Standard ECG is a very important diagnostic tool in arrhythmogenic cardiomyopathy, In 1994 McKenna et al. epsilon waves in right precordial leads and QRS prolongation (> 110 msec) of right precordial leads as major criteria . In the revised form 2010, epsilon waves and right precordial T-wave inversions appeared as major criteria and terminal activation delay a minor criterion . This criteria has a sensitivity of 97%, but specificity is reduced due to pulmonary embolism, RVOT ventricular tachycardia, sarcoidosis, andUhl anomaly .
In recent years another ECG criterion has been established: a typical appearance in lead aVR with a large Q wave, a small R wave, and a negative T wave with 2 mm or less . An additional negative T-wave in lead V1 of 2mm leads to high sensitivity and specificity. With a negative predictive value of 99% arrhythmogenic cardiomyopathy can be definitely excluded . A very interesting combination of two different diseases exists: In Emery-Dreifuss FHL1 mutation characterized by non-compaction left ventricle and arrhythmogenic right ventricular cardiomyopathy. Neither localized right precordial QRS prolongation nor terminal activation delay nor QRS prolongation are present, but typical appearance in lead aVR and significant T-wave inversion in lead V1 are positive [6, 7].
The findings suggest that typical appearance in lead aVR together of significant T-wave inversion in lead V1 (2 mm of more) is a better indicator of arrhythmogenic right ventricular cardiomyopathy than right precordial QRS prolongation.