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

Jeffrey A. Towbin, William J. McKenna, Dominic J. Abrams, 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, Julia H. Indik, Jodie Ingles, Cynthia A. James, Roy M. John, Daniel P. Judge, Roberto Keegan, Andrew D. Krahn, Mark S. LinkFrank I. Marcus, Christopher J. McLeod, Luisa Mestroni, Silvia G. Priori, Jeffrey E. Saffitz, Shubhayan Sanatani, Wataru Shimizu, J. Peter van Tintelen, Arthur A.M. Wilde, Wojciech Zareba

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)e373-e407
JournalHeart rhythm
Volume16
Issue number11
DOIs
StatePublished - Nov 2019

Fingerprint

Risk Management
Cardiomyopathies
Arrhythmogenic Right Ventricular Dysplasia
Cardiac Arrhythmias
Heart Valve Diseases
Inborn Genetic Diseases
Chagas Disease
Dilated Cardiomyopathy
Amyloidosis
Sarcoidosis
Myocardial Ischemia
Dilatation
Myocardium
Phenotype

Keywords

  • 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

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy : Executive summary. / Towbin, Jeffrey A.; McKenna, William J.; Abrams, Dominic J.; Ackerman, Michael J.; Calkins, Hugh; Darrieux, Francisco C.C.; Daubert, James P.; de Chillou, Christian; DePasquale, Eugene C.; Desai, Milind Y.; Estes, N. A.Mark; Hua, Wei; Indik, Julia H.; Ingles, Jodie; James, Cynthia A.; John, Roy M.; Judge, Daniel P.; Keegan, Roberto; Krahn, Andrew D.; Link, Mark S.; Marcus, Frank I.; McLeod, Christopher J.; Mestroni, Luisa; Priori, Silvia G.; Saffitz, Jeffrey E.; Sanatani, Shubhayan; Shimizu, Wataru; van Tintelen, J. Peter; Wilde, Arthur A.M.; Zareba, Wojciech.

In: Heart rhythm, Vol. 16, No. 11, 11.2019, p. e373-e407.

Research output: Contribution to journalArticle

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: Executive summary', Heart rhythm, vol. 16, no. 11, pp. e373-e407. https://doi.org/10.1016/j.hrthm.2019.09.019
Towbin, Jeffrey A. ; McKenna, William J. ; Abrams, Dominic J. ; Ackerman, Michael J. ; Calkins, Hugh ; Darrieux, Francisco C.C. ; Daubert, James P. ; de Chillou, Christian ; DePasquale, Eugene C. ; Desai, Milind Y. ; Estes, N. A.Mark ; Hua, Wei ; Indik, Julia H. ; Ingles, Jodie ; James, Cynthia A. ; John, Roy M. ; Judge, Daniel P. ; Keegan, Roberto ; Krahn, Andrew D. ; Link, Mark S. ; Marcus, Frank I. ; McLeod, Christopher J. ; Mestroni, Luisa ; Priori, Silvia G. ; Saffitz, Jeffrey E. ; Sanatani, Shubhayan ; Shimizu, Wataru ; van Tintelen, J. Peter ; Wilde, Arthur A.M. ; Zareba, Wojciech. / 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy : Executive summary. In: Heart rhythm. 2019 ; Vol. 16, No. 11. pp. e373-e407.
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AU - Towbin, Jeffrey A.

AU - McKenna, William J.

AU - Abrams, Dominic J.

AU - Ackerman, Michael J.

AU - Calkins, Hugh

AU - Darrieux, Francisco C.C.

AU - Daubert, James P.

AU - de Chillou, Christian

AU - DePasquale, Eugene C.

AU - Desai, Milind Y.

AU - Estes, N. A.Mark

AU - Hua, Wei

AU - Indik, Julia H.

AU - Ingles, Jodie

AU - James, Cynthia A.

AU - John, Roy M.

AU - Judge, Daniel P.

AU - Keegan, Roberto

AU - Krahn, Andrew D.

AU - Link, Mark S.

AU - Marcus, Frank I.

AU - McLeod, Christopher J.

AU - Mestroni, Luisa

AU - Priori, Silvia G.

AU - Saffitz, Jeffrey E.

AU - Sanatani, Shubhayan

AU - Shimizu, Wataru

AU - van Tintelen, J. Peter

AU - Wilde, Arthur A.M.

AU - Zareba, Wojciech

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N2 - 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.

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KW - Disease mechanisms

KW - Electrophysiology

KW - Exercise restriction

KW - Genetic testing

KW - Genetic variants

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KW - Left ventricular noncompaction

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KW - Treatment of arrhythmogenic cardiomyopathy

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