Purpose of review 

To better understand the risk of sudden death from coronary anomalies.

Recent findings 

Most coronary anomalies are benign, but there are certain coronary anomalies that increase the risk of sudden cardiac death (SCD) in the young, notably anomalous aortic origin of a coronary artery from the wrong sinus. Previously, the risk of SCD attributed to certain coronary anomalies was felt to be quite high, as the risk assessment was based on autopsy series. Recent studies have shown that the risk attributed to anomalous coronary arteries is much lower than once believed. Risk appears to be highest with anomalous left coronary artery from the right sinus of Valsalva with interarterial course, notably when the young patient is participating in vigorous physical exertion, such as with competitive sports.

Summary 

Treatment strategies may include exercise restriction or surgical repair. Management decisions should be based on a complete understanding of the risk for SCD because of anomalous coronary arteries. Future directions should focus on better methods to risk stratify these pediatric patients.