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J Am Coll Cardiol, 2005; 45:400-408, doi:10.1016/j.jacc.2004.08.068
© 2005 by the American College of Cardiology Foundation
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The impact of implantable cardioverter-defibrillator therapy on survival in autosomal-dominant arrhythmogenic right ventricular cardiomyopathy (ARVD5)

Kathy A. Hodgkinson, MSc*,{dagger}, Patrick S. Parfrey, MD, FRCPC, FACP*,*, Anne S. Bassett, MD, FRCPC{dagger},{ddagger}, Christine Kupprion, MD§, Jörg Drenckhahn, MD§, Mark W. Norman, MD, MRCP§, Ludwig Thierfelder, MD§, Susan N. Stuckless, MSc*, Elizabeth L. Dicks, MSc*, William J. McKenna, MD, FRCP|| and Sean P. Connors, MD, DPhil, FRCPC

* Clinical Epidemiology Unit
{dagger} Discipline of Genetics, Memorial University, Health Sciences Centre, St. John's, Newfoundland, Canada
{ddagger} Clinical Genetics Research Program, Centre for Addiction & Mental Health, University of Toronto, Toronto, Ontario, Canada
§ Max-Delbrück Centrum fur Molekulare Medizin, Berlin, Germany
|| The Heart Hospital, London, England
Division of Cardiology, Memorial University, St. John's, Newfoundland, Canada



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Figure 1 Part of one of 11 autosomal-dominant families with arrhythmogenic right ventricular cardiomyopathy linked to 3p25 showing family members at high risk (n = 197), low risk (n = 92), and unknown (n = 78) status.

 


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Figure 2 Time to death or last follow-up (censored) in high-risk males and females with arrhythmogenic right ventricular cardiomyopathy linked to 3p25.

 


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Figure 3 (A) Time to death or last follow-up (censored) in 30 male subjects at high-risk for arrhythmogenic right ventricular cardiomyopathy linked to 3p25 who received implantable cardioverter-defibrillators (ICDs) and 36 matched control subjects. (B) Time to death or last follow-up (censored) in 18 female subjects at high risk for arrhythmogenic right ventricular cardiomyopathy linked to 3p25 who received ICDs and 22 matched control subjects.

 


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Figure 4 Time to first appropriate discharge or last follow-up (censored) for sustained ventricular tachycardia (VT) and for VT >240 beats/min in males who received an implantable cardioverter-defibrillator and time to death or last follow-up (censored) in matched controls with arrhythmogenic right ventricular cardiomyopathy linked to 3p25.

 




 
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