SummaryAtrial tachyarrhythmias (ATAs) occur in a significant proportion of Brugada syndrome (BrS) patients and are often an important cause of inappropriate shocks. The aim of this retrospective study was to evaluate the incidence of ATAs and ATA-induced inappropriate shocks in early repolarization syndrome (ERS) patients as compared to BrS patients.We analyzed data from 20 consecutive patients who were diagnosed with ERS and compared them with patients diagnosed with BrS (n = 31). Clinical and ICD interrogation data were collected and analyzed for all events with ICD shocks.Three patients had a history of atrial fibrillation (AF) prior to ICD implantation. One patient had AV reentrant tachycardia and was successfully ablated before ICD implantation. ATAs were newly diagnosed in 4 patients with no prior history of AF. There were no significant differences in gender, age, or left atrial diameter between ATA development. Four (20%) of 20 consecutive patients received inappropriate ICD shocks for ATAs. One suffered from repeat inappropriate shocks triggered by paroxysmal AF and received catheter ablation for AF.ATAs were not infrequent in patients with ERS and seemed to be related to inappropriate ICD therapy. Careful ICD programming is required to reduce ATA-related inappropriate ICD shock in patients with ERS. (Int Heart J 2017; 58: 43-49) Key words: Early repolarization syndrome, Brugada syndrome, Sudden cardiac death E arly repolarization syndrome (ERS) has recently been uncovered as an important cause of sudden cardiac death (SCD) or life-threatening arrhythmias in patients with structurally normal hearts.1-3) Several basic studies reported that transmural or epicardial dispersion of action potentials in the early repolarization phase is responsible for the electrocardiographic phenotype of the ST segment and J wave elevation, suggesting a common electrophysiologic background between Brugada syndrome (BrS) and ERS. 4,5) A significant proportion of patients with BrS develop atrial tachyarrhythmias (ATAs), which are often an important cause of inappropriate shock. [6][7][8][9] There are many similarities between BrS and ERS in clinical and electrocardiographic characteristics.5) As the dysfunction of cardiac ion channels is not limited to the ventricular myocardium, it may be possible that electrical abnormalities coexist in the atrial and ventricular myocardium of patients with ERS. Gain or loss-of-function mutations in atrial ion channels may lead to shortening or prolongation of atrial action potential durations, resulting in increased dispersion of atrial repolarization or intra-atrial conduction time in patients with ERS. The aim of this retrospective study was to evaluate the incidence of ATAs and ATAinduced inappropriate shock in patients with ERS as compared to those with BrS.