Survivors of palliative surgery for single ventricle heart disease (SVHD) are at risk of poor neurodevelopmental outcomes and reduced exercise capacity. In healthy populations, reduced exercise capacity is related to decreased cognition suggesting a possible relationship between exercise capacity and neurodevelopment. Using cardiopulmonary exercise testing (CPET) and neuropsychological testing (NPT) as indicators of exercise capacity and neurodevelopment, respectively, we hypothesized that in SVHD, higher CPET measures are related to better NPT performance. Patients were retrospectively identified. CPET variables included VO2max, anaerobic threshold, peak heart rate, ventilatory efficiency, and respiratory exchange ratio. NPT instruments were divided into domains measuring attention, executive functioning, adaptive functioning, and emotional functioning. Linear regression was used to test for associations between CPET and NPT. 23 subjects with SVHD met inclusion criteria. On both CPET and NPT, the cohort scored worse than healthy, age-matched subjects. Higher VO2max and anaerobic threshold were associated with better parent-rated overall adaptive functioning (p = 0.01 and p = 0.02, respectively). Higher peak heart rate was related to better sustained visual attention (p = 0.01). In SVHD, CPET measures indicating better exercise capacity were positively associated with a subset of scores on NPT. Larger, multisite studies implementing cardiorespiratory fitness intervention and incorporating cognitive outcome measures will be needed to better characterize the relationship between neurodevelopment and functional capacity in this population. Results may assist in providing anticipatory guidance and optimizing post-Fontan developmental trajectories.
Keywords: Cardiopulmonary exercise testing; Exercise capacity; Fontan; Neurodevelopment; Neuropsychological testing; Single ventricle heart disease.