To determine whether there are differences in pulsatile cerebrospinal fluid (CSF) flow between children and adolescents with congenital heart disease (CHD) and healthy, age-matched peers, and to determine if abnormal CSF flow is associated with abnormal CSF volumes and whether it predicts executive function outcomes.

CSF flow was measured across the lumen of the aqueduct of Sylvius using cardiac-gated phase-contrast MRI at 3.0 T on 60 children and adolescents (CHD = 22, healthy controls = 38). CSF flow modeled as standard pulsatility characteristics (anterograde and retrograde peak velocities, mean velocity, and velocity variance measurements) and dynamic pulsatility characteristics (each participant's CSF flow deviation from study cohort's consensus flow quantified using the root mean squared deviation) were measured. Participants underwent neurocognitive assessments for executive function, focused on inhibition, cognitive flexibility, and working memory domains.

Compared with controls, the CHD group demonstrated greater dynamic pulsatility over the entire cardiac cycle (higher overall flow root mean squared deviation: P = .0353 for the study cohort fitted; P = .0292 for the control only fitted), but no difference in standard pulsatility measures. However, a lower mean velocity (P = .0323) and lower dynamic CSF flow pulsatility (root mean squared deviation P = .0181 for the study cohort fitted; P = .0149 for the control only fitted) predicted poor inhibitory executive functional outcomes.

Although the whole CHD group exhibited higher dynamic CSF flow pulsatility compared with controls, the subset of patients with CHD with relatively reduced static and dynamic CSF flow pulsatility had the worst inhibitory domain executive functioning. These findings suggest that altered CSF flow pulsatility may be related to not only brain compensatory mechanisms, but also to cognitive impairment in CHD.