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:

  1. replacement of most of the right ventricular myocardium by fat 
  2. genetic susceptibility to environmental agents (myocarditis)

Etiology:

a genetic, inherited, heart disease.

Manifestation:

may cause ventricular arrhythmias.

  1. a rare but important cause of sudden arrhythmic death in young, otherwise healthy persons
  2. a subtle cause of congestive heart failure.

Diagnosis and Diagnostics:

3 standard diagnostic features: (positively identifies majority of individuals)

  1. echocardiography
  2. nuclear medicine
  3. magnetic resonance imaging

Definitive diagnostics:

  1. 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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