Arrythmogenic right ventricular dysplasia/cardiomyopathy

Arrythmogenic right ventricular dysplasia/cardiomyopathy (ARVD) is a familial cardiomyopathy characterised clinically by right ventricular (RV) dysfunction as well as ventricular tachycardia1-4 and histopathologically by fibro-fatty replacement of the myocardium.5 Left ventricular (LV) involvement can occur and appears to correlate with increased disease severity.3,4 Owing to the complexity of the disease, Task Force Criteria for diagnosis of ARVD were drawn up in 1994 and revised in 2010.6,7 Cardiovascular magnetic resonance (CMR) findings are now included in the list of major and minor criteria and currently play an important role in establishing the diagnosis of ARVD (see Table I). CMR is extremely valuable for delineation of right ventricular (RV) anatomy and function as well as for characterising the composition of the RV wall, especially regarding the presence of fatty and/or fibrous tissue.