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.
Highlights
Up to 10% of all sudden cardiac deaths are caused by primary electrical disorders or ion channel diseases
ICD implantation may be considered in symptomatic family members of early repolarization syndrome patients with a history of syncope in the presence of ST segment elevation >1mm in 2 or more inferior or lateral leads
The incidence of ventricular fibrillation episodes and electrical storm is relatively common in early repolarization syndrome patients after ICD implantation
Summary
Up to 10% of all sudden cardiac deaths are caused by primary electrical disorders or ion channel diseases. The identification of genetic mutations affecting these ion channels has opened a new area of translational research in cardiac electrophysiology [1, 2]. An early repolarization pattern had been considered as a benign finding, it is frequently observed on surface ECGs, characterized by J-point and ST segment elevation in 2 or more contiguous leads. The early repolarization pattern has increasingly attracted attention as it has been reported as a risk to idiopathic ventricular fibrillation and sudden cardiac death in case-control studies, characterized as early repolarization syndrome. This review provides a historic, epidemiologic and pathophysiologic background and describes diagnostic and therapeutic approaches in the treatment of early repolarization syndrome
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