In case reports early repolarization (ER) is associated with arrhythmogenic cardiomyopathy. In smaller case analyses ER is a discussable risk marker. Furthermore,  the association with inversions of right precordial T-waves should be analysed.

Method: In a cohort of 360 patients with typical ESC/ISFC criteria of arrhythmogenic cardiomyopathy (176 males, mean age 47.3 +/- 13.7 years) the presentation with ER and the risk assessment for sudden cardiac arrest was analyzed.

Results:  A total of 113 cases (31%) with inferior (22%), inferolateral (3%), and lateral (6%) notching or slurring was presented as typical signs of early repolarization syndrome. Together with the results of ajmaline challenge it is in close relation with the number of right precodial T-wave inversions. Early repolarization does not identify patients at risk for sudden cardiac arrest. In single cases the risk was increased in cases with ST elevation in early repolarization pattern as primary electrical disease.

Conclusions: The number of early repolarization pattern in typical arrhythmogenic cardiomyopathy is increased and relates to the number of right precordial T-wave inversions of 55% in this cohort. Early repolarization is generally not a risk marker, but in single cases overlapping primary electrical disease can be documented.