Arrhythmogenic right ventricle cardiomyopathy

Abstract

Arrhythmogenic cardiomyopathies are diseases of the heart muscle with a predisposition to arrhythmias not caused by hypertension, ischemia, or valvular disease. Within this group is arrhythmogenic right ventricular cardiomyopathy which is now also known to be left ventricular. This is a hereditary disease usually autosomal dominant and, in most cases, caused by a mutation in the desmosomes involved in cell union, this causes myocyte death and replacement of these by fibrofatty tissue when stressed. Predisposing to fatal arrhythmias and progression to heart failure mimicking dilated cardiomyopathy. The natural course of the classic disease is characterized by four phases, initially the occult phase manifested exclusively by fatal arrhythmias and sudden death occurs, then there is the electrical alteration in which there are alterations in the ECG and imaging tests, followed by right ventricular failure and finally biventricular failure. The diagnosis is confirmed using the diagnostic criteria proposed by the ITF, ideally with non-invasive tests or ultimately with invasive tests, then the risk must be stratified to define the treatment, for all patients the restriction of physical activity is essential and for most beta blockers are used, the decision to place an ICD (implantable cardioverter-defibrillator) and use of other antiarrhythmics or ablation is made according to the  characteristics of each patient For patients with suspicion but no confirmed diagnosis, close follow-up for early diagnosis is the most important thing.  The prognosis varies with a mortality of 0.08% to 3.6% per year due to heterogeneity in the presentation of the disease.