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

Chest. 2017 Jan;151(1):139-148. doi: 10.1016/j.chest.2016.08.1457. Epub 2016 Sep 8.

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.

Keywords: cardiomyopathy; imaging; methotrexate; mycophenolate; sarcoidosis; sarcoidosis heart.

Publication types

  • Clinical Trial

MeSH terms

  • Adult
  • Antibiotics, Antineoplastic / therapeutic use
  • Cardiomyopathies* / diagnosis
  • Cardiomyopathies* / etiology
  • Cardiomyopathies* / physiopathology
  • Cardiomyopathies* / therapy
  • China / epidemiology
  • Defibrillators, Implantable / statistics & numerical data*
  • Female
  • Follow-Up Studies
  • Heart Transplantation* / methods
  • Heart Transplantation* / statistics & numerical data
  • Humans
  • Kaplan-Meier Estimate
  • Magnetic Resonance Imaging, Cine / methods
  • Male
  • Middle Aged
  • Mycophenolic Acid / therapeutic use*
  • Positron-Emission Tomography / methods
  • Prognosis
  • Sarcoidosis* / complications
  • Sarcoidosis* / mortality
  • Stroke Volume
  • Tachycardia, Ventricular* / diagnosis
  • Tachycardia, Ventricular* / etiology
  • Tachycardia, Ventricular* / prevention & control
  • Ventricular Dysfunction, Left / diagnosis
  • Ventricular Dysfunction, Left / etiology

Substances

  • Antibiotics, Antineoplastic
  • Mycophenolic Acid

Associated data

  • ClinicalTrials.gov/NCT02356445