2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy

Research output: Contribution to journalArticle


  • Jeffrey A. Towbin
  • William J. McKenna
  • Michael J. Ackerman
  • Hugh Calkins
  • Francisco C.C. Darrieux
  • James P. Daubert
  • Christian de Chillou
  • Eugene C. DePasquale
  • Milind Y. Desai
  • N. A.Mark Estes
  • Wei Hua
  • Jodie Ingles
  • Cynthia A. James
  • Roy M. John
  • Daniel P. Judge
  • Roberto Keegan
  • Andrew D. Krahn
  • Mark S. Link
  • Frank I. Marcus
  • Christopher J. McLeod
  • Silvia G. Priori
  • Shubhayan Sanatani
  • Wataru Shimizu
  • J. Peter van Tintelen
  • Arthur A.M. Wilde
  • Wojciech Zareba

External Institution(s)

  • Le Bonheur Children's Medical Center
  • University of Tennessee Health Science Center
  • University College London
  • Mayo Clinic Rochester, MN
  • Johns Hopkins University
  • Instituto do Coração do Hospital das Clínicas
  • Duke University Medical Center
  • CHU de Nancy
  • University of California at Los Angeles
  • Cleveland Clinic Foundation
  • University of Pittsburgh
  • Chinese Academy of Medical Sciences
  • University of Sydney
  • Vanderbilt University
  • Medical University of South Carolina
  • Hospital Privado del Sur
  • Hospital Español
  • University of British Columbia
  • University of Texas Southwestern Medical Center
  • University of Arizona
  • University of Pavia
  • European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN GUARD-Heart)
  • and Vascular Health
  • Nippon Medical School
  • University of Amsterdam
  • Utrecht University
  • Columbia University
  • University of Rochester


Original languageEnglish (US)
Pages (from-to)e301-e372
JournalHeart Rhythm
Issue number11
StatusPublished - Nov 2019


Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.

    Research areas

  • Arrhythmogenic cardiomyopathy, Arrhythmogenic left ventricular cardiomyopathy, Arrhythmogenic right ventricular cardiomyopathy, Cascade family screening, Catheter ablation, Diagnosis of arrhythmogenic cardiomyopathy, Disease mechanisms, Electrophysiology, Exercise restriction, Genetic testing, Genetic variants, ICD decisions, Left ventricular noncompaction, Risk stratification, Treatment of arrhythmogenic cardiomyopathy

Citation formats


Towbin, J. A., McKenna, W. J., Abrams, D. J., Ackerman, M. J., Calkins, H., Darrieux, F. C. C., ... Zareba, W. (2019). 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart Rhythm, 16(11), e301-e372. https://doi.org/10.1016/j.hrthm.2019.05.007


Towbin, JA, McKenna, WJ, Abrams, DJ, Ackerman, MJ, Calkins, H, Darrieux, FCC, Daubert, JP, de Chillou, C, DePasquale, EC, Desai, MY, Estes, NAM, Hua, W, Indik, JH, Ingles, J, James, CA, John, RM, Judge, DP, Keegan, R, Krahn, AD, Link, MS, Marcus, FI, McLeod, CJ, Mestroni, L, Priori, SG, Saffitz, JE, Sanatani, S, Shimizu, W, van Tintelen, JP, Wilde, AAM & Zareba, W 2019, '2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy', Heart Rhythm, vol. 16, no. 11, pp. e301-e372. https://doi.org/10.1016/j.hrthm.2019.05.007