Arrhythmogenic cardiomyopathies includes arrhythmogenic dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and arrhythmogenic left dominant cardiomyopathy. This mixture of arrhythmogenic syndromes can be caused by filamin C mutations.

Method: The question is whether standard ECG can differentiate between these three forms of cardiomyopathies. Typical features of arrhythmogenic cardiomyopathy are epsilon waves, T-wave inversions in right precordial leads, but also low voltage in limb leads, typical features in lead aVR and an amplitude of negative T-waves in lead V1 of 2mm or more. ECG’s of arrhythmogenic dilated cardomyopathy (n=7), arrhythmogenic left ventricular cardiomyopathy (n=2) and arrhythmogenic right ventricular cardiomyopathy (n=2) were analyzed.

Results: Arrhythmogenic dilated cardiomyopathy presented by ECG with left bundle branch block in 4 cases and left ventricular hypertrophy and typical features in lead aVR, T inversions in lead V1 of 1mm or in 3 cases. Arrhythmogenic left dominant cardiomyopathy were characterized by low voltgae in limb leads and T wave inversions or flattening in inferolateral leads. Arrhythmogenic right ventricular cardiomyopathy was presented by either complete right bundle branch block with a T-wave inversion in lead V1 of 2mm or more. The other ECG presented with T inversions in right precordial leads (amplitude in lead V1 of 2mm or more), epsilon waves, and typical features in lead aVR.

Conclusions: Standard ECG is an excellent parameter for the differentiation of arrhythmogenic cardiomyopathies (ADCM, ALVC, ARVC).