Arrhythmogenic right ventricular dysplasia (ARVD)
form of cardiomyopathy that causes ventricular arrhythmias.
There is a wide clinical spectrum of arrhythmogenic right ventricular cardiomyopathies/dysplasia.
They appear to be the result of one or possibly two factors:
- replacement of most of the right ventricular myocardium by fat
- genetic susceptibility to environmental agents (myocarditis)
Etiology:
a genetic, inherited, heart disease.
Manifestation:
may cause ventricular arrhythmias.
- a rare but important cause of sudden arrhythmic death in young, otherwise healthy persons
- a subtle cause of congestive heart failure.
Diagnosis and Diagnostics:
3 standard diagnostic features: (positively identifies majority of individuals)
- echocardiography
- nuclear medicine
- magnetic resonance imaging
Definitive diagnostics:
- With the exception of full-thickness histological examination of the right ventricular free wall, contrast ventriculography remains the most definitive standard for a positive diagnosis.
Treatment and Management:
- Implantable defibrillators, used alone or in combination with drug therapy are most common.
- Drug therapy.
- Catheter or surgical ablative techniques of affected area or tissue.
- Treatment and management of congestive heart failure, if present.
- Heart transplant is exceptionally rare.
Chemla, D, Fontaine, G, Fontaliran, F, Frank, R, Hébert, J,L, Lecarpentier, Y, and Zenati, O. 1999. ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA. Annual Review of Medicine. 50: 17-35.
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