| Institution: | 1. Le Bonheur Children’s Hospital, Memphis, Tennessee;2. University of Tennessee Health Science Center, Memphis, Tennessee;3. University College London, Institute of Cardiovascular Science, London, United Kingdom;4. Boston Children’s Hospital, Boston, Massachusetts;5. Mayo Clinic, Rochester, Minnesota;6. Johns Hopkins University, Baltimore, Maryland;7. Universidade de São Paulo, Instituto do Coração HCFMUSP, São Paulo, Brazil;8. Duke University Medical Center, Durham, North Carolina;9. Nancy University Hospital, Vandoeuvre-lès-Nancy, France;10. University of California Los Angeles, Los Angeles, California;11. Cleveland Clinic, Cleveland, Ohio;12. University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania;13. Fu Wai Hospital, Beijing, China;14. University of Arizona, Sarver Heart Center, Tucson, Arizona;15. Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney, Sydney, Australia;16. Vanderbilt University Medical Center, Nashville, Tennessee;17. Medical University of South Carolina, Charleston, South Carolina;18. Hospital Privado Del Sur, Buenos Aires, Argentina;19. Hospital Español, Bahia Blanca, Argentina;20. The University of British Columbia, Vancouver, Canada;21. UT Southwestern Medical Center, Dallas, Texas;22. University of Colorado Anschutz Medical Campus, Aurora, Colorado;23. University of Pavia, Pavia, Italy;24. European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN GUARD-Heart);25. ICS Maugeri, IRCCS, Pavia, Italy;26. Beth Israel Deaconess Medical Center, Boston, Massachusetts;27. Children’s Heart Center, Vancouver, Canada;28. Department of Cardiovascular Medicine, Nippon Medical School, Tokyo, Japan;29. University of Amsterdam, Academic Medical Center, Amsterdam, the Netherlands;30. Utrecht University Medical Center Utrecht, University of Utrecht, Department of Genetics, Utrecht, the Netherlands;31. Department of Medicine, Columbia University Irving Medical Center, New York, New York;32. University of Rochester Medical Center, Rochester, New York |
| 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. |
