Interv Akut Kardiol. 2014;13(2):87-91

Arrhythmogenic right ventricular cardiomyopathy

Luděk Haman

¹ . interní kardioangiologická klinika, FN v Hradci Králové a UK v Praze, LF v Hradci Králové

Arrhythmogenic right ventricular cardiomyopathy is a genetic form of cardiomyopathy usually transmitted with an autosomal dominant

pattern. It primary affects the right ventricle, but may involve also left ventricle and lead to biventricular heart failure, ventricular tachyarrhythmia’s

and sudden cardiac death. Pathologically is characterized by myocardial atrophy and fibro-fatty replacement. Diagnosis is difficult

due to broad spectrum of phenotypic variations, therefore diagnostic criteria combined history, electrocardiography, echocardiography and

magnetic resonance imaging have been developed. Basic diagnostic evaluation is early detection, risk stratification and family screening.

Main therapeutic goal is sudden cardiac death prevention – treatment of arrhythmias and cardioverter-defibrilator implantation.

Keywords: arrhythmogenic right ventricular cardiomyopathy, sudden cardiac death, ventricular tachycardia

Published: May 15, 2014  Show citation