Early Repolarization Syndrome: Diagnostic and Therapeutic Approach

Abstract

An early repolarization pattern can be observed in 1% up to 13% of the overall population. Whereas, this pattern was associated with a benign outcome for many years, several more recent studies demonstrated an association between early repolarization and sudden cardiac death, so-called early repolarization syndrome. In early repolarization syndrome patients, current imbalances between epi- and endo-cardial layers result in dispersion of de- and repolarization. As a consequence, J waves or ST segment elevations can be observed on these patients' surface ECGs as manifestations of those current imbalances. Whereas, an early repolarization pattern is relatively frequently found on surface ECGs in the overall population, the majority of individuals presenting with an early repolarization pattern will remain asymptomatic and the isolated presence of an early repolarization pattern does not require further intervention. The mismatch between frequently found early repolarization patterns in the overall population, low incidences of sudden cardiac deaths related to early repolarization syndrome, but fatal, grave consequences in affected patients remains a clinical challenge. More precise tools for risk stratification and identification of this minority of patients, who will experience events, remain a clinical need. This review summarizes the epidemiologic, pathophysiologic and diagnostic background and presents therapeutic options of early repolarization syndrome.

Keywords: ICD implantation; J wave; early repolarization syndrome; idiopathic ventricular fibrillation; sudden cardiac death.

Figure 1

(A) ECG example of a patient Nr. 1 with early repolarization syndrome. Two frequent clinical PVCs, arising from the septal basal RV, are documented. (B) The clinical PVC of early repolarization syndrome patient Nr. 1 induces an episode of ventricular fibrillation.

Figure 2

ECG of patient Nr. 2 presenting with early repolarization syndrome. J point elevation is predominantly present in lead v3.

Figure 3

(A) An episode of non-sustained ventricular fibrillation, which occurred during baseline ECG recording of early repolarization syndrome patient Nr. 3. The VF episode was induced by the patient's early clinical PVC. (B) ECG recording of patient Nr. 3. The clinical PVC now induces an episode of sustained ventricular fibrillation.

Figure 4

ECG recording of patient Nr. 4 shows a predominant early repolarization pattern in the inferior leads.