Key result
Isoproterenol infusion suppresses J waves and mitigates VF in a patient with SCN5A-mutated ERS.
Why the study?
The management of ventricular fibrillation storms in early repolarization syndrome, particularly with SCN5A mutations, and the role of isoproterenol remain unclear.
Does isoproterenol infusion mitigate ventricular fibrillation storm in a patient with early repolarization syndrome and SCN5A mutation?
Case Report (n=1)
Does isoproterenol infusion mitigate ventricular fibrillation storm in a patient with early repolarization syndrome and SCN5A mutation?
Isoproterenol infusion can successfully manage ventricular fibrillation storms in patients with early repolarization syndrome and SCN5A mutations.
May inform refractory VF management in early repolarization syndrome; hypothesis-generating, requires prospective validation.
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.
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Kim et al. (2025) conducted a case report in Ventricular fibrillation storm in early repolarization syndrome with SCN5A mutation (n=1). Isoproterenol vs. Conventional therapy was evaluated on Suppression of J wave and mitigation of ventricular fibrillation episodes. Isoproterenol infusion successfully suppressed J waves and mitigated ventricular fibrillation episodes in a patient with early repolarization syndrome and an SCN5A mutation.
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