Why Is There an Increased Risk for Sudden Cardiac Death in Patients With Early Repolarization Syndrome?

The last two decades have changed the viewpoint on early repolarization syndrome (ERS). The prevalence of the early repolarization pattern is variable and ranges between 3-24% depending upon age, gender, and criteria used for J-point upliftment from baseline (0.05mV vs. 1 mV). While this pattern was previously linked with a benign result, multiple recent investigations have found a link between early repolarization and Sudden Cardiac Death (SCD) by causing life-threatening arrhythmias like Ventricular tachycardia/Ventricular fibrillation, a condition known as early repolarization syndrome. The syndrome falls under a broader bracket of J wave syndromes, which can be caused by early repolarization or depolarization abnormalities. The characteristics of early repolarization that are considered high risk for Sudden Cardiac Death include the amplitude of J-point upliftment from baseline ( > 0.2 mV), Inferior-lateral location of Early Repolarization pattern, and horizontal and downsloping ST-segment. Patients with symptomatic early repolarisation patterns on ECG are more likely to have repeated cardiac episodes. Implantable Cardioverter-Defibrillator (ICD) implantation and isoproterenol are the recommended treatments in symptomatic patients. On the other hand, asymptomatic patients with early repolarization patterns are prevalent and have a better outcome. Risk categorization is still obscure in asymptomatic early repolarization patterns. This traditional review outlines the known knowledge of pathophysiology behind the increased risk of sudden cardiac death, risk stratification of patients with ERS, and the treatment guidelines for patients with ERS. Further prospective studies are recommended to elucidate the exact mechanism for ventricular arrhythmogenesis in ERS patients and to risk stratifying asymptomatic patients with ERS.