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


BACKGROUND
ClinicalTrials.gov; No.: NCT02356445; URL: www.clinicaltrials.gov.
Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH; Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
Ying
Y
Zhou
Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH.
Elyse E
EE
Lower
Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
Hui-Ping
HP
Li
Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH.
Alexandru
A
Costea
Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH.
Mehran
M
Attari
Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH. Electronic address: bob.baughman@uc.edu.
Robert P
RP
Baughman
D000328:Adult; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D009202:Cardiomyopathies / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D002681:China / Q000453:epidemiology; D017147:Defibrillators, Implantable / Q000706:statistics & numerical data*; D005260:Female; D005500:Follow-Up Studies; D016027:Heart Transplantation / Q000379:methods / Q000706:statistics & numerical data; D006801:Humans; D053208:Kaplan-Meier Estimate; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000627:therapeutic use*; D049268:Positron-Emission Tomography / Q000379:methods; D011379:Prognosis; D012507:Sarcoidosis / Q000150:complications / Q000401:mortality; D013318:Stroke Volume; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000209:etiology / Q000517:prevention & control; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis / Q000209:etiology
D000903:Antibiotics, Antineoplastic; D009173:Mycophenolic Acid
cardiomyopathy; imaging; methotrexate; mycophenolate; sarcoidosis; sarcoidosis heart