Abstract

Arrhythmogenic cardiomyopathy (AC) is a hereditary disorder characterized by degeneration of cardiac myocytes and their subsequent replacement by fat and fibrous tissue primarily in the right ventricle. Our study aimed to systematically evaluate the impact of significant demographic, clinical, electrocardiographic, and echocardiographic factors in arrhythmic events in AC patients. MEDLINE and Cochrane library databases were manually searched without year or language restriction or any other limits until July 31, 2017. A pooled odds ratio with 95% confidence intervals was calculated for each of the risk factors. Our search retrieved 26 studies (n = 2680 patients, mean age: 37.9 years old, males: 51.9%) which were included in the quantitative synthesis. The most reliable predicting factors/parameters are the following: (1) male gender, (2) presyncope, (3) left ventricular dysfunction, (4) T-wave inversions in inferior leads, (5) proband status, (6) late potentials, (7) syncope, (8) inducibility at electrophysiological study, (9) right ventricular dysfunction, (10) epsilon waves, and (11) premature ventricular contractions greater than 1000/24 h. On the contrary, family history of sudden cardiac death, palpitations, premature ventricular contractions greater than 500/24 h, and T-wave inversions in right precordial leads fail to determine the outcome in this meta-analysis. In conclusion, multiple risk factors have been associated with arrhythmic events in AC patients. However, larger studies are needed to discriminate those patients who will benefit from implantable cardioverter defibrillators.

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