood flow to the heart and sudden death in children and young adults. This risk is most frequently associated with vigorous exercise.

Julie Brothers, MD, and her colleagues in the Coronary Anomaly Management Program (CAMP) are evaluating exercise stress test protocols for children with AAOCA in order to determine who is most at risk for sudden cardiac death. Understanding which children are most at risk for SCD can allow for greater customization of treatment. Physicians would potentially have a way to help determine who should have surgery due to increased risk versus who could potentially be permitted to participate in competitive sports.

Why understanding risk is important

One of the greatest challenges physicians face when treating children with AAOCA is the inability to adequately predict who is at increased risk for sudden cardiac death. In the absence of any signs or symptoms indicating decreased blood flow to the heart and with a normal standardized exercise stress test, the treatment of children with AAOCA is largely dependent on the physicians caring for them. Some patients are referred for surgery  while others are restricted from competitive sports. Both of these options have significant life-changing implications.

Dr. Brothers and her team are hoping to determine whether different exercise stress test (EST) protocols will result in symptoms or evidence of decreased blood flow to the heart in children and young adults with AAOCA. CHOP’s researchers hope that the use of these new protocols will help determine who is truly at risk for SCD.

The information from this study could be transformative in helping physicians understand how to best evaluate patients with AAOCA, and ultimately allow greater customization of treatment – which in turn could be standardized for other pediatric centers across the country.