EARLY REPOLARIZATION SYNDROME

Early repolarization syndrome (ERS) refers to a specific electrocardiographic anomaly in the electrocardiogram (ECG), known as the early repolarization pattern (ERP). This pattern is characterized by a positive deflection (J wave) that appears immediately after the QRS complex, particularly in inferolateral ECG leads. While the early repolarization pattern is relatively common (1–13%) and is usually considered benign in healthy, asymptomatic young individuals and athletes, there is growing evidence suggesting a potential link to severe cardiac arrhythmias and sudden cardiac death.

The link between ERP and malignant arrhythmias is further supported by J-wave amplification before the onset of ventricular fibrillation (VF), VF storms, and premature ventricular contractions (PVCs). Clinical studies have shown that among patients who survived idiopathic ventricular fibrillation, about 31% exhibited an ERP, compared to only 5% in a control group without heart disease.

The role of genetics in the context of ERS is an active area of research. Studies indicate that ERP shows a degree of heritability and is over-represented in families of individuals who survived unexplained cardiac arrest, as well as in families with a history of sudden cardiac death. Early genetic studies have identified variants, particularly in the SCN5A gene, that are associated with ERS. These variants, specifically those that slow cardiac conduction and may increase the risk of malignant arrhythmias.

References

Wilde et al. 2022, Europace 24:1307