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Outcomes of patients with definite and suspected isolated cardiac sarcoidosis treated with an implantable cardiac defibrillator

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Abstract

Purpose

Cardiac sarcoidosis (CS) patients are at increased risk for sudden death. Isolated CS is rare and can be difficult to diagnose.

Methods

In this multicenter retrospective review, patients with CS and an implantable cardiac defibrillator (ICD) were identified.

Results

Of 235 patients with CS and ICD, 13 (5.5 %) had isolated CS, including 7 (3.0 %) with definite isolated CS (biopsy or necropsy-proven) and 6 (2.6 %) with suspected isolated CS based on a constellation of clinical, ECG, and imaging findings. Among 13 patients with isolated CS, 10 (76.9 %) were male, mean age was 53.8 ± 7.6 years, and mean left ventricular ejection fraction was 38.3 ± 16.5. Diagnosis was made by cardiac magnetic resonance (CMR) (n = 2), biopsy (n = 3), CMR and biopsy (n = 2), CMR and positron emission tomography (PET) (n = 2), PET (n = 1), late enhanced cardiac CT (n = 1), pathology at heart transplant (n = 1), and autopsy (n = 1). Eight of 13 (61.5 %) patients with isolated CS had a secondary prevention indication (VT in 6 and VF in 2) vs. 80 of 222 (36.0 %) with sarcoidosis in other organs (p = 0.04). Over a mean of 4.2 years, 9 of 13 (69.2 %) patients with isolated CS received appropriate ICD therapy, including anti-tachycardia pacing (ATP) and/or shock, compared with 75 of 222 (33.8 %) patients with cardiac and extracardiac sarcoidosis (p = 0.0150). Six of 7 (85.7 %) patients with definite isolated CS received appropriate ICD intervention, compared with 78 of 228 patients (34.2 %) without definite isolated CS (p = 0.0192.)

Conclusions

In this retrospective study, patients with isolated CS had very high rates of appropriate ICD therapy. Prospective, long-term follow-up of consecutive patients with isolated CS is needed to determine the true natural history and rates of ventricular arrhythmias in this rare and difficult-to-diagnose disease.

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Abbreviations

ATP:

Anti-tachycardia pacing

CMR:

Cardiac magnetic resonance

CS:

Cardiac sarcoidosis

DE:

Delayed enhancement

ECG:

Electrocardiogram

ICD:

Implantable cardiac defibrillator

LVEF:

Left ventricular ejection fraction

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Conflict of interest

Dr. Kron has reported that she has no relationships relevant to the contents of this paper to disclose.

Dr. Sauer has reported that he received education grants for EP fellowship and consulting fees from Medtronic Inc., St. Jude Medical, and Boston Scientific Corp.

Dr. Mueller has reported that she has no relationship relevant to the contents of this paper to disclose.

Dr. Schuller has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Bogun has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Sarsam has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Rosenfeld has reported that she has received fellowship support from Medtronic Inc., Boston Scientific, and St. Jude Medical.

Dr. Mitiku has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Cooper has received honoraria from Medtronic Inc., Boston Scientific, Biotronik, St. Jude Medical, and Spectranetics.

Dr. Mehta has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Greenspon has received honoraria from Medtronic Inc., Boston Scientific, and St. Jude Medical.

Dr. Ortman has received speaking fees from St. Jude Medical and Boston Scientific.

Dr. Delurgio has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Valadri has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Narasimhan has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Swapna has reported that she has no relationships relevant to the contents of this paper to disclose.

Dr. Singh has reported that he received research grants from St. Jude Medical, Medtronic Inc., Boston Scientific Corp., and Biotronik; consultant fees from Boston Scientific Corp., Biotronik, St. Jude Medical, Medtronic Inc., CardioInsight Inc., Thoratec Inc., and Biosense Webster; honoraria from Medtronic Inc., Biotronik, Guidant Corp., St. Jude Medical, and Sorin Group.

Dr. Danik has reported consultant fees from St. Jude Medical, Boston Scientific.

Dr. Markowitz has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Almquist has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Krahn has reported that he has no relationships relevant to the contents of this paper to disclose.

Mr. Wolfe has reported that he has no relationships relevant to the contents of this paper to disclose.

Mr. Feinstein has reported that he has no relationships relevant to the contents of this paper to disclose.

