Arrhythmogenic right ventricular dysplasia (ARVD): an overlooked and underdiagnosed condition?

We present three cases of arrhythmogenic right ventricular dysplasia causing or contributing to sudden death. These cases serve to highlight the range of clinical presentations and histological features seen in this rare and poorly understood condition. Arrhythmogenic right ventricular dysplasia (ARVD) is a cardiomyopathy characterized by a normal or mild increase in heart weight with right-sided heart dilatation. Typically a proportion of the right ventricular muscle is replaced by fat and fibrous tissue. Clinically, the condition is characterized by episodes of ventricular tachycardia and global dyskinesia of the right ventricle. It may cause ventricular fibrillation and sudden death, especially in young persons engaged in strenuous exercise or who experience severe emotional outburst. The aetiology of the condition is unknown, although it has been noted to show familial clustering in some cases.