title: Risk stratification for sudden death in arrhythmogenic right ventricular cardiomyopathy.
abstract: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) is an uncommon but increasingly recognized inherited cardiomyopathy that is associated with malignant ventricular arrhythmias and sudden cardiac death, particularly in young individuals. The implantable cardioverter-defibrillator (ICD) is widely regarded as the only treatment modality with evidence to support improved survival in patients with ARVC and secondary prevention indications. In contrast, there is no universally accepted risk stratification scheme to guide ICD therapy for primary prevention against sudden cardiac death. Potential benefits must be weighed against the considerable risks of complications and inappropriate shocks in this young patient population. This article tackles the challenges of risk stratification for sudden cardiac death in ARVC and critically appraises available evidence for various proposed risk factors. The authors' over-arching objective is to provide the clinician with evidence-based guidance to inform decisions regarding the selection of appropriate candidates with ARVC for ICD therapy.
authors.affiliation: From the Electrophysiology Service, Montreal Heart Institute and Department of Medicine, Université de Montréal, 5000 Bélanger St. E., Montreal, Quebec, H1T 1C8, Canada.
authors.forename: Julia
authors.initials: J
authors.lastname: Cadrin-Tourigny
authors.forename: Rafik
authors.initials: R
authors.lastname: Tadros
authors.forename: Mario
authors.initials: M
authors.lastname: Talajic
authors.forename: Lena
authors.initials: L
authors.lastname: Rivard
authors.forename: Sylvia
authors.initials: S
authors.lastname: Abadir
authors.forename: Paul
authors.initials: P
authors.lastname: Khairy
mesh_terms: D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control*; D017147:Defibrillators, Implantable / Q000009:adverse effects; D006801:Humans; D018579:Patient Selection; D011322:Primary Prevention / Q000379:methods; D018570:Risk Assessment; D012307:Risk Factors; D055502:Secondary Prevention / Q000379:methods
keywords: arrhythmogenic right ventricular cardiomyopathy/dysplasia; cardiac genetics; implantable cardioverter-defibrillators; sudden cardiac death; therapy; ventricular arrhythmias