Application of current diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy in every day clinical practice.

Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiac muscle disease associated with sudden cardiac death, ventricular arrhythmias and cardiac failure. It is primarily a disease of the desmosome, with mutations in desmosomal protein genes identified in approximately 50% of affected individuals. Mutations result in fibrofatty replacement of cardiomyocytes, aneurysm formation and dilatation of the right and often the left ventricle. The clinical diagnosis of ARVC is based upon complex criteria that consider pathology, genetics and clinical presentation. This review describes the application of the revised criteria for ARVC in everyday practice and illustrates the requirement for continued modification to improve their sensitivity and specificity.