Relationships between Physiologic and Neuropsychologic Functioning after Fontan

Kelly R. Wolfe, Deborah R. Liptzin, Dania Brigham, Sarah L. Kelly, Carey Rafferty, Megan Albertz, Adel K. Younoszai, Michael V. Di Maria

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


Objective: To investigate potential relationships between neuropsychologic functioning and cardiac, gastroenterologic/hepatologic, and pulmonary complications in the single ventricle heart disease (SVHD) post-Fontan population. Study design: Following the initiation of a Fontan Multidisciplinary Clinic, patients with SVHD were evaluated systematically according to a clinical care pathway, and data from multiple subspecialty evaluations were collected prospectively from 2016 to 2019. Biomarkers of cardiology, pulmonary, and hepatology/gastroenterology functioning were abstracted, along with neuropsychologic testing results. Bivariate correlations and regression analyses examined cross-sectional relationships between physiologic predictors and neuropsychologic outcomes. Results: The sample included a cohort of 68 youth with SVHD age 3-19 years, after Fontan palliation. Sleep-disordered breathing was related to poorer visual-motor integration skills (r = −0.33; P < .05) and marginally related to poorer executive functioning (r = −0.33; 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 = −0.34; P < .01). These results were maintained in regression analyses controlling for history of stroke and/or seizures. Conclusions: We demonstrated associations between neuropsychologic functioning and potentially modifiable aspects of physiologic functioning in a prospectively evaluated cohort of patients with SVHD with Fontan physiology. Our findings emphasize the importance of multidisciplinary screening and care after a Fontan procedure and suggest avenues for intervention that may improve patient outcomes and quality of life.

Original languageEnglish
Pages (from-to)239-246
Number of pages8
JournalJournal of Pediatrics
StatePublished - Dec 2020


  • cognition
  • hypoplastic left heart
  • multiorgan comorbidities
  • neurodevelopment
  • pediatric neuropsychology
  • single ventricle


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