EVALUATING THE PREVALENCE AND FACTORS ASSOCIATED WITH AN OPTIMAL NEURODEVELOPMENTAL OUTCOME IN CHILDREN WITH FONTAN CIRCULATION

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) /=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.