HRS Expert Consensus Statement on the Diagnosis and Management of Arrhythmias Associated With Cardiac Sarcoidosis David H. Birnie, MD (Chair), 1 William H. Sauer, MD, FHRS, CCDS (Chair), 2 Frank Bogun, MD, 3 Joshua M. Cooper, MD, FHRS, 4 Daniel A. Culver, DO, 5, * Claire S. Duvernoy, MD, 6,† Marc A. Judson, MD, 7,‡ Jordana Kron, MD, 8 Davendra Mehta, MD, PhD, FHRS, 9 Jens Cosedis Nielsen, MD, 10 Amit R. Patel, MD, 11,§ Tohru Ohe, MD, FHRS, 12,J Pekka Raatikainen, MD, 13,¶ Kyoko Soejima, MD 14 From the 1 University of Ottawa Heart Institute, Ottawa, Ontario, Canada, 2 University of Colorado, Aurora, Colorado, 3 University of Michigan, Ann Arbor, Michigan, 4 Temple University Health System, Philadelphia, Pennsylvania, 5 Cleveland Clinic, Cleveland, Ohio, 6 VA Ann Arbor Healthcare System and University of Michigan, Ann Arbor, Michigan, 7 Albany Medical College, Albany, New York, 8 Virginia Commonwealth University, Richmond, Virginia, 9 Mount Sinai School of Medicine, New York, New York, 10 Aarhus University Hospital, Aarhus, Denmark, 11 University of Chicago, Chicago, Illinois, 12 Sakakibara Heart Institute of Okayama, Okayama, Japan, 13 Heart Center, Tampere University Hospital, Tampere, Finland, and 14 Kyorin University School of Medicine, Mitaka City, Japan. TABLE OF CONTENTS 1. Introduction ......................................................................... 1304 2. Background ......................................................................... 1305 3. Diagnosis of Cardiac Sarcoidosis ............................... 1306 4. Screening for Cardiac Sarcoidosis ............................. 1308 5. Management of Conduction Abnormalities ........... 1312 6. Management of Atrial Arrhythmias .......................... 1313 7. Management of Ventricular Arrhythmias ............... 1314 8. Risk Stratification for Sudden Cardiac Death ............ 1315 9. ICD Implantation and Follow-Up .............................. 1317 10. Conclusions and Future Directions ........................... 1319 Appendix 1 .......................................................................... 1319 1. Introduction This international expert consensus statement was written by experts in the field who were chosen by the Heart Rhythm Society in collaboration with representatives from the American College of Cardiology, American College of Chest Physicians, American Heart Association, Asia Pacific Heart Rhythm Society, European Heart Rhythm Association, and World Association for Sarcoidosis and Other Granu- lomatous Disorders (WASOG). The goals of this document are as follows: 1. Establish working criteria for the diagnosis of cardiac sarcoidosis (CS) on the basis of expert opinion and the limited available data. 2. Provide guidance and recommendations to physicians treating extracardiac sarcoidosis on appropriate screening for possible cardiac involvement. 3. Provide guidance and recommendations to cardiologists and cardiac electrophysiologists on the management of specific arrhythmias associated with CS. KEYWORDS Sarcoidosis; Heart block; Ventricular tachycardia; Catheter ablation; Immunosuppression; Implantable cardioverter-defibrillator; Atrial arrhythmias; Risk stratification ABBREVIATIONS AF = atrial fibrillation; ARVC = arrhythmogenic right ventricular cardiomyopathy; AV = atrioventricular; CS = cardiac sarcoidosis; EMB = endomyocardial biopsy; ICD = implantable cardioverter-defibrillator; PES = programmed electrical stimulation; SVT = supraventricular tachycardia; VF = ventricular fibrillation; VT = ventricular tachycardia (Heart Rhythm 2014;11:1304–1323) Developed in collaboration with and endorsed by the World Associ- ation for Sarcoidosis and Other Granulomatous Disorders (WASOG), the American College of Cardiology (ACC), the American College of Chest Physicians (ACCP), the American Heart Association (AHA), the Asia Pacific Heart Rhythm Society (APHRS), and the European Heart Rhythm Association (EHRA). Address correspondence: David H. Birnie and William H. Sauer. E-mail address: dbirnie@ottawaheart.ca; William.Sauer@ucdenver.edu. * Representative for the World Association for Sarcoidosis and Other Granulo- matous Disorders (WASOG); † Representative for the American College of Cardiology (ACC); ‡ Representative for the American College of Chest Physicians (ACCP); § Representative for the American Heart Associa- tion (AHA); J Representative for the Asia Paci fic Heart Rhythm Society (APHRS); ¶ Representative for the European Heart Rhythm Association (EHRA) 1547-5271/$-see front matter B 2014 Heart Rhythm Society. All rights reserved. http://dx.doi.org/10.1016/j.hrthm.2014.03.043