title: Focal myocardial fibrosis assessed by late gadolinium enhancement cardiovascular magnetic resonance in children and adolescents with dilated cardiomyopathy.
abstract: BACKGROUND
  Different patterns of late gadolinium enhancement (LGE) including mid-wall fibrosis using cardiovascular magnetic resonance (CMR) have been reported in adult patients presenting with non-ischemic dilated cardiomyopathy (DCM). In these studies, LGE was associated with pronounced LV remodelling and predicted adverse cardiac outcomes. Accordingly, the purpose of our study was to determine the presence and patterns of LGE in children and adolescents with DCM.
  
  
  METHODS
  Patients <18 years of age presenting with severe congestive heart failure who were admitted for evaluation of heart transplantation at our centre underwent CMR examination which consisted of ventricular functional analysis and assessment of LGE for detection of myocardial fibrosis. Ischemic DCM was excluded by coronary angiography, and right ventricular endomyocardial biopsies ruled out acute myocarditis.
  
  
  RESULTS
  Thirty-one patients (mean age 2.1 ± 4.2 years) with severe LV dilatation (mean indexed LVEDV 136 ± 48 ml/m(2)) and LV dysfunction (mean LV-EF 23 ± 8%) were examined. LGE was detected in 5 of the 31 patients (16%) appearing in various patterns characterized as mid-wall (n = 1), focal patchy (n = 1), RV insertion site (n = 1) and transmural (n = 2). Based on histopathological analysis, 4 of the 5 LGE positive patients had lymphocytic myocarditis, whereas one patient was diagnosed with idiopathic DCM.
  
  
  CONCLUSIONS
  In children and adolescents with DCM, focal histologically proven myocardial fibrosis is rarely detected by LGE CMR despite marked LV dilatation and severely depressed LV function. LGE occurred in various patterns and mostly in patients with inflammatory cardiomyopathy. It remains unclear whether myocardial fibrosis in childhood DCM reflects different endogenous repair mechanisms that enable favourable reverse remodelling. Larger trials are needed to assess the prognostic implications of LGE in childhood DCM.
authors.affiliation: Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. heiner.latus@googlemail.com.
authors.forename: Heiner
authors.initials: H
authors.lastname: Latus
authors.affiliation: Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. kerstin.gummel@web.de.
authors.forename: Kerstin
authors.initials: K
authors.lastname: Gummel
authors.affiliation: Department of Molecular Pathology, University Hospital Tübingen, Tübingen, Germany. karin.klingel@med.uni-tuebingen.de.
authors.forename: Karin
authors.initials: K
authors.lastname: Klingel
authors.affiliation: Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. axel.moysich@paediat.med.uni-giessen.de.
authors.forename: Axel
authors.initials: A
authors.lastname: Moysich
authors.affiliation: Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. markus.khalil@paediat.med.uni-giessen.de.
authors.forename: Markus
authors.initials: M
authors.lastname: Khalil
authors.affiliation: Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. nona.mazhari@paediat.med.uni-giessen.de.
authors.forename: Nona
authors.initials: N
authors.lastname: Mazhari
authors.affiliation: Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. juergen.bauer@paediat.med.uni-giessen.de.
authors.forename: Juergen
authors.initials: J
authors.lastname: Bauer
authors.affiliation: Department of Molecular Pathology, University Hospital Tübingen, Tübingen, Germany. reinhard.kandolf@med.uni-tuebingen.de.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Kandolf
authors.affiliation: Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. dietmar.schranz@paediat.med.uni-giessen.de.
authors.forename: Dietmar
authors.initials: D
authors.lastname: Schranz
authors.affiliation: Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. capitz@aol.com.
authors.forename: Christian
authors.initials: C
authors.lastname: Apitz
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001706:Biopsy; D006328:Cardiac Catheterization; D002311:Cardiomyopathy, Dilated / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media / Q000008:administration & dosage*; D005260:Female; D005355:Fibrosis; D019786:Gadolinium DTPA / Q000008:administration & dosage*; D006333:Heart Failure / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D016027:Heart Transplantation; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000175:diagnosis / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D009206:Myocardium / Q000473:pathology*; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012720:Severity of Illness Index; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis / Q000209:etiology; D016277:Ventricular Function, Left; D020257:Ventricular Remodeling
chemical_list: D003287:Contrast Media; D019786:Gadolinium DTPA