Clinical management of arrhythmogenic right ventricular cardiomyopathy: an update

Current Pharmaceutical Design
Federico MiglioreDomenico Corrado

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart muscle disease characterized by the peculiar right ventricular (RV) involvement. Distinctive pathologic features are myocardial atrophy and fibro-fatty replacement of the RV free wall, and clinical presentation is usually related to ventricular tachycardias with a left bundle branch block pattern or ventricular fibrillation leading to cardiac arrest, mostly in young people and athletes. Later in the disease evolution, progression and extension of RV muscle disease and left ventricular involvement may result in right or biventricular heart failure. In the 1994 an International Task Force proposed standardized diagnostic criteria designed to guarantee an adequate specificity and based on the presence of major and minor criteria encompassing electrocardiographic, arrhythmic, morphofunctional, histopathologic, and genetic factors; more recently, Task Force criteria have been modified to increase their diagnostic sensitivity. Retrospective analysis of clinical and pathologic series including fatal cases identified a series of risk factors such as malignant familial background, youthful age, previous syncope or cardiac arrest, competitive sport activity, severe...Continue Reading

Citations

Jan 15, 2015·International Journal of Cardiology·Javier Molina-Martin de NicolasFernando Arribas Ynsaurriaga
Jul 4, 2015·Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology·Alessandro ZorziDomenico Corrado
May 30, 2020·Journal of Cardiovascular Development and Disease·Tyler L StevensPeter J Mohler
Oct 22, 2016·Journal of Thoracic Imaging·Sandra L Castaños GutiérrezStefan L Zimmerman

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