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Evaluating the Prevalence and Factors Associated With an Optimal Neurodevelopmental Outcome in 4- to 6-Year-Old
Michael Khoury1, Morteza Hajihosseini2, Charlene M T Robertson3
1Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
Insights
More than half of children with Fontan circulation achieve optimal neurodevelopmental outcomes. Factors like female sex, maternal education, younger age at surgery, and avoiding AVV intervention are key. This research highlights modifiable factors for better outcomes.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Congenital Heart Disease
Background:
- Fontan circulation is a complex surgical procedure for single-ventricle congenital heart defects.
- Neurodevelopmental outcomes in children with Fontan circulation are a significant clinical concern.
- Optimal neurodevelopmental outcomes are not fully understood in this population.
Purpose of the Study:
- To determine the prevalence of optimal neurodevelopmental outcomes in 4- to 6-year-old children with Fontan circulation.
- To identify factors associated with optimal neurodevelopmental outcomes in this cohort.
- To explore potentially modifiable factors influencing neurodevelopmental trajectories.
Main Methods:
- A cohort study of children followed through the Western Canadian Complex Pediatric Therapies Follow-Up Program.
- Optimal neurodevelopmental outcome defined by specific scores on intelligence, visual-motor integration, and adaptive behavior assessments, plus absence of specific disabilities.
- Multivariable regression models and decision algorithms used to analyze associated variables.
Main Results:
- Optimal neurodevelopmental outcome was observed in 55% of 205 children with Fontan circulation.
- Independently associated factors included female sex, higher maternal schooling, younger age at Fontan surgery, no need for AVV intervention, and faster lactate normalization.
- A subgroup of children (31) with earlier Fontan surgery (<3.25 years), no AVV intervention, and rapid lactate normalization achieved optimal outcomes in 87% of cases.
Conclusions:
- Over half of children with Fontan circulation achieve optimal neurodevelopmental outcomes.
- Key factors associated with optimal outcomes include female sex, maternal education, younger age at Fontan surgery, and avoiding concomitant atrioventricular valve intervention.
- Younger age at surgery and avoiding AVV intervention are potentially modifiable factors that could improve neurodevelopmental outcomes.
Background:
We sought to evaluate the prevalence and factors associated with "optimal" neurodevelopmental outcomes in 4- to 6-year-old children with Fontan circulation.
Methods:
Patients followed through the Western Canadian Complex Pediatric Therapies Follow-Up Program, and born between September 1996 and December 2015, were included. Optimal neurodevelopmental outcome was defined as full-scale intelligence quotient; visual-motor integration; adaptive behaviour assessment system-general adaptive composite scores of ≥ 80 each; and the absence of chronic motor disability, permanent hearing loss, visual impairment, and seizure disorder. Multivariable regression models and decision algorithms evaluated variables associated with optimal outcomes.
Results:
The Fontan procedure was completed on 225 children, with neurodevelopmental outcome data available for 205 (mean [standard deviation]) age at Fontan 3.4 (0.9) years, 37% female). Optimal neurodevelopmental outcome was identified in 55% (112 of 205). Factors independently associated with optimal neurodevelopmental outcome were female sex (odds ratio [OR], 2.1; 95% confidence interval [CI] 1.1-4.1), years of maternal schooling (OR, 1.2 [1.1-1.4]), age at Fontan (years) (OR, 0.97 [0.94-1.0]), need for concomitant atrioventricular valve (AVV) intervention (OR, 0.4 [0.2-1.0]), and time (hours) for lactate to be ≤ 2 mmol/L (OR, 0.9 [0.8-1.0]). Of those with Fontan completion < 3.25 years, without concomitant AVV intervention and lactate normalization within 8 hours post-Fontan, 87% (27 of 31) had optimal neurodevelopmental outcomes.
Conclusions:
Optimal neurodevelopmental outcome was present in more than one-half of 4- to 6-year-old children with Fontan circulation in this cohort study, with important associated factors identified, including potentially modifiable factors such as younger age at Fontan surgery and lack of concomitant AVV intervention.
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