Dr. Ellenbogen has received honoraria from Medtronic Inc., Boston Scientific, and Cameron Medical; speaking fees from Medtronic Inc., Boston Scientific, St. Jude Medical, Biotronik, and Sanofi; research grants from Medtronic Inc., Boston Scientific, Biosense Web, and Sanofi; fellowship support from Medtronic Inc., Boston Scientific, and Biosense Web.

Dr. Crawford has received grant support from Cardiovascular Center at the University of Michigan and Boston Scientific, Inc. for the development and maintenance of cardiac sarcoidosis registry.

Author information

Authors and Affiliations

  1. Department of Cardiac Electrophysiology, Virginia Commonwealth University, P.O. Box 980053, Richmond, VA, 23298-0053, USA

    Jordana Kron, Shawn Feinstein & Kenneth A. Ellenbogen

  2. Department of Cardiology, University of Colorado Denver, Denver, CO, USA

    William Sauer & Joseph Schuller

  3. Department of Radiology, University of Michigan, Ann Arbor, MI, USA

    Gisela Mueller

  4. Department of Cardiology, University of Michigan, Ann Arbor, MI, USA

    Frank Bogun, Sinan Sarsam & Thomas Crawford

  5. Department of Cardiac Electrophysiology, Yale University, New Haven, CT, USA

    Lynda Rosenfeld

  6. Wayne State University, Detroit, MI, USA

    Teferi Y. Mitiku

  7. Department of Cardiology, Temple University, Philadelphia, PA, USA

    Joshua M. Cooper

  8. Department of Cardiology, Mount Sinai Hospital, New York, NY, USA

    Davendra Mehta

  9. Division of Cardiology, Thomas Jefferson University Hospital, Philadelphia, PA, USA

    Arnold J. Greenspon

  10. Cooper University Hospital, Camden, NJ, USA

    Matthew Ortman

  11. Department of Cardiology, Emory University, Atlanta, GA, USA

    David B. Delurgio

  12. Wright Center for Graduate Medical Education, Scranton, PA, USA

    Ravinder Valadri

  13. Department of Cardiology, CARE Hospital, Hyderabad, India

    Calambur Narasimhan & Nalla Swapna

  14. Department of Cardiology, Massachusetts General Hospital, Boston, MA, USA

    Jagmeet P. Singh

  15. St. Luke’s Roosevelt Hospital, New York, NY, USA

    Stephan Danik

  16. Department of Cardiology, Weill Cornell Medical Center, New York, NY, USA

    Steven M. Markowitz

  17. Department of Cardiology, Minneapolis Heart Institute, Minneapolis, MN, USA

    Adrian K. Almquist

  18. Division of Cardiology, University of British Columbia, Vancouver, BC, Canada

    Andrew D. Krahn

  19. Department of General Surgery, Virginia Commonwealth University, P.O. Box 980053, Richmond, VA, 23298-0053, USA

    Luke G. Wolfe

Authors
  1. Jordana Kron
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  2. William Sauer
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  3. Gisela Mueller
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  4. Joseph Schuller
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  5. Frank Bogun
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  6. Sinan Sarsam
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  7. Lynda Rosenfeld
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  8. Teferi Y. Mitiku
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  9. Joshua M. Cooper
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  10. Davendra Mehta
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  11. Arnold J. Greenspon
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  12. Matthew Ortman
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  13. David B. Delurgio
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  14. Ravinder Valadri
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  15. Calambur Narasimhan
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  16. Nalla Swapna
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  17. Jagmeet P. Singh
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  18. Stephan Danik
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  19. Steven M. Markowitz
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  20. Adrian K. Almquist
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  21. Andrew D. Krahn
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  22. Luke G. Wolfe
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  23. Shawn Feinstein
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  24. Kenneth A. Ellenbogen
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  25. Thomas Crawford
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Corresponding author

Correspondence to Jordana Kron.

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Kron, J., Sauer, W., Mueller, G. et al. Outcomes of patients with definite and suspected isolated cardiac sarcoidosis treated with an implantable cardiac defibrillator. J Interv Card Electrophysiol 43, 55–64 (2015). https://doi.org/10.1007/s10840-015-9978-3

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  • DOI: https://doi.org/10.1007/s10840-015-9978-3

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