Familial arrhythmogenic right ventricular dysplasia-4 (ARVD4) is characterized by progressive degeneration of the myocardium of the right ventricle, with focal necrosis of muscle cells followed by adipose and connective tissue replacement. The left ventricle may be partially involved. Patches of replacement tissue result in electrical instability and arrhythmias. Patients experience syncopal episodes, and sudden death may occur (summary by Rampazzo et al., 1997).
For phenotypic information and evidence of genetic heterogeneity in this disorder, see ARVD1 (107970). [from
OMIM]