Ying Zhou1, Elyse E Lower2, Hui-Ping Li3, Alexandru Costea2, Mehran Attari2, Robert P Baughman4. 1. 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. 2. Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH. 3. Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. 4. Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH. Electronic address: bob.baughman@uc.edu.
Abstract
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.
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.
Authors: Rajat Kalra; Shray Malik; Ko-Hsuan Amy Chen; Fredrick Ogugua; Pal Satyajit Singh Athwal; Andrew C Elton; Pratik S Velangi; Mohamed F Ismail; Sanya Chhikara; Jeremy S Markowitz; Prabhjot S Nijjar; Lisa von Wald; Henri Roukoz; Maneesh Bhargava; David Perlman; Chetan Shenoy Journal: Circ Arrhythm Electrophysiol Date: 2021-09-01
Authors: Emanuele Bobbio; Marie Lingbrant; Bright I Nwaru; Eva Hessman; Jukka Lehtonen; Kristjan Karason; Entela Bollano Journal: Heart Fail Rev Date: 2020-05 Impact factor: 4.214