“…Usually it starts from right ventricular subepicardial layers towards the endocardium. Initial changes are in the "triangle of dysplasia" (outflow tract, inflow tract and apex), but it may be diffuse involvement of both ventricles [1,2].A two base-pair deletion in the plakoglobin (cell adhesion protein) gene (Pk2157del2TG), which maps to17q21, has been identified as the cause of ND and provided evidence that the pathogenesis might be related to a defect in myocardial mechanical coupling. Defects in the linking sites of these proteins can interrupt the contiguous chain of cell adhesion, particularly under conditions of increased mechanical stress or stretch, leading to cell death, progressive loss of myocardium and fibro-fatty replacement [1][2][3][4].…”