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Neurodevelopmental outcomes in children with Fontan repair of functional single ventricle
K Uzark1, A Lincoln, J J Lamberti
1Children's Heart Institute, San Diego, California 92123, USA.
Insights
Children undergoing Fontan repair for single ventricle heart defects generally show normal intellectual development. However, visual motor integration deficits are more common in these patients.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Congenital Heart Disease
Background:
- Functional single ventricle is a complex congenital heart defect.
- The Fontan procedure is a palliative surgery for single ventricle physiology.
- Neurodevelopmental outcomes after Fontan palliation require ongoing assessment.
Purpose of the Study:
- To evaluate the neurodevelopmental status of children post-Fontan repair.
- To investigate the relationship between cognitive function and patient characteristics in this cohort.
Main Methods:
- Utilized the Stanford-Binet Intelligence (IQ) scale and Developmental Test of Visual Motor Integration (VMI).
- Assessed 32 children aged 26 months to 16 years with complex single ventricle.
- Compared scores to population norms and analyzed correlations with patient factors.
Main Results:
- Most children exhibited normal intellectual function (mean IQ 97.5 +/- 12.1).
- A significant portion (21.4%) presented with below-average VMI scores.
- No significant correlation found between cognitive function and preoperative oxygen saturation or age at Fontan repair.
Conclusions:
- Intellectual development is typically within the normal range following Fontan repair.
- Visual motor integration deficits appear more prevalent in children with repaired single ventricle defects.
- Preoperative hypoxemia duration and severity did not significantly impact cognitive outcomes in this study.
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.
