right ventricular dysplasia


arrhythmogenic right ventricular cardiomyopathy

A non-ischaemic right ventricular cardiomyopathy, which is the single most common cause of sudden cardiac death attributable to heredity. It presents in children and young (male) adults as syncope due to ventricular arrhythmia or as sudden cardiac death in young athletes.
Clinical findings Strong familial tendency; asymptomatic, 10%; recurrent ventricular tachycardia with left bundle branch pattern, 45%; congestive heart failure, 25%; heart murmur, 10%; sudden death, 5%. Usually presents with palpitations, syncope, symptomatic arrhythmias, or sudden death during exercise in a young person.
Diagnosis EKG T wave inversion in V1–V3; 2-D and M mode echocardiography may be useful.
Management Beta-blockers, ablation with 90% initial success rate but 60% recurrence, implantable cardioverter-defibrillators, treatment of heart failure when it develops.

right ventricular dysplasia

Arrhythmogenic right ventricular dysplasia, right ventricular cardiomyopathy Cardiology An idiopathic form of right ventricular cardiomyopathy Clinical Strong familial tendency with variable infiltration or replacement of myocardium by fat and/or fibrous tissue Findings 10% are asymptomatic, recurrent ventricular tachycardia with left bundle branch pattern–45%, CHF–25%, heart murmur–10%, sudden death–5% Diagnosis T wave inversion in V1-V3 of the EKGl. See Apoptosis, Cardiomyopathy.