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Volume 6, Issue 7 , Pages 984-992 , July 2009

Arrhythmogenic right ventricular cardiomyopathy/dysplasia clinical presentation and diagnostic evaluation: Results from the North American Multidisciplinary Study

Frank I. Marcus, MD
- Address reprint requests and correspondence: Dr. Frank I. Marcus, Section of Cardiology, Cardiology Room 6304, University of Arizona, 1501 N. Campbell Avenue, Tucson, Arizona 85724-0001
Wojciech Zareba, MD
Hugh Calkins, MD
Jeffrey A. Towbin, MD
Cristina Basso, MD
David A. Bluemke, MD, PhD
N.A. Mark Estes III, MD
Michael H. Picard, MD
Danita Sanborn, MD
Gaetano Thiene, MD
Thomas Wichter, MD
David Cannom, MD
David J. Wilber, MD
Melvin Scheinman, MD
Henry Duff, MD
James Daubert, MD
Mario Talajic, MD
Andrew Krahn, MD
Michael Sweeney, MD
Hasan Garan, MD
Scott Sakaguchi, MD
Bruce B. Lerman, MD
Charles Kerr, MD
Jack Kron, MD
Jonathan S. Steinberg, MD
Duane Sherrill, PhD
Kathleen Gear, RN
Mary Brown, MS
Patricia Severski, BS
Slava Polonsky, MS
Scott McNitt, MS

Received 3 November 2008 ,Accepted 6 March 2009.

Sequence of evaluation of patients suspected of having arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). PI = principal investigator.

Sequence of evaluation of patients suspected of having arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). PI = principal investigator.
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A: Relationship between initial classification of patients as affected at enrollment and final classification based on task force criteria after core laboratory analysis of diagnostic tests. B: Relati

A: Relationship between initial classification of patients as affected at enrollment and final classification based on task force criteria after core laboratory analysis of diagnostic tests. B: Relationship of initial classification of patients as borderline at enrollment and final classification based on task force criteria after core laboratory analysis of diagnostic tests.
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Percentage of the individual tests evaluated by the core laboratories as affected in 73 arrhythmogenic right ventricular cardiomyopathy/dysplasia affected probands and 28 borderline probands. ECG = el

Percentage of the individual tests evaluated by the core laboratories as affected in 73 arrhythmogenic right ventricular cardiomyopathy/dysplasia affected probands and 28 borderline probands. ECG = electrocardiogram; MRI = magnetic resonance imaging; SAECG = signal-averaged electrocardiogram.
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Decline in C statistic for each test removed individually from a combined seven-test model predicting the final phenotype of affected arrhythmogenic right ventricular cardiomyopathy/dysplasia. The lar

Decline in C statistic for each test removed individually from a combined seven-test model predicting the final phenotype of affected arrhythmogenic right ventricular cardiomyopathy/dysplasia. The largest decrease of diagnostic accuracy of this multivariant model is observed after removing the echocardiogram; the least decline is observed when removing the magnetic resonance imaging (MRI). ECG = electrocardiogram; SAECG = signal-averaged electrocardiogram.
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