To investigate potential relationships between neuropsychological functioning and cardiac, gastroenterologic/hepatologic, and pulmonary complications in the single ventricle heart disease (SVHD) post-Fontan population.
Following the initiation of a Fontan Multidisciplinary Clinic, SVHD patients were evaluated systematically according to a clinical care pathway, and data from multiple subspecialty evaluations were collected prospectively from 2016-2019. Biomarkers of cardiology, pulmonary, and hepatology/gastroenterology functioning were abstracted, along with neuropsychological testing results. Bivariate correlations and regression analyses examined cross-sectional relationships between physiological predictors and neuropsychological outcomes.
The sample included a cohort of 68 SVHD youth age 3-19 years, post-Fontan palliation. Sleep-disordered breathing was related to poorer visual-motor integration skills (r = -.33, p < .05) and marginally related to poorer executive functioning (r = -.33, P = .05). Lower arterial blood oxygen content was related to poorer executive functioning (r = .45, p < .05). Greater atrioventricular valve regurgitation was related to lower parent-rated adaptive functioning (ρ = -.34, p < .01). These results were maintained in regression analyses controlling for history of stroke and/or seizures.
We demonstrated associations between neuropsychological functioning and potentially modifiable aspects of physiological functioning in a prospectively-evaluated cohort of SVHD with Fontan physiology. Our findings emphasize the importance of multidisciplinary screening and care post-Fontan and suggest avenues for intervention that may improve patient outcomes and quality of life.

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