Successful Isoproterenol Treatment for Ventricular Fibrillation Storm in Early Repolarization Syndrome With SCN5A Mutation.

A 58-year-old man experienced a ventricular fibrillation storm with prominent inferolateral J waves and was diagnosed with early repolarization syndrome. Initial coronary angiography showed no significant stenosis and the other evaluations for ventricular fibrillation were unremarkable. Despite conventional therapy for ventricular fibrillation, it recurred. Isoproterenol infusion suppressed the J wave and successfully mitigated ventricular fibrillation episodes. This case highlights the role of isoproterenol in managing early repolarization syndrome-related ventricular fibrillation storms and the possible pathogenic link between SCN5A mutations and J wave syndromes.