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Arrhythmogenic right ventricular cardiomyopathy: From genetics to diagnostic and therapeutic challenges

World Journal of Cardiology 2014年第12期6卷 1234-1244页

作者： bruno pinamonti Francesca Brun Luisa Mestroni Gianfranco Sinagra Cardiovascular Department Ospedali Riuniti of Trieste34100Trieste Italy Cardiovascular Institute and Adult Medical Genetics Program University of Colorado Denver AMC Aurora CO 80045-2507 United States

Arrhythmogenic right ventricular cardiomyopathy(ARVC) is a genetic disease characterized by myocyte loss and fibro-fatty tissue replacement. Diagnosis of ARVC remains a clinical challenge mainly at its early stages and in patients with minimal echocardiographic right ventricular(RV) abnormalities. ARVC shares some common features with other cardiac diseases, such as RV outflow ventricular tachycardia, Brugada syndrome, and myocarditis, due to arrhythmic expressivity and biventricular involvement. The identification of ARVC can be often challenging, because of the heterogeneous clinical presentation, highly variable intra- and inter-family expressivity and incomplete penetrance. This genotypephenotype "plasticity" is largely unexplained. A familial history of ARVC is present in 30% to 50% of cases, and the disease is considered a genetic cardiomyopathy, usually inherited in an autosomal dominant pattern with variable penetrance and expressivity; in addition, autosomal recessive forms have been reported(Naxos disease and Carvajal syndrome). Diagnosis of ARVC relays on a scoring system, with major or minorcriteria on the Revised Task Force Criteria. Implantable cardioverter defibrillators(ICDs) are increasingly utilized in patients with ARVC who have survived sudden death(SD)(secondary prevention). However, there are few data available to help identifying ARVC patients in whom the prophylactic implantation of an ICD is truly warranted. Prevention of SD is the primary goal of management. Pharmacologic treatment of arrhythmias, catheter ablation of ventricular tachycardia, and ICD are the mainstay of treatment of ARVC.

关键词： Arrhythmogenic right ventricular cardiomyopathy Sudden cardiac death Risk stratification Genetic Implantable cardioverter-defibrillator

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