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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 Cardiology18.64
· 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)
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)
References29
Newest
#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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#1Aditya Bhonsale (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 19
#2Judith A. Groeneweg (UU: Utrecht University)H-Index: 13
Last.Richard N.W. Hauer (UU: Utrecht University)H-Index: 49
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#1Judith A. Groeneweg (UU: Utrecht University)H-Index: 13
#2Aditya Bhonsale (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 19
Last.Hugh Calkins (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 104
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#1Christian F. Camm (University of Oxford)H-Index: 11
#2Cynthia Anne James (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 38
Last.Hugh Calkins (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 104
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#1Cristina Basso (UNIPD: University of Padua)H-Index: 82
#2Barbara Bauce (UNIPD: University of Padua)H-Index: 39
Last.Gaetano Thiene (UNIPD: University of Padua)H-Index: 94
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#1Jeffrey A. Towbin (UTHSC: University of Tennessee Health Science Center)H-Index: 120
#2William J. McKenna (UCL: University College London)H-Index: 109
Last.Wojciech Zareba (URMC: University of Rochester Medical Center)H-Index: 86
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#1Jeffrey A. Towbin (UTHSC: University of Tennessee Health Science Center)H-Index: 120
#2William J. McKenna (UCL: University College London)H-Index: 109
Last.Wojciech Zareba (URMC: University of Rochester Medical Center)H-Index: 86
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#1Julia Cadrin-Tourigny (MHI: Montreal Heart Institute)H-Index: 4
#2Laurens P. Bosman (UU: Utrecht University)H-Index: 2
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#1Karyn M. Austin (Boston Children's Hospital)H-Index: 11
#2Michael A. Trembley (Boston Children's Hospital)H-Index: 1
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#1James McKinney (UBC: University of British Columbia)H-Index: 6
#2Amer M. Johri (Queen's University)H-Index: 14
Last.Paul Dorian (UBC: University of British Columbia)H-Index: 77
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