title: Risk factors for adverse neurocognitive outcomes in school-aged patients after the Fontan operation.
abstract: OBJECTIVE
  As the surgical results for Fontan candidates improve, much attention has been given to the long-term cognitive outcomes. This study aimed to assess the neurocognitive outcomes after Fontan completion, and to determine the factors associated with an extremely low intelligence quotient (IQ <70).
  
  
  METHODS
  A total of 70 local eligible school-aged patients were enrolled in the study (hypoplastic left heart syndrome, 17; right atrial isomerism, 15 and others, 38). Fontan operations were performed at a median age of 1.8 (range, 0.5-8.9) years (primary Fontan, 4 of 70 [6%]). The Wechsler Intelligence Test was taken at a median age of 9 (range, 5.1-14.4) years for the purpose of neurocognitive evaluation. Patients' data were collected from medical records, and a retrospective analysis of potential predictors for an IQ <70 was performed.
  
  
  RESULTS
  The median full-scale IQ (FSIQ) for the entire cohort was 85 (range, 43-118). Of the lower order composites, the perceptual reasoning index and the working memory index were significantly lower in low-FSIQ patients (P < 0.05). Overall, 15 of 70 (21%) of the cohort had an IQ <70. Univariate analysis identified three significant risk factors for FSIQ <70 (15 of 70, 21%): body weight <2.5 kg at initial palliative surgery (P < 0.05), low 5-min Apgar score <4 (P < 0.05) and inter-stage events requiring cardiopulmonary resuscitation (CPR) (P < 0.05). No other patient-specific factors (e.g. cardiac morphology) or modifiable surgical factors (e.g. the use of hypothermic cardiac arrest) were associated with FSIQ <70.
  
  
  CONCLUSIONS
  Low body weight (<2.5 kg) at initial operation, low 5-min Apgar score (<4) and inter-stage CPR were significant risk factors for impaired neurocognitive outcomes. An evolving strategy for preventing inter-stage CPR may improve cognitive outcomes.
authors: Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan.
authors: Ai
authors: A
authors: Sugimoto
authors: Noritaka
authors: N
authors: Ota
authors: Keijiro
authors: K
authors: Ibuki
authors: Chisato
authors: C
authors: Miyakoshi
authors: Masaya
authors: M
authors: Murata
authors: Yuko
authors: Y
authors: Tosaka
authors: Toru
authors: T
authors: Yamazaki
authors: Kisaburo
authors: K
authors: Sakamoto
mesh_terms: D000704:Analysis of Variance; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018729:Fontan Procedure / Q000009:adverse effects* / Q000379:methods; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000209:etiology*; D007361:Intelligence Tests; D011183:Postoperative Complications / Q000209:etiology; D012189:Retrospective Studies; D012307:Risk Factors
keywords: Adverse effects; Fontan procedure; Hypoplastic left heart syndrome; Intelligence tests; Neurobehavioural manifestations; Right atrial isomerism