Arrhythmogenic Right Ventricular Cardiomyopathy

Author(s)	J.S.S.G. de Jong, MD
Moderator	J.S.S.G. de Jong, MD
Supervisor
some notes about authorship

What is ARVC?

A section throught the heart of a ARVC patient. (A) Transmural fatty replacement of the right ventricular free wall. (B) Myocardial atrophy is confined to the right ventricle and substantially spares the interventricular septum as well as the left ventricular free wall. Corrado Reproduced with permission from BMJ Publishing Group Ltd.

Arrythmogenic Right Ventricular Cardiomyopathy, (ARVC, or ARVD: Arrythmogenic Right Ventricular Disease) is characterized by fatty replacement and fibrosis of the heart. Especially the right ventricle apex and outflow tract are involved. However the left ventricle can be affected to.

As a result of the fatty replacement and fibrosis, ventricular arrhythmias are common in this disease and can lead to palpitations, syncope and sudden death. At older age right ventricular pump failure can occur.

The diagnosis is based on major and minor criteria, as published by the European Society of Cardiology.McKenna1994

ARVC is a progressive disease. The incidence is estimated to be 1:3.000-1:10.000. Manifestations is usually during teenage. Although the diagnosis is more often made in athletes, sports are not thought to have a causative relationship with the disease. ARVD can occur in families; more than 9 different chromosomal defects have been described, most often with autosomal dominant inheritance.

One unique form of ARVD, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.

The European Society of Cardiology has created a list of diagnositc criteria for the diagnosis of ARVC.

Major diagnostic criteria for Aritmogenic Right Ventricular CardiomyopathyMcKenna1994
Familial disease confirmed at necroscopy or surgery Severe dilatation and reduciton of right ventricular ejection fraction with no (or only mild) LV impairment Localised irhgt ventricular aneurysms (akinetic or diskinetic areas with diastolic bulging) Severe segmental dilataion of the right ventricle Fibrofatty replacement of myocardium on endomyocardial biopsy
Diagnostic criteria that can be diagnosed on the ECG
(major) Epsilon wave or localised prolongation (>110ms) of the QRS complex in right precordial leads (V1-V3) (minor) Inverted T waves in right precordial leads (V2 and V3) (people aged more than 12 yr; in absence of RBBB (minor) Late potentials (signal averaged ECG) (minor) Left bundle branch block type ventricular tachycardia (sutained and non-sustained) (ECG, Holter, exercise testing (minor) Frequent ventricular extrasystoles (more than 1000/24h) (Holter)

Treatment

Treatment focusses on avoiding complications.ACC2006

• Medication:
  • anti-arrithmics: Amiodarone, Sotalol
  • ACE-inhibitors to prevent cardiac remodelling
• Preventive ICD placements in patients in whom optimal medical treatment did not prevent ventricular tachycardia or ventricular fibrillation. Other patients who qualify for ICD placement: patients with severe disease with left ventricular involvement, patients with more family members who died of ARVD, patients who had a syncope while on medication and in whom arrhythmias cannot be excluded as a cause.
• Radiofrequency ablation in patients with recurrent ventricular tachycardia.

Referenties

<biblio>

1. McKenna1994 pmid=8142187
2. Corrado pmid=10768917
3. ACC2006 pmid=16949478

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