Abstract
BACKGROUND: Patients with congenital long QT syndrome (LQTS) are at increased risk for sudden cardiac death. CASE SUMMARY: We present the case of a young woman with a history of syncopal episodes triggered by emotional stress and menstruation who experienced recurrent ventricular arrhythmias requiring implantable cardioverter-defibrillator shocks. She was diagnosed with LQTS type 2 and found to have an anomalous right coronary artery from the left coronary cusp with malignant features. DISCUSSION: This clinical case highlights the complex interplay between LQTS type 2, sex hormonal influences, and an anomalous right coronary artery in a young female patient with recurrent ventricular arrhythmias.