VF Arrest at Rest in a Teenager With Anomalous Origin of Coronaries and DSG2 Variant

Sudden cardiac arrest (SCA) in adolescents is rare and often attributed to genetic or structural cardiac abnormalities. The differentials remain broad including anomalous aortic origin of a coronary artery (AAOCA).

A 16-year-old male experienced ventricular fibrillation arrest at rest. Imaging revealed anomalous aortic origin of the right and left coronary arteries (AAOCA) with high-risk features. Genetic testing identified a sequence variation in DSG2. The patient underwent surgical unroofing and was discharged with a wearable cardioverter-defibrillator. A subcutaneous defibrillator was implanted 8 weeks later. The patient remains well without further events.

This case highlights the diagnostic complexity when rare structural and potential genetic etiologies coexist, especially in the context of SCA at rest. The literature supports advanced imaging, surgical correction for AAOCA, and comprehensive genetic evaluation in pediatric SCA at rest.