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Arrhythmogenic Right Ventricular Cardiomyopathy : Clinical Course and Predictors of Arrhythmic Risk

Published on Dec 13, 2016in Journal of the American College of Cardiology18.639
· DOI :10.1016/j.jacc.2016.09.951
Andrea Mazzanti12
Estimated H-index: 12
,
Kevin Ng2
Estimated H-index: 2
+ 16 AuthorsSilvia G. Priori87
Estimated H-index: 87
(UNIPV: University of Pavia)
Sources
Abstract
Abstract Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a leading cause of sudden cardiac death, but its progression over time and predictors of arrhythmias are still being defined. Objectives This study sought to describe the clinical course of ARVC and occurrence of life-threatening arrhythmic events (LAE) and cardiovascular mortality; identify risk factors associated with increased LAE risk; and define the response to therapy. Methods We determined the clinical course of 301 consecutive patients with ARVC using the Kaplan-Meier method adjusted to avoid the bias of delayed entry. Predictors of LAE over 5.8 years of follow-up were determined with Cox multivariable analysis. Treatment efficacy was assessed comparing LAE rates during matched time intervals. Results A first LAE occurred in 1.5 per 100 person-years between birth and age 20 years, in 4.0 per 100 person-years between ages 21 and 40 years, and in 2.4 per 100 person-years between ages 41 and 60 years. Cumulative probability of a first LAE at follow-up was 14% at 5 years, 23% at 10 years, and 30% at 15 years. Higher risk of LAE was predicted by atrial fibrillation (hazard ratio [HR]: 4.38; p = 0.002), syncope (HR: 3.36; p  Conclusions The high risk of life-threatening arrhythmias in patients with ARVC spans from adolescence to advanced age, reaching its peak between ages 21 and 40 years. Atrial fibrillation, syncope, participation in strenuous exercise after the diagnosis of ARVC, hemodynamically tolerated sustained monomorphic ventricular tachycardia, and male sex predicted lethal arrhythmias at follow-up. The lack of efficacy of antiarrhythmic therapy and the life-saving role of the implantable cardioverter-defibrillator highlight the importance of risk stratification for patient management.
  • References (29)
  • Citations (47)
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References29
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#1Pasquale Santangeli (HUP: Hospital of the University of Pennsylvania)H-Index: 30
#2Erica S. Zado (HUP: Hospital of the University of Pennsylvania)H-Index: 47
Last. Francis E. Marchlinski (HUP: Hospital of the University of Pennsylvania)H-Index: 92
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Background— Catheter ablation of ventricular tachycardia (VT) in arrhythmogenic right ventricular cardiomyopathy improves short-term VT-free survival. We sought to determine the long-term outcomes of VT control and need for antiarrhythmic drug therapy after endocardial (ENDO) and adjuvant epicardial (EPI) substrate modification in patients with arrhythmogenic right ventricular cardiomyopathy. Methods and Results— We examined 62 consecutive patients with Task Force criteria for arrhythmogenic rig...
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Authors/Task Force Members: Silvia G. Priori* (Chairperson) (Italy), Carina Blomstrom-Lundqvist* (Co-chairperson) (Sweden), Andrea Mazzanti† (Italy), Nico Blom (The Netherlands), Martin Borggrefe (Germany), John Camm (UK), Perry Mark Elliott (UK), Donna Fitzsimons (UK), Robert Hatala (Slovakia), Gerhard Hindricks (Germany), Paulus Kirchhof (UK/Germany), Keld Kjeldsen (Denmark), Karl-Heinz Kuck (Germany), Antonio Hernandez-Madrid (Spain), Nikolaos Nikolaou (Greece), Tone M. Norekval (Norway), Chr...
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#3Silvia G. PrioriH-Index: 77
Last. D. J. Van VeldhuisenH-Index: 116
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ACC : American College of Cardiology ACE : angiotensin-converting enzyme ACS : acute coronary syndrome AF : atrial fibrillation AGNES : Arrhythmia Genetics in the Netherlands AHA : American Heart Association AMIOVIRT : AMIOdarone Versus Implantable cardioverter-defibrillator:
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#1Domenico Corrado (UNIPD: University of Padua)H-Index: 78
#2Thomas WichterH-Index: 53
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Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inheritable heart muscle disease that predominantly affects the right ventricle (RV) and predisposes to ventricular arrhythmias and sudden cardiac death (SCD).1–17 In the last three decades, there have been a significant number of studies defining the pathogenesis, genetic aspects, and clinical manifestations of the disease (See ‘Etiology, pathogenesis, diagnosis and natural history’ in the online-only Data Supplement). In ...
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Background— Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a progressive cardiomyopathy. We aimed to define long-term outcome in a transatlantic cohort of 1001 individuals. Methods and Results— Clinical and genetic characteristics and follow-up data of ARVD/C index-patients (n=439, fulfilling of 2010 criteria in all) and family members (n=562) were assessed. Mutations were identified in 276 index-patients (63%). Index-patients presented predominantly with sustained ventric...
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