Objectives. The purpose of this study was to assess the neurodevelopme
ntal status of children after Fontan repair of functional single ventr
icle and to examine the relationship between cognitive function and se
lected patient characteristics. Study Design. Neurodevelopmental tests
including the Stanford-Binet Intelligence (IQ) scale and the Developm
ental Test of Visual Motor Integration (VMI) were administered to 32 c
hildren (26 months to 16 years of age) with complex single ventricle.
The mean and distribution of Ig and VMI scores were compared with popu
lation norms. The relationship between test scores and patient charact
eristics was examined utilizing analysis of variance and correlational
methods. Results. The majority of children had intellectual function
within the normal range (mean, 97.5 +/- 12.1). Below average VMI score
s were found in 21.4% of children. There were no significant correlati
ons between intellectual function or visual motor integration ability
and preoperative oxygen saturation or age at Fontan. Children who had
deep hypothermic circulatory arrest during a prior Norwood procedure t
ended to have a lower IQ score. Conclusions. Intellectual development
in children with Fontan repair of complex heart defects is essentially
within the normal range. Visual motor integration deficits may be mor
e prevalent in these children. In our population, the duration and deg
ree of preoperative hypoxemia had no apparent effect on cognitive func
tion.