arrhythmogenic right ventricular dysplasia
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Related to arrhythmogenic right ventricular dysplasia: Arrhythmogenic right ventricular cardiomyopathy
arrhythmogenic right ventricular cardiomyopathyA 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.
arrhythmogenic right ventricular dysplasiaRight ventricular dysplasia, see there.
arrhythmogenic right ventricular dysplasiaAbbreviation: ARVD
A rare degenerative disease of desmosomes within heart muscle that may produce life-threatening cardiac arrhythmias. It is a cause of sudden death or ventricular tachycardia in athletes. The dysplasia is diagnosed by a combination of tests (specific angiographic, electrocardiographic, echocardiographic, MRI, and biopsy or necropsy criteria). Some forms of the disease are autosomal dominantly inherited. Synonym: arrhythmogenic right ventricular cardiomyopathy; arrhythmogenic right ventricular dysplasia/cardiomyopathy
See also: dysplasia