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The association between the modified Norwood procedure for the hypoplastic left heart syndrome and the presence of a ventricular septal defect is believed to be an important determinant of neurodevelopmental outcome. Our objective was to study a large population of infants to assess the neurodevelopmental outcome of this cohort. This was a retrospective review of infants who underwent the Norwood procedure between 1994 and 1998. We compared the outcomes of patients with and without a ventricular septal defect at any time during postoperative management. Outcome was assessed using the Bayley Scales of Infant Development-III, and developmental quotients were compared using the Mann-Whitney U test, the Student t test, the Fisher exact test, or both. One hundred thirty-four infants were identified, and 69 (51%) had a ventricular septal defect. There was a trend toward a worse intellectual outcome in the subset of infants with a ventricular septal defect (median developmental quotient, 75 versus 87). This did not achieve statistical significance, possibly reflecting the small number of patients with the defect. However, those patients with a preoperative neurologic deficit were more likely to have a developmental quotient that was less than 85 (P =.03). There was a trend toward an earlier age of death in patients with a ventricular septal defect (6.8 versus 6.3 months, P =.08). There was no difference in the cause of death, length of stay in the hospital, or survival. The mortality and morbidity of infants undergoing the modified Norwood procedure for the hypoplastic left heart syndrome was not altered by the presence of a ventricular septal defect. Infants with a ventricular septal defect had a slight tendency toward a worse developmental outcome that did not achieve statistical significance. Those infants with a preoperative neurologic deficit had worse developmental outcome and should be followed closely.Free-agent goaltender Tim Thomas hasn’t landed a job yet. But he got better.
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