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PEDIATRICS Vol. 101 No. 4 April 1998, pp. 630-633

Neurodevelopmental Outcomes in Children With Fontan Repair of Functional Single Ventricle

Received May 1, 1997; accepted Aug 12, 1997.

Karen Uzark, Alan Lincoln, John J. Lamberti, Richard D. Mainwaring, Robert L. Spicer, and John W. Moore

From the Children's Heart Institute, San Diego, California.

Objectives.  The purpose of this study was to assess the neurodevelopmental status of children after Fontan repair of functional single ventricle and to examine the relationship between cognitive function and selected patient characteristics.

Study Design.  Neurodevelopmental tests including the Stanford-Binet Intelligence (IQ) scale and the Developmental Test of Visual Motor Integration (VMI) were administered to 32 children (26 months to 16 years of age) with complex single ventricle. The mean and distribution of IQ and VMI scores were compared with population norms. The relationship between test scores and patient characteristics 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 scores were found in 21.4% of children. There were no significant correlations 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 tended 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 more prevalent in these children. In our population, the duration and degree of preoperative hypoxemia had no apparent effect on cognitive function.

Key words: neurodevelopmental outcome, Fontan repair, single ventricle.




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