title: Upgraded heart failure therapy leads to an improved outcome of dilated cardiomyopathy in infants and toddlers.
abstract: OBJECTIVE
  Dilated cardiomyopathy is a leading cause of cardiac death in children. Approximately 30% of children die or need cardiac transplantation in the first year after establishing the diagnosis. New strategies are needed to improve the outcome in this high-risk patient population.
  
  
  RESULTS
  We present our experience in 38 patients below the age of three years, who were diagnosed with dilated cardiomyopathy and who were treated at our institution between 2006 and 2012. The treatment strategy involved institution of β-blockers and angiotensin-converting enzyme inhibitors as soon as feasible. In selected cases, pulmonary artery banding or intracoronary autologous bone marrow-derived cell therapy was performed. The median age at presentation was six months (range 1-26 months). The median follow-up age was 16 months (range 2-80 months). Kaplan-Meier analysis of survival after dilated cardiomyopathy diagnosis revealed a one-year survival of 97% and a five-year survival of 86%. The rate of freedom from death or heart transplantation was 82% at one year and 69% at five years. Surviving patients who were free of transplantation, at the follow-up at 25 months (3-80 months), showed a significant improvement in left ventricular ejection fraction (from 19±11 to 46±16%) and left ventricular end-diastolic diameter (z-score from 4.6±2.4 to 1.4±1.6). In addition, the levels of B-type natriuretic peptide improved significantly (from 3330±3840 to 171±825 pg/ml).
  
  
  CONCLUSIONS
  Our data suggest that the clinical approach described here may result in a markedly improved medium-term outcome in young children with dilated cardiomyopathy. Further studies are required to evaluate whether these approaches reduce end-points such as transplantation or death.
authors.affiliation: 1Pediatric Heart Center,University of Giessen and Marburg,Giessen,Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Rupp
authors.affiliation: 1Pediatric Heart Center,University of Giessen and Marburg,Giessen,Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Apitz
authors.affiliation: 1Pediatric Heart Center,University of Giessen and Marburg,Giessen,Germany.
authors.forename: Leonie
authors.initials: L
authors.lastname: Tholen
authors.affiliation: 1Pediatric Heart Center,University of Giessen and Marburg,Giessen,Germany.
authors.forename: Heiner
authors.initials: H
authors.lastname: Latus
authors.affiliation: 1Pediatric Heart Center,University of Giessen and Marburg,Giessen,Germany.
authors.forename: Stefan H
authors.initials: SH
authors.lastname: Ostermayer
authors.affiliation: 1Pediatric Heart Center,University of Giessen and Marburg,Giessen,Germany.
authors.forename: Dorle
authors.initials: D
authors.lastname: Schmidt
authors.affiliation: 1Pediatric Heart Center,University of Giessen and Marburg,Giessen,Germany.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Bauer
authors.affiliation: 1Pediatric Heart Center,University of Giessen and Marburg,Giessen,Germany.
authors.forename: Dietmar
authors.initials: D
authors.lastname: Schranz
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D002311:Cardiomyopathy, Dilated / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000188:drug therapy*; D016027:Heart Transplantation / Q000379:methods; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D009205:Myocarditis / Q000473:pathology*; D020097:Natriuretic Peptide, Brain / Q000187:drug effects; D013318:Stroke Volume; D016277:Ventricular Function, Left / Q000187:drug effects
chemical_list: D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D020097:Natriuretic Peptide, Brain
keywords: Dilated cardiomyopathy; angiotensin-converting enzyme inhibitor; children; heart failure; pulmonary artery banding; β-blocker