Abstract
Arrhythmogenic cardiomyopathies are a heterogeneous group of pathological conditions that give rise to myocardial dysfunction with an increased risk for atrial or ventricular arrhythmias. Inherited defects in cardiomyocyte proteins in the sarcomeric contractile apparatus, the cytoskeleton and desmosomal cell-cell contact junctions are becoming recognized increasingly as major causes of sudden cardiac death in the general population. Animal models have been developed for the systematic dissection of the genetic pathways involved in the pathogenesis of arrhythmogenic cardiomyopathies. This review presents an overview of current animal models for arrhythmogenic right ventricular cardiomyopathy (ARVC), hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) associated with cardiac arrhythmias and sudden cardiac death.