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

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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·Heart Rhythm
DOI 10.1016/j.hrthm.2019.05.007
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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 amyloid and sarcoidosis, Chagas' disease and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation which 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 (Patient, Intervention, Comparison, Outcome) 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 ACC and AHA 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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Metadata

Authors
Towbin, J. A., McKenna, W. J., Abrams, D. J., Ackerman, M. J., Calkins, H., Darrieux, F. C. C., Daubert, J. P., de Chillou, C., DePasquale, E. C., Desai, M. Y., Estes, N. A. M., Hua, W., Indik, J. H., Ingles, J., James, C. A., John, R. M., Judge, D. P., Keegan, R., Krahn, A. D., Link, M. S., Marcus, F. I., McLeod, C. J., Mestroni, L., Priori, S. G., Saffitz, J. E., Sanatani, S., Shimizu, W., van Tintelen, J. P., Wilde, A. A. M., & Zareba, W.
Venue
Heart Rhythm
Publisher
Elsevier BV
Published
2019-11
Volume
16
Issue
11
Pages
e301-e372
ISSN
1547-5271 (ISSN-L)
DOI
10.1016/j.hrthm.2019.05.007
OpenAlex
W2944140727
Cited by
846
References
465
FWCI
63.74
Type
journal article
Language
en
Open access
bronze
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)

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CoChat. (2026). Citation check: 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. https://app.cochat.ai/papers/10.1016/j.hrthm.2019.05.007
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Towbin, J. A., McKenna, W. J., Abrams, D. J., Ackerman, M. J., Calkins, H., Darrieux, F. C. C., Daubert, J. P., de Chillou, C., DePasquale, E. C., Desai, M. Y., Estes, N. A. M., Hua, W., Indik, J. H., Ingles, J., James, C. A., John, R. M., Judge, D. P., Keegan, R., Krahn, A. D., . . . Zareba, W. (2019). 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart Rhythm. https://doi.org/10.1016/j.hrthm.2019.05.007

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