title: Neuropsychological performance of school-aged children after staged surgical palliation of hypoplastic left heart syndrome.
abstract: OBJECTIVE
  Despite advances in perioperative management during surgical treatment of hypoplastic left heart syndrome (HLHS), patients are still at risk for adverse neurodevelopmental sequelae including cognitive dysfunction. This study aimed to assess a neuropsychological profile of HLHS patients at school age who underwent the Norwood operation between 1996 and 2003 with deep hypothermic circulatory arrest (DHCA) or antegrade selective cerebral perfusion (ASCP), respectively.
  
  
  METHODS
  Forty HLHS patients (DHCA: n = 11 and ASCP: n = 29) were compared with healthy controls (DHCA controls: n = 10 and ASCP controls: n = 24), recruited according to age, sex and socioeconomic status. Neuropsychological assessment included non-verbal intelligence (IQ norms) and raw score measures of visual and verbal short- and long-term memory as well as executive functions, processing speed and concentration. Neuropsychological data were correlated with bypass and circulatory arrest times.
  
  
  RESULTS
  Compared with control subjects, both patient groups had average non-verbal intelligence scores [DHCA: 102 (72-112) and ASCP: 92 (70-127)], but showed reduced long-term memory capacities and decreased executive performance as well as reduced processing speed. DHCA patients, furthermore, had a reduced visual attention span, and ASCP patients scored less on the verbal learning task than controls. The duration of DHCA and cardiopulmonary bypass (CPB) was associated with visual executive functions and short-term memory. In the DHCA group, the duration of DHCA was correlated with the time to complete the Trail Making Test A measuring visual executive functions (Spearman rank correlation, rS = 0.867, P = 0.018). In patients provided with ASCP, the cumulated CPB duration was negatively correlated with the score of the block span test measuring visual short memory (rS = -0.476, P = 0.020) as well as with the copy score of the Rey Figure assessing visual executive functions (rS = -0.399, P = 0.032).
  
  
  CONCLUSIONS
  School-age children with HLHS who underwent the Norwood procedure either with DHCA or ASCP show cognitive impairments compared with healthy controls. Our data indicate deficits in specific cognitive domains such as memory, executive functions and processing speed rather than basic intellectual dysfunction.
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany.
authors.forename: Annabell
authors.initials: A
authors.lastname: Bergemann
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany jan.hansen@uk-sh.de.
authors.forename: Jan Hinnerk
authors.initials: JH
authors.lastname: Hansen
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany.
authors.forename: Ina
authors.initials: I
authors.lastname: Rotermann
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany.
authors.forename: Inga
authors.initials: I
authors.lastname: Voges
authors.affiliation: Department of Cardiovascular Surgery, University Hospital Schleswig-Holstein, Kiel, Germany.
authors.forename: Jens
authors.initials: J
authors.lastname: Scheewe
authors.affiliation: Department of Paediatric Neurology, University Hospital Schleswig-Holstein, Kiel, Germany.
authors.forename: Christine
authors.initials: C
authors.lastname: Otto-Morris
authors.affiliation: Department of General Paediatrics, University Hospital Schleswig-Holstein, Kiel, Germany.
authors.forename: Friedemann
authors.initials: F
authors.lastname: Geiger
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany.
authors.forename: Hans-Heiner
authors.initials: HH
authors.lastname: Kramer
mesh_terms: D002648:Child; D050792:Circulatory Arrest, Deep Hypothermia Induced / Q000379:methods*; D003071:Cognition / Q000502:physiology*; D003072:Cognition Disorders / Q000209:etiology*; D005260:Female; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery*; D008297:Male; D008570:Memory, Short-Term / Q000502:physiology; D009483:Neuropsychological Tests; D058327:Norwood Procedures / Q000009:adverse effects*; D010166:Palliative Care / Q000379:methods*; D012189:Retrospective Studies
keywords: Cardiopulmonary bypass; Hypoplastic left heart syndrome; Hypothermic circulatory arrest; Norwood operation