Abstract

Desmoglein-2 and Desmocollin-2 mutations cause different types of arrhythmogenic cardiomyopathy either dominant right, biventricular or dominant left forms. These two mutations present with myocardial cell necrosis and calcification [1,2]. Signs of electrocardiographic right ventricular hypertrophy can be found in more than 50% [3] besides typical ECG criteria of arrhythmogenic cardiomyopathy like right precordial epsilon waves and T-wave inversions [4], QRS fragmentation [5], localized right precordial QRS prolongation [6], terminal activation delay [7], and large Q-waves, small R-waves and T-wave inversion in lead aVR [8].

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