Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy : A Multinational Collaboration

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Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy : A Multinational Collaboration

Cadrin-Tourigny, Julia (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA; Cardiovascular Genetics Center, Montreal Heart Institute, Université de Montréal, Canada,Johns Hopkins Hospital

Bosman, Laurens P. (author): University Medical Center Utrecht,Interuniversity Cardiology Institute of the Netherlands,Netherlands Heart Institute, Netherlands

Wang, Weijia (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA,Netherlands Heart Institute Department of Cardiology University Medical Center Utrecht, Utrecht University, the Netherlands; Institute of Cardiovascular Science & Institute of Health Informatics, Faculty of Population Health Sciences, University College London, United Kingdom

Tadros, Rafik (author): Cardiovascular Genetics Center, Montreal Heart Institute, Université de Montréal, Canada

Bhonsale, Aditya (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA

Bourfiss, Mimount (author): University Medical Center Utrecht,Department of Cardiology, Utrecht University, the Netherlands

Lie, Oyvind H. (author): Norwegian Radium Hospital,Department of Cardiology and Research group for Cardiogenetics and Sudden Cardiac Death, Oslo University Hospital, Rikshospitalet, Norway

Saguner, Ardan M. (author): University Hospital of Zurich,Department of Cardiology, University Heart Center Zurich, Switzerland

Svensson, Anneli, 1972- (author): Linköpings universitet,Linköping University,Avdelningen för diagnostik och specialistmedicin,Medicinska fakulteten,Region Östergötland, Kardiologiska kliniken US,Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA

Andorin, Antoine (author): Cardiovascular Genetics Center, Montreal Heart Institute, Université de Montréal, Canada,Amsterdam UMC, University of Amsterdam, Heart Center, the Netherlands,Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA

Tichnell, Crystal (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA

Murray, Brittney (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA

Zeppenfeld, Katja (author): Department of Cardiology, Leiden University Medical Center, Netherlands

Van Den Berg, Maarten P. (author): Department of Cardiology, University Medical Center Groningen, University of Groningen, the Netherlands

Asselbergs, Folkert W. (author): University Medical Center Utrecht,Interuniversity Cardiology Institute of the Netherlands,University College London

Wilde, Arthur A.M. (author): Academic Medical Center of University of Amsterdam (AMC)

Krahn, Andrew D. (author): Division of Cardiology, University of British Columbia, Vancouver, Canada

Talajic, Mario (author): Cardiovascular Genetics Center, Montreal Heart Institute, Université de Montréal, Canada

Rivard, Lena (author): Cardiovascular Genetics Center, Montreal Heart Institute, Université de Montréal, Canada

Chelko, Stephen (author): Department of Medicine, Division of Cardiology Johns Hopkins Hospital, Baltimore, USA; Department of Biomedical Sciences, Florida State University College of Medicine, USA,Florida State University

Zimmerman, Stefan L. (author): The Russell H. Morgan Department of Radiology and Radiological Science , Johns Hopkins Hospital, USA

Kamel, Ihab R. (author): The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins Hospital, USA

Crosson, Jane E. (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA

Judge, Daniel P. (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA

Yap, Sing Chien (author): Erasmus University Medical Center,Department of Cardiology, Erasmus Medical Center, Rotterdam

Van Der Heijden, Jeroen F. (author): Department of Cardiology, University Medical Center Utrecht, Utrecht University, the Netherlands

Tandri, Harikrishna (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, Baltimore, USA

Jongbloed, Jan D.H. (author): Department of Genetics, University Medical Center Groningen, University of Groningen, the Netherlands

Van Tintelen, J. Peter (author): Netherlands Heart Institute, University Medical Center Utrecht, Utrecht University, the Netherlands; Department of Clinical Genetics, Amsterdam UMC, University of Amsterdam, the Netherlands,Interuniversity Cardiology Institute of the Netherlands,Academic Medical Center of University of Amsterdam (AMC)

Platonov, Pyotr G. (author): Lund University,Lunds universitet,Kardiologi,Sektion II,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Electrocardiology Research Group - CIEL,Forskargrupper vid Lunds universitet,Cardiology,Section II,Department of Clinical Sciences, Lund,Faculty of Medicine,Lund University Research Groups,Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, Baltimore, USA

Duru, Firat (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA

Haugaa, Kristina H. (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, Baltimore, USA,Norwegian Radium Hospital

Khairy, Paul (author): Montreal Heart Institute,Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, Baltimore, USA

Hauer, Richard N.W. (author): Johns Hopkins Hospital,Interuniversity Cardiology Institute of the Netherlands

Calkins, Hugh (author): Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, USA

Te Riele, Anneline S.J.M. (author): University Medical Center Utrecht,Interuniversity Cardiology Institute of the Netherlands,Johns Hopkins Hospital

James, Cynthia A. (author): Johns Hopkins Hospital

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Lippincott Williams & Wilkins, 2021
2021
English 11 s.
In: Circulation: Arrhythmia and Electrophysiology. - : Lippincott Williams & Wilkins. - 1941-3149 .- 1941-3084. ; 14:1

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Journal article (peer-reviewed)

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Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with ventricular arrhythmias (VA) and sudden cardiac death (SCD). A model was recently developed to predict incident sustained VA in patients with ARVC. However, since this outcome may overestimate the risk for SCD, we aimed to specifically predict life-threatening VA (LTVA) as a closer surrogate for SCD. Methods: We assembled a retrospective cohort of definite ARVC cases from 15 centers in North America and Europe. Association of 8 prespecified clinical predictors with LTVA (SCD, aborted SCD, sustained, or implantable cardioverter-defibrillator treated ventricular tachycardia >250 beats per minute) in follow-up was assessed by Cox regression with backward selection. Candidate variables included age, sex, prior sustained VA (≥30s, hemodynamically unstable, or implantable cardioverter-defibrillator treated ventricular tachycardia; or aborted SCD), syncope, 24-hour premature ventricular complexes count, the number of anterior and inferior leads with T-wave inversion, left and right ventricular ejection fraction. The resulting model was internally validated using bootstrapping. Results: A total of 864 patients with definite ARVC (40±16 years; 53% male) were included. Over 5.75 years (interquartile range, 2.77-10.58) of follow-up, 93 (10.8%) patients experienced LTVA including 15 with SCD/aborted SCD (1.7%). Of the 8 prespecified clinical predictors, only 4 (younger age, male sex, premature ventricular complex count, and number of leads with T-wave inversion) were associated with LTVA. Notably, prior sustained VA did not predict subsequent LTVA (P=0.850). A model including only these 4 predictors had an optimism-corrected C-index of 0.74 (95% CI, 0.69-0.80) and calibration slope of 0.95 (95% CI, 0.94-0.98) indicating minimal over-optimism. Conclusions: LTVA events in patients with ARVC can be predicted by a novel simple prediction model using only 4 clinical predictors. Prior sustained VA and the extent of functional heart disease are not associated with subsequent LTVA events.

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MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Clinical Medicin ...; and Cardiac and Card ...

Articles in the publication: Circulation: Arr ...

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Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy : A Multinational Collaboration

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