title: Structural cerebral abnormalities and neurodevelopmental status in single ventricle congenital heart disease before Fontan procedure.
abstract: Neonates with single ventricle congenital heart disease are at risk for structural cerebral abnormalities. Little is known about the further evolution of cerebral abnormalities until Fontan procedure.
  
  
  
  Between August 2012 and July 2015, we conducted a prospective cross-sectional two centre study using cerebral magnetic resonance imaging (MRI) and neuro-developmental outcome assessed by the Bayley-III. Forty-seven children (31 male) were evaluated at a mean age of 25.9 ± 3.4 months with hypoplastic left heart syndrome (25) or other single ventricle (22).
  
  
  
  Cerebral MRI was abnormal in 17 patients (36.2%) including liquor space enlargements (10), small grey (9) and minimal white (5) matter injuries. Eight of 17 individuals had combined lesions. Median (range) cognitive composite score (CCS) (100, 65-120) and motor composite score (MCS) (97, 55-124) were comparable to the reference data, while language composite score (LCS) (97, 68-124) was significantly lower ( P  = 0.040). Liquor space enlargement was associated with poorer performance on all Bayley-III subscores (CCS: P  = 0.02; LCS: P  = 0.002; MCS: P  = 0.013). The number of re-operations [odds ratio (OR) 2.2, 95% confidence interval (CI) 1.1-4.3] ( P  = 0.03) and re-interventions (OR 2.1, 95% CI 1.1-3.8) ( P  = 0.03) was associated with a higher rate of overall MRI abnormalities.
  
  
  
  Cerebral MRI abnormalities occur in more than one third of children with single ventricle, while the neuro-developmental status is less severely affected before Fontan procedure. Liquor space enlargement is the predominant MRI finding associated with poorer neuro-developmental status, warranting further studies to determine aetiology and further evolution until school-age.
authors.affiliation: Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital Zurich, Switzerland.|Children's Research Center, University Children's Hospital Zurich, Switzerland.
authors.forename: Walter
authors.initials: W
authors.lastname: Knirsch
authors.affiliation: Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital Zurich, Switzerland.|Children's Research Center, University Children's Hospital Zurich, Switzerland.|Diagnostic Imaging, MR center, University Children's Hospital Zurich, Switzerland.|Child Development Center, University Children's Hospital Zurich, Switzerland.
authors.forename: Kristina Nadine
authors.initials: KN
authors.lastname: Mayer
authors.affiliation: Children's Research Center, University Children's Hospital Zurich, Switzerland.|Diagnostic Imaging, MR center, University Children's Hospital Zurich, Switzerland.
authors.forename: Ianina
authors.initials: I
authors.lastname: Scheer
authors.affiliation: Children's Research Center, University Children's Hospital Zurich, Switzerland.|Diagnostic Imaging, MR center, University Children's Hospital Zurich, Switzerland.
authors.forename: Ruth
authors.initials: R
authors.lastname: Tuura
authors.affiliation: Pediatric Heart Center, University Hospital Giessen, Germany.
authors.forename: Dietmar
authors.initials: D
authors.lastname: Schranz
authors.affiliation: Pediatric Neurology, University Hospital Giessen, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Hahn
authors.affiliation: Child Development Center, Frankfurt/Main, Germany.
authors.forename: Kristina
authors.initials: K
authors.lastname: Wetterling
authors.affiliation: Children's Research Center, University Children's Hospital Zurich, Switzerland.|Child Development Center, University Children's Hospital Zurich, Switzerland.
authors.forename: Ingrid
authors.initials: I
authors.lastname: Beck
authors.affiliation: Children's Research Center, University Children's Hospital Zurich, Switzerland.|Child Development Center, University Children's Hospital Zurich, Switzerland.
authors.forename: Beatrice
authors.initials: B
authors.lastname: Latal
authors.affiliation: Pediatric Heart Center, University Hospital Giessen, Germany.
authors.forename: Bettina
authors.initials: B
authors.lastname: Reich
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging* / Q000523:psychology; D001921:Brain / Q000002:abnormalities* / Q000000981:diagnostic imaging; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000523:psychology / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis / Q000523:psychology / Q000601:surgery; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D065886:Neurodevelopmental Disorders / Q000209:etiology*; D059906:Neuroimaging / Q000379:methods; D009483:Neuropsychological Tests
keywords: Congenital heart disease ; MRI ; Neuroimaging; Outcome 