Introduction

While coronary artery anomalies (CAAs) are relatively rare overall, they are one of the most common types of congenital heart disease.1 2 Multiple classification schemes have been proposed, typically taking into account abnormalities of vessel origin, course, number and ventricular myocardium supply.3 4 These anomalies can occur in otherwise structurally normal hearts or associated with other types of congenital heart disease. While many CAAs are not associated with untoward outcomes, some may have a more concerning clinical course resulting in presentation with sudden cardiac death (SCD).5 6 Unfortunately, this dramatic presentation may be the first presenting symptom, though patients may have had prior symptoms that were not linked to the underlying potentially fatal diagnosis.5 This review will focus on the evaluation and management of CAA among adolescents and young adults with otherwise structurally normal hearts, primarily focusing on anomalous aortic origin of a coronary artery (AAOCA) at or above the opposite sinus of Valsalva, the second leading cause of SCD in the young.6

AAOCA may involve the right coronary artery (AAORCA) originating from the left (opposite anterior) sinus of Valsalva, the left coronary originating from the right (opposite anterior) sinus of Valsalva (AAOLCA), or, more rarely, the origin of a coronary artery from or near the ‘non-coronary’ (posterior) sinus.7 They are typically also categorised into the following groups based on their course: interarterial, subpulmonic (also called intraseptal or intraconal), pre-pulmonic, retroaortic and retrocardiac (figure 1). Based on this anatomical definition, lesions have historically been classified as normal variants without increased risk of mortality (typically including those variants without an …