Sudden cardiac death in early repolarization syndrome despite detailed evaluation for syncope: a case report

Early repolarization syndrome (ERS) has typically been regarded as a benign electrocardiographic variant; however, recent studies have demonstrated its association with ventricular fibrillation (VF) and sudden cardiac death (SCD) in specific patients. Risk stratification poses significant challenges due to the high prevalence of early repolarization patterns in healthy individuals and the low incidence of malignant arrhythmias.

We report a case of a 30-year-old man with no significant medical history who presented with unexplained syncope and demonstrated J-wave abnormalities in the lateral leads. Since the patient did not meet guideline-based criteria for implantable cardioverter-defibrillator (ICD) implantation, he underwent management with an insertable cardiac monitor (ICM) for long-term rhythm surveillance. Six months later, the patient suffered SCD secondary to VF, which the ICM confirmed.

This retrospective review revealed several subtle indicators of arrhythmic risk, including nocturnal J-wave augmentation and pilsicainide-induced J-point elevation. This case highlights the limitations of current ERS risk-stratification strategies and underscores the need to consider non-ICD preventive approaches, such as enhanced risk communication and strategic installation of an automated external defibrillator for patients who do not meet ICD therapy criteria but remain at potential arrhythmic risk.