title: Two-year general and neurodevelopmental outcome after neonatal complex cardiac surgery in patients with deletion 22q11.2: a comparative study.
abstract: OBJECTIVE
  Neonatal complex cardiac surgery carries a significant risk for adverse neurodevelopmental outcome. We hypothesized this risk to be higher in patients with deletion 22q11.2.
  
  
  METHODS
  From 1996 to 2004, neonates who had complex cardiac surgery at age 6 weeks or less had multisite, multidisciplinary health and neurodevelopmental outcomes (Bayley Scales of Infant Development II; mental and psychomotor developmental indices [MDI, PDI] as mean [SD] and delay [<70]) assessed at 18 to 24 months of age. All 16 patients with deletion 22q11.2 (group 1) were compared with 16 patients without deletion 22q11.2 (group 2) having undergone neonatal complex cardiac surgery at the same center and matched for cardiac lesion, socioeconomic status, and year of operation. Outcomes were compared by univariate and multivariate analyses.
  
  
  RESULTS
  Heart lesions in each group consisted of 6 (37.5%) cases of interrupted aortic arch, 6 (37.5%) cases of truncus arteriosus, and 4 (25%) cases of tetralogy of Fallot. Outcomes were available for all survivors. Mortality was 3 (19%) and 1 (6%) in groups 1 and 2, respectively (P = .6). MDI and PDI scores were 66.1 (10.6) and 55.0 (9.4) for group 1 and 86.3 (14.6) and 82.3 (14.3) for group 2 (P < .001). Only deletion 22q11.2 was significant in stepwise multiple regression to predict MDI and PDI scores. Mental delay occurred in 8 (61.5%) of 13 in group 1 and 2 (13.8%) of 15 in group 2 (P = .016). Psychomotor delay occurred in 11 (84.6%) of 13 in group 1 and 1 (6.7%) of 15 in group 2 (P < .001).
  
  
  CONCLUSIONS
  Neonates affected by deletion 22q11.2 and having neonatal complex cardiac surgery have significantly worse neurodevelopmental outcome than do those without deletion 22q11.2.
authors.affiliation: Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Joseph
authors.initials: J
authors.lastname: Atallah
authors.forename: Ari R
authors.initials: AR
authors.lastname: Joffe
authors.forename: Charlene M T
authors.initials: CM
authors.lastname: Robertson
authors.forename: Norma
authors.initials: N
authors.lastname: Leonard
authors.forename: Patricia M
authors.initials: PM
authors.lastname: Blakley
authors.forename: Alberto
authors.initials: A
authors.lastname: Nettel-Aguirre
authors.forename: Reg S
authors.initials: RS
authors.lastname: Sauve
authors.forename: David B
authors.initials: DB
authors.lastname: Ross
authors.forename: Ivan M
authors.initials: IM
authors.lastname: Rebeyka
mesh_terms: D006348:Cardiac Surgical Procedures; D002657:Child Development; D002872:Chromosome Deletion; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000235:genetics* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009420:Nervous System / Q000254:growth & development*; D011597:Psychomotor Performance; D012307:Risk Factors; D013997:Time Factors