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Original Research: Diffuse Lung Disease |

Cardiac Sarcoidosis: The Impact of Age and Implanted Devices on Survival

Ying Zhou, MD; Elyse E. Lower, MD; Hui-ping Li, MD; Alexandru Costea, MD; Mehran Attari, MD; Robert P. Baughman, MD
Author and Funding Information

FUNDING/SUPPORT: The authors have reported to CHEST that no funding was received for this study.

aDepartment of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH

bDepartment of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China

CORRESPONDENCE TO: Robert P. Baughman, MD, Internal Medicine, University of Cincinnati, University of Cincinnati Medical Center, 1001 Holmes, Eden Ave, Cincinnati, OH 45267-0565


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2017;151(1):139-148. doi:10.1016/j.chest.2016.08.1457
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Objective  To assess the clinical characteristics, diagnosis, and outcome of cardiac sarcoidosis in a single institution sarcoidosis clinic.

Methods  Patients with cardiac sarcoidosis were identified using refined World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG) criteria of highly probable and probable. Patient demographics, local and systemic treatments, and clinical outcome were collected.

Results  Of the 1,815 patients evaluated over a 6-year period, 73 patients met the WASOG criteria for cardiac sarcoidosis. The median age at diagnosis was 46 years, with a median follow-up of 8.8 years. Reduced left ventricular ejection fraction (LVEF) was the most common manifestation (54.8%). Patients with arrhythmias experienced ventricular tachycardia or severe heart block, (35.6% and 19.2%, respectively) with or without reduced LVEF. A total of 45 (61.6%) patients underwent cardiac PET scan and/or MRI, with 41 (91.1%) having a positive study. During follow-up, 10 patients (13.7%) either underwent transplant (n = 3) or died (n = 7) from sarcoidosis. Kaplan-Meier survival curves revealed 5- and 10-year survival rates of 95.5% and 93.4%, respectively. Univariate factors of age at diagnosis < 46 years, implantation of pacemaker or defibrillator, mycophenolate treatment, or LVEF > 40% were associated with improved survival. Cox regression analysis demonstrated that age ≥ 46 years and lack of an implanted pacemaker or defibrillator were the only independent predictors of mortality.

Conclusions  The new WASOG criteria were able to characterize cardiac involvement in our sarcoidosis clinic. Age and lack of pacemaker or defibrillator were the significant predictors of mortality for cardiac sarcoidosis, and reduced LVEF < 40% was associated with worse prognosis.

Trial Registry  ClinicalTrials.gov; No.: NCT02356445; URL: www.clinicaltrials.gov.

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