Neuropsychological Status and Structural Brain Imaging in Adolescents With Single Ventricle Who Underwent the Fontan Procedure

Abstract

BackgroundFew studies have described the neuropsychological outcomes and frequency of structural brain or genetic abnormalities in adolescents with single ventricle who underwent the Fontan procedure.Methods and ResultsIn a cross‐sectional, single‐center study, we enrolled 156 subjects with single ventricle, mean age 14.5±2.9 years, who had undergone the Fontan procedure. Scores in the entire cohort on a standard battery of neuropsychological tests were compared with those of normative populations or to those of a group of 111 locally recruited healthy adolescents. They also underwent brain magnetic resonance imaging and were evaluated by a clinical geneticist. Genetic abnormalities were definite in 16 subjects (10%) and possible in 49 subjects (31%). Mean Full‐Scale IQ was 91.6±16.8, mean Reading Composite score was 91.9±17.2, and mean Mathematics Composite score was 92.0±22.9, each significantly lower than the population means of 100±15. Mean scores on other neuropsychological tests were similarly lower than population norms. In multivariable models, risk factors for worse neuropsychological outcomes were longer total support and circulatory arrest duration at first operation, presence of a genetic abnormality, more operations and operative complications, more catheterization complications, and seizure history. The frequency of any abnormality on magnetic resonance imaging was 11 times higher among Fontan adolescents than referents (66% versus 6%); 19 (13%) patients had evidence of a stroke, previously undiagnosed in 7 patients (40%).ConclusionsThe neuropsychological deficits and high frequencies of structural brain abnormalities in adolescents who underwent the Fontan procedure highlight the need for research on interventions to improve the long‐term outcomes in this high‐risk group.

Reduced cortical volume and thickness and their relationship to medical and operative features in post-Fontan children and adolescents

“…Post-Fontan brain MRI studies of SV patients are few, but suggest that evidence of brain injury is still present in some patients into childhood (14,15). Recently, we reported increased number of gross anatomic brain abnormalities by MRI in our current sample of SV patients 10-19 y of age as compared to a referent sample (7). Accompanying these differences in brain structure are deficits in several cognitive and behavioral domains including attention, memory, executive function, motor function, and visual-spatial processing.…”

“…Consequently, focus has turned progressively toward neurologic injury and its effects on neurodevelopmental outcomes in the aging SV population. Studies of the burgeoning SV population now reaching adulthood have revealed a more complete picture of their longterm neurodevelopment, including cognitive deficits similar to those seen in other CHD, but greater in severity (7,8). The structural correlates of these cognitive and neurodevelopmental deficits have not been fully defined.…”

Executive Function in Children and Adolescents with Critical Cyanotic Congenital Heart Disease

“…Of note, broad neurobehavioral outcomes from the three larger studies, including a D-KEFS composite score and BRIEF General Executive Composite scores, have been described in previous reports (Bellinger et al, 2011, 2014a, 2014b). The present study provides a detailed analysis of EF outcomes, utilizing individual D-KEFS subtest scores and BRIEF subscale/index scores that have not been published elsewhere.…”

“…Data were compiled from three large-scale, single-center studies: 1) the Boston Circulatory Arrest Study of children/adolescents with TGA (Bellinger et al, 2011); 2) a study of children/adolescents with TOF (Bellinger et al, 2014a); and 3) a study of children/adolescents with single-ventricle cardiac anatomy who underwent the Fontan operation (SVF; Bellinger et al, 2014b). All three studies included extensive neuropsychological evaluation (lasting approximately 4 hours).…”

Genetic contribution to neurodevelopmental outcomes in congenital heart disease: are some patients predetermined to have developmental delay?

“…In one large cohort of adolescents with single ventricle, nearly 90% of subjects required developmental/educational services; over one-third had received special education and nearly a quarter experienced grade retention. [12] Given the burgeoning number of survivors with critical CHD, neurodevelopmental and behavioral disabilities affect not only the individual child and family, but also exert a significant societal burden.…”