Arrhythmogenic Right Ventricular Cardiomyopathy
|Author(s)||J.S.S.G. de Jong, MD|
|Moderator||J.S.S.G. de Jong, MD|
|some notes about authorship|
Arrhythmogenic Right Ventricular Cardiomyopathy, (ARVC, or ARVD: Arrhythmogenic Right Ventricular Disease) is characterized by fatty replacement and fibrosis of the heart. Most commonly 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 more advanced ages right ventricular pump failure can occur.
The diagnosis is based on major and minor criteria, as published by the European Society of Cardiology.
ARVC is a progressive disease. The incidence is estimated to be 1:3.000-1:10.000. Manifestations are usually seen in teenagers. 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 diagnostic criteria for the diagnosis of ARVC (see table).
|Major diagnostic criteria for Arrhythmogenic Right Ventricular Cardiomyopathy|
|Diagnostic criteria that can be diagnosed on the ECG|
In 2009 three more criteria were added:
- Terminal activation duration of QRS≥55ms
- ventricular tachycardia with left bundle branch block morphology and superior axis, and
- genetic criteria.
Treatment focuses on avoiding complications.
- Anti-arrhythmics: 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 (Sudden Cardiac Death), 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.
|Original Task Force Criteria||Revised Task Force Criteria|
|I. Global or regional dysfunction and structural alterations∗|
By 2D echo:
By RV angiography:
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