Arrhythmogenic Right Ventricular Cardiomyopathy

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Author(s) J.S.S.G. de Jong, MD
Moderator J.S.S.G. de Jong, MD
Supervisor
some notes about authorship
ECG with an epsilon wave in V1
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. [1] 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.[2]

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.

Diagnosis ARVC is a difficult diagnosis to make. Therefore, the European Society of Cardiology has created a list of diagnositc criteria for the diagnosis of ARVC[2] (see table).

Major diagnostic criteria for Aritmogenic Right Ventricular Cardiomyopathy[2]
  • 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


Treatment focuses on avoiding complications.[3]

  • Medication:
    • anti-arrithmics: Sotalol better than Amiodarone.
    • ACE-inhibitors to prevent cardiac remodelling
  • ICD implantation is recommended for the prevention of sudden cardiac death in patients with ARVC with documented sustained VT or VF who are receiving chronic optimal medical therapy.
  • ICD] implantation can be considered for the prevention of sudden cardiac death in patients with ARVC with extensive disease, including those with left ventricular involvement, 1 or more affected family member with SCD, or undiagnosed syncope when ventricular tachycardia or ventricular Fibrillation has not been excluded as the cause of syncope, who are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 y.
  • Radiofrequency ablation can be useful as adjunctive therapy in management of patients with ARVC with recurrent ventricular tachycardia, despite optimal antiarrhythmic drug therapy.

Referenties

Error fetching PMID 8142187:
Error fetching PMID 10768917:
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  1. Error fetching PMID 10768917: [Corrado]
  2. Error fetching PMID 8142187: [McKenna1994]
  3. Error fetching PMID 16949478: [ACC2006]

All Medline abstracts: PubMed | HubMed