Evolving Guidelines for Student Athletes with Anomalous Coronary Arteries

In a recent issue of the World Journal for Pediatric and Congenital Heart Surgery, Children's Hospital of Philadelphia Cardiologists Julie Brothers, MD, FAAP, and Stephen Paridon, MD, discuss new formal AHA/ACC guidelines for children with anomalous aortic origin of a coronary artery (AAOCA) who want to participate in competitive sports.

The authors detail the evolution of these guidelines over the past 30 years, as well as the need for further research to better assess the risk of sudden cardiac death in patients with specific forms of AAOCA.

AAOCA with an interarterial course is one of the most common causes of sudden cardiac death from structural heart disease in young athletes. The risk of death appears to be highest during or just after high-intensity competitive sports. Because of this, sports participation has traditionally been restricted once the diagnosis of interarterial AAOCA is made.

Interarterial AAOCA can occur when either the left main coronary artery arises from, or above, the right sinus of Valsalva (AAOLCA) or the right coronary artery arises from, or above, the left sinus of Valsalva (AAORCA). The distinction between these two is important because the risk of myocardial ischemia and sudden cardiac death occurring during competitive athletics is significantly higher with AAOLCA than AAORCA.

Over the past 30 years, formal competitive guidelines for sports participation in those with AAOCA have evolved, with the most recent ones published by the American Heart Association/American College of Cardiology (AHA/ACC) in late 2015. 

Prior to the most recent guidelines, youth with either AAORCA or AAOLCA were restricted from competitive sports, irrespective of signs or symptoms concerning for myocardial ischemia.  If they underwent surgical repair, children could return to sports three months after surgery if an exercise stress test was normal. There was no differentiation made between those with AAORCA and AAOLCA.

For the first time, the most recent AHA/ACC Scientific Statement makes a distinction between the high-risk interarterial AAOLCA, from the much lower-risk AAORCA diagnosis. With this distinction made, the guidelines now allow for an asymptomatic child with AAORCA — who has a normal exercise stress test — to participate in competitive sports after adequate family counseling regarding the risk of sudden cardiac death.

While this is significant progress, the use of the word “asymptomatic” is too broad, Brothers and Paridon say. “Unless a patient has symptoms that are convincingly due to ischemia or arrhythmia, children with non-cardiac symptoms should not be restricted from sports,” the cardiologists assert.

Also, new stress imaging modalities should be considered in addition to the exercise stress test alone to increase sensitivity for inducible myocardial ischemia. “More data are needed to help us better risk stratify patients, notably those with AAORCA, to help us decide who should be allowed to play competitive sports and who should be referred for surgical repair,” the authors say.

Drs. Brothers and Paridon both have years of experience working with children with anomalous aortic origin of a coronary artery at Children's Hospital of Philadelphia. Dr. Brothers is the medical director of the Coronary Anomaly Management Program (CAMP), and Dr. Paridon is director of CHOP's Exercise Physiology Laboratory.

Brothers JA, Paridon SM. The New AHA/ACC Guidelines for Competitive Sports Participation in Young Athletes with Anomalous Coronary Arteries: The Evolution of Change. World J Pediatr Congenit Heart Surg. 2016 Mar;7(2):241-4. doi: 10.1177/2150135116634315.

Contributed by: Julie Brothers, MD, FAAP