title: Long-term outcome and its predictors in giant cell myocarditis.
abstract: There are no studies focusing on prognostic factors in giant cell myocarditis (GCM). We aimed to identify predictors of transplant-free survival in GCM.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Kaj
authors.initials: K
authors.lastname: Ekström
authors.affiliation: Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Jukka
authors.initials: J
authors.lastname: Lehtonen
authors.affiliation: Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Riina
authors.initials: R
authors.lastname: Kandolin
authors.affiliation: Department of Pathology, HUSLAB, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Anne
authors.initials: A
authors.lastname: Räisänen-Sokolowski
authors.affiliation: Department of Pathology, HUSLAB, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Kaisa
authors.initials: K
authors.lastname: Salmenkivi
authors.affiliation: Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Markku
authors.initials: M
authors.lastname: Kupari
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D000638:Amiodarone / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D054537:Atrioventricular Block / Q000209:etiology; D002304:Cardiac Pacing, Artificial; D017147:Defibrillators, Implantable; D005260:Female; D006333:Heart Failure / Q000209:etiology; D016027:Heart Transplantation / Q000706:statistics & numerical data*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009205:Myocarditis / Q000150:complications / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D009206:Myocardium / Q000473:pathology; D010138:Pacemaker, Artificial; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D017180:Tachycardia, Ventricular / Q000209:etiology; D014693:Ventricular Fibrillation / Q000209:etiology
chemical_list: D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D000889:Anti-Arrhythmia Agents; D007166:Immunosuppressive Agents; D000638:Amiodarone
keywords: Inflammatory cardiomyopathy; Myocardial biopsy; Transplantation