Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review
Open Access
- 1 January 2013
- journal article
- Published by SAGE Publications in Clinical Medicine Insights: Cardiology
- Vol. 7, CMC.S10940-114
- https://doi.org/10.4137/cmc.s10940
Abstract
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic form of cardiomyopathy (CM) usually transmitted with an autosomal dominant pattern. It primary affects the right ventricle (RV), but may involve the left ventricle (LV) and culminate in biventricular heart failure (HF), life threatening ventricular arrhythmias and sudden cardiac death (SCD). It accounts for ll%-22% of cases of SCD in the young athlete population. Pathologically is characterized by myocardial atrophy, fibrofatty replacement and chamber dilation. Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations. Therefore consensus diagnostic criteria have been developed and combined electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI) and myocardial biopsy. Early detection, family screening and risk stratification are the cornerstones in the diagnostic evaluation. Implantable cardioverter-defibrillator (ICD) implantation, ablative procedures and heart transplantation are currently the main therapeutic options.Keywords
This publication has 119 references indexed in Scilit:
- Incidence and Predictors of Implantable Cardioverter-Defibrillator Therapy in Patients With Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Undergoing Implantable Cardioverter-Defibrillator Implantation for Primary PreventionJournal of the American College of Cardiology, 2011
- Value of the signal-averaged electrocardiogram in arrhythmogenic right ventricular cardiomyopathy/dysplasiaHeart Rhythm, 2010
- Efficacy of Antiarrhythmic Drugs in Arrhythmogenic Right Ventricular CardiomyopathyJournal of the American College of Cardiology, 2009
- Arrhythmogenic Right Ventricular Cardiomyopathy Type 5 Is a Fully Penetrant, Lethal Arrhythmic Disorder Caused by a Missense Mutation in the TMEM43 GeneAmerican Journal of Human Genetics, 2008
- ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac DeathJournal of the American College of Cardiology, 2006
- DSG2 Mutations Contribute to Arrhythmogenic Right Ventricular Dysplasia/CardiomyopathyAmerican Journal of Human Genetics, 2006
- Right Ventricular Cardiomyopathy and Sudden Death in Young PeopleNew England Journal of Medicine, 1988
- Arrhythmogenic right ventricular dysplasia: a generalized cardiomyopathy?Circulation, 1983
- Total disconnection of the right ventricular free wall: surgical treatment of right ventricular tachycardia associated with right ventricular dysplasia.Circulation, 1983
- Right ventricular dysplasia: a report of 24 adult cases.Cell Metabolism, 1982