BACKGROUND
While youth with Fontan circulation are at increased risk for adverse neurodevelopmental
outcomes, not all children encounter these limitations. We sought to evaluate the
prevalence and factors associated with “optimal” neurodevelopmental outcomes in 4-6
year old children with Fontan circulation.
METHODS AND RESULTS
Patients who underwent the Fontan procedure born between September 1996 and December
31, 2015 and followed through the Western Canadian Complex Pediatric Therapies Follow-up
Program (CPTFP) were included. All patients underwent a comprehensive neurocognitive
assessment between 48-72 months of age. A composite outcome termed optimal neurodevelopmental
outcome was defined as the absence of intellectual disability (full-scale intellectual
quotient (FSIQ) < 80, visual motor index < 80, adaptive behavior assessment system,
general adaptive composite < 80), chronic motor disability, permanent hearing loss,
visual impairment, seizure disorder, or autism spectrum disorder. Multivariable regression
models and decision algorithms (utilizing variables from the multivariable regression
analysis, with cut-points determined to optimize a global accuracy index) were generated
to evaluate demographic and clinical factors associated with optimal neurodevelopmental
outcome. The Fontan procedure was completed on 225 children in the study period, with
neurodevelopmental outcome data available for 205 (age at Fontan 3.4 +/- 0.9 years,
age at assessment 4.6 +/- 0.5 years, 37% female). Optimal neurodevelopmental outcome
was identified in 55% (112/205). In the multivariable regression analysis, factors
independently associated with an increased odds ratio of optimal neurodevelopmental
outcome were female sex [2.1 (95% confidence interval (CI) 1.1-4.1)], years of maternal
schooling [1.2 (1.1-1.5)], age at Fontan (years) [0.97 (0.9-1.0)], need for concomitant
atrioventricular valve (AVV) intervention [0.4 (0.2-1.0)], and time (hours) for lactate
to be < 2mmol/L [0.9 (0.8-1.0)]. There was a wide range in the prevalence of optimal
neurodevelopmental outcomes according to the presence or absence of clinical and demographic
variables (Table). For example, if a patient was < 3.25 years at Fontan, did not have
a concomitant AVV intervention, and the lactate was < 2mmol/L within eight hours post-operatively,
87% (27/31) had an optimal neurodevelopmental outcome. Conversely, the absence of
each of these variables (>/=3.25 years, need for AVV intervention, and >/=8 hours
to clear lactate) was associated with 7% (1/14) having optimal neurodevelopmental
outcomes.
CONCLUSION
Optimal neurodevelopmental outcome was present in over half of 4-6 year old children
with Fontan circulation in this single-center study, with important associated factors
identified, including potentially modifiable factors such as younger age at Fontan
and lack of concomitant AVV intervention. Multi-center evaluations are needed to further
evaluate this previously undefined composite outcome.
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© 2021 Published by Elsevier Inc.