title: Patient characteristics are important determinants of neurodevelopmental outcome at one year of age after neonatal and infant cardiac surgery.
abstract: OBJECTIVE
  Many studies of neurodevelopmental outcomes after neonatal and infant cardiac surgery have focused on potentially modifiable risk factors for adverse outcomes, primarily intraoperative management strategies and the use of deep hypothermic circulatory arrest. There is increasing evidence that patient-specific factors are more important determinants of outcome.
  
  
  METHODS
  We investigated predictors of neurodevelopmental outcomes at 1 year of age after neonatal and infant cardiac surgery in a subgroup of infants enrolled in a prospective study of apolipoprotein E (APOE) genotype and neurodevelopmental outcome. Children with a variety of 2-ventricle cardiac defects repaired with only 1 operation with cardiopulmonary bypass and no more than 1 episode of deep hypothermic circulatory arrest were included. Neurodevelopmental outcomes at 1 year of age included the Bayley Scales of Infant Development-II, which yield 2 indices, the Mental Developmental Index and the Psychomotor Developmental Index.
  
  
  RESULTS
  Two hundred forty-seven infants underwent surgical repair between October 1998 and April 2003 with 1 hospital death and 3 deaths before 1 year of age. Neurodevelopmental evaluation was performed in 188 (77%) of 243 survivors, including 56 patients with tetralogy of Fallot, 39 with transposition of the great arteries with intact ventricular septum, 34 with ventricular septal defects, and 59 with other defects. The median age at operation was 56 days (1-186 days), including 72 (38%) neonates. Confirmed or suspected genetic syndromes were present in 59 (31%) of 188 infants. Deep hypothermic circulatory arrest was used in 67 (35%) infants with a median duration of 34 minutes (1-80 minutes). For the entire cohort, the mean Mental Developmental Index was 90.6 +/- 14.9 and the mean Psychomotor Developmental Index was 81.6 +/- 17.2. For patients without genetic syndromes, the mean Mental Developmental Index was 93.7 +/- 13.6 and the mean Psychomotor Developmental Index was 85.1 +/- 14.6. For the entire cohort, predictors of lower scores for both the Mental Developmental Index and Psychomotor Developmental Index were presence of a confirmed or suspected genetic syndrome, lower birth weight, and presence of the APOE epsilon2 allele (all P < .04). Black race was associated with higher scores on the Psychomotor Developmental Index (P = .018). Lower nasopharyngeal temperature during cardiopulmonary bypass was associated with a lower score on the Psychomotor Developmental Index (P = .03) and was the only intraoperative factor that was a significant predictor of either the Mental or Psychomotor Developmental Index.
  
  
  CONCLUSIONS
  The strongest predictors of a worse neurodevelopmental outcome at 1 year of age were patient-specific factors including presence of a genetic syndrome, low birth weight, and presence of the APOE epsilon2 allele. Patient-specific factors eclipsed the use and duration of deep hypothermic circulatory arrest as predictors of worse neurodevelopmental outcomes.
authors.affiliation: Division of Cardiothoracic Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. gaynor@email.chop.edu
authors.forename: J William
authors.initials: JW
authors.lastname: Gaynor
authors.forename: Gil
authors.initials: G
authors.lastname: Wernovsky
authors.forename: Gail P
authors.initials: GP
authors.lastname: Jarvik
authors.forename: Judy
authors.initials: J
authors.lastname: Bernbaum
authors.forename: Marsha
authors.initials: M
authors.lastname: Gerdes
authors.forename: Elaine
authors.initials: E
authors.lastname: Zackai
authors.forename: Alex S
authors.initials: AS
authors.lastname: Nord
authors.forename: Robert R
authors.initials: RR
authors.lastname: Clancy
authors.forename: Susan C
authors.initials: SC
authors.lastname: Nicolson
authors.forename: Thomas L
authors.initials: TL
authors.lastname: Spray
mesh_terms: D053329:Apolipoprotein E2 / Q000235:genetics; D001724:Birth Weight; D006348:Cardiac Surgical Procedures / Q000009:adverse effects*; D002315:Cardiopulmonary Bypass; D002657:Child Development; D002658:Developmental Disabilities / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D005838:Genotype; D006324:Heart Arrest, Induced; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D007036:Hypothermia, Induced; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009483:Neuropsychological Tests; D011110:Polymorphism, Genetic; D012307:Risk Factors
chemical_list: D053329:Apolipoprotein E2