2019 HRS Expert Consensus Statement on Evaluation, Risk Stratification, and Management of Arrhythmogenic Cardiomyopathy

Published 9 May 2019

Jeffrey A. Towbin, MS, MD; William J. McKenna, MD, DSc; Dominic J. Abrams, MD, MRCP, MBA; Michael J. Ackerman, MD, PhD; Hugh Calkins, MD, FHRS, CCDS; Francisco C.C. Darrieux, MD, PhD; James P. Daubert, MD, FHRS; Christian de Chillou, MD, PhD; Eugene C. DePasquale, MD; Milind Y. Desai, MD; N.A. Mark Estes III, MD, FHRS, CCDS; Wei Hua, MD, FHRS; Julia H. Indik, MD, PhD, FHRS; Jodie Ingles, MPH, PhD, FHRS; Cynthia A. James, ScM, PhD, CGC; Roy M. John, MBBS, PhD, CCDS, FHRS; Daniel P. Judge, MD; Roberto Keegan, MD; Andrew D. Krahn, MD, FHRS; Mark S. Link, MD, FHRS; Frank I. Marcus, MD; Christopher J. McLeod, MBChB, PhD, FHRS; Luisa Mestroni, MD; Silvia G. Priori, MD, PhD; Jeffrey E. Saffitz, MD, PhD; Shubhayan Sanatani, MD, FHRS, CCDS; Wataru Shimizu, MD, PhD; J. Peter van Tintelen, MD, PhD; Arthur A.M. Wilde, MD, PhD; Wojciech Zareba, MD, PhD

Heart Rhythm. 2019 Nov;16(11):e301-72

• Advanced Heart Failure & Transplantation
• Cardiology
• Cardiothoracic Surgery
• Consensus Document
• Endorsement
• Pediatrics
• Publications & Journals
• Research & Immunology
• Standards & Guidelines

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 international consensus statement from the Heart Rhythm Society (HRS) is intended to help cardiologists and other health care professionals involved in the care of adult and pediatric patients with arrhythmogenic cardiomyopathy (ACM), which encompasses a broad range of disorders, by providing recommendations for evaluation and management and supporting shared decision making between health care providers and patients in a document format that is also useful at the point of care.

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