Long-term outcome and its predictors in giant cell myocarditis

Eur J Heart Fail. 2016 Dec;18(12):1452-1458. doi: 10.1002/ejhf.606. Epub 2016 Jul 13.

Abstract

Aims: There are no studies focusing on prognostic factors in giant cell myocarditis (GCM). We aimed to identify predictors of transplant-free survival in GCM.

Methods and results: We analysed the details of 46 patients with GCM (31 women, mean age 51 ± 12 years) seen at our hospital since 1991 and followed for the occurrence of cardiac death or transplantation till May 2015. The association of transplant-free survival with patient characteristics, laboratory data on admission, and myocardial histology in the 38 patients diagnosed prior to death or transplantation was examined. Altogether 26 patients died (n = 8) or underwent transplantation (n = 18) a median of 11 months following symptom onset. The 5-year estimate of transplant-free survival was 42% [95% confidence interval (CI) 35-48%]. By Cox regression analysis, the hazard ratio for death or transplantation was 0.87 (95% CI 0.75-0.99) per +5% difference in LVEF, 1.06 (95% CI 1.03-1.10) per + 1000 ng/L difference in NT-proBNP, and 4.57 (95% CI 1.63-11.28) for cardiac troponin-T above the median of 85 ng/L at presentation. The severity of necrosis and fibrosis in myocardial biopsy, graded by the consensus of two cardiac pathologists as none, mild, moderate, or severe, predicted the outcome with a hazard ratio of 7.17 (95% CI 2.29-22.40) for the presence of either necrosis or fibrosis of at least moderate extent.

Conclusions: In GCM, the probability of transplant-free survival is 42% at 5 years from symptom onset. Markers of myocyte injury and cardiac dysfunction help predict the outcome.

Keywords: Inflammatory cardiomyopathy; Myocardial biopsy; Transplantation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenergic beta-Antagonists / therapeutic use
  • Adult
  • Amiodarone / therapeutic use
  • Angiotensin-Converting Enzyme Inhibitors / therapeutic use
  • Anti-Arrhythmia Agents / therapeutic use
  • Atrioventricular Block / etiology
  • Cardiac Pacing, Artificial
  • Defibrillators, Implantable
  • Female
  • Heart Failure / etiology
  • Heart Transplantation / statistics & numerical data*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Middle Aged
  • Myocarditis / complications
  • Myocarditis / mortality*
  • Myocarditis / pathology
  • Myocarditis / therapy
  • Myocardium / pathology
  • Pacemaker, Artificial
  • Prognosis
  • Proportional Hazards Models
  • Retrospective Studies
  • Severity of Illness Index
  • Survival Rate
  • Tachycardia, Ventricular / etiology
  • Ventricular Fibrillation / etiology

Substances

  • Adrenergic beta-Antagonists
  • Angiotensin-Converting Enzyme Inhibitors
  • Anti-Arrhythmia Agents
  • Immunosuppressive Agents
  • Amiodarone