Abstract
Early repolarization syndrome (ERS) was originally considered a normal variant with benign outcome. However, recent studies have demonstrated that it can be linked to a considerable risk of life-threatening arrhythmias and sudden cardiac death. We report a case with an extraordinary, extremely malignant clinical expression of ERS refractory to all antiarrhythmic drugs including quinidine. This case demonstrates real-time changes of dynamic electrocardiogram (ECG) preceding a polymorphic ventricular tachycardia (VT)-ventricular fibrillation (VF) and possible external factors triggering arrhythmia onset. Implantable cardioverter-defibrillator (ICD) function was terminated 6 months after implantation due to multiple-incessant electrical storm (ES). Catheter ablation was the definite treatment of this malignant entity.