Use of mouse models to evaluate novel therapeutic approaches to treatment of arrhythmogenic right ventricular cardiomyopathy the future is now.

Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy that is characterized by ventricular arrhythmias, an increased risk of sudden death, and abnormalities of right ventricular (RV) structure and function. The pathological hallmark of ARVD is myocyte loss with fibro-fatty replacement. Since the first detailed clinical description of the disorder in 1982, significant advances have been made in our understanding of all aspects of this disease (1–3). Particular focus has been placed on optimizing the approach to diagnosis of ARVD (4).Over the last decade, mutations in several desmosomal proteins have been identified as the genetic basis of ARVD (5–7). On the basis