Quality of life and cognitive function in Fontan patients, a population-based study.

BACKGROUND: After the Fontan procedure patients are at risk for reduced quality of life (QoL) and cognitive function. We aimed to assess these important factors in Danish Fontan patients and to compare the results with a group of healthy controls. METHODS: All Fontan patients living in Denmark were identified and invited to participate. QoL was evaluated using the Pediatric Quality of Life Inventory (PedsQL) version 4.0 generic core module in patients <16 years and the Short Form 36 questionnaire (SF-36) in patients ≥16 years. Cognitive function was evaluated in all patients ≥6 years using the Quick Test of Cognitive Speed. To evaluate if QoL correlated with exercise capacity, patients performed a symptom-limited bicycle test. RESULTS: 158 of 179 eligible patients (88%) consented to participate. Median age was 13.9 years (IQR: 10.2-19.3). PedsQL scores increased with age but were significantly lower among patients than among controls. SF-36 physical scores were significantly lower in patients compared to controls while psychosocial scores were similar. Cognitive speed was significantly reduced in patients at all ages compared to controls. No significant difference in PedsQL-/SF-36 scores or cognitive speed was found between hypoplastic left heart syndrome (HLHS) and non-HLHS Fontan patient. PedsQL-/SF-36 scores in patients ≥10 years correlated significantly to cognitive speed but not to peak exercise capacity. CONCLUSION: QoL is reduced in Fontan children compared to their healthy counterparts whereas in patients ≥16 years only physical, but not psychosocial QoL is reduced. Cognitive speed was significantly lower in patients at all ages compared to controls.

An additive model for relations between AQT single- and dual-dimension naming speed.

Color-form naming in A Quick Test of Cognitive Speed (AQT) is used to assess processing speed on three rapid automatic naming tasks, two of which measure single-dimension and the third of which measures dual-dimension naming speed. These tests have been used to identify changes in processing speed associated with normal aging. The present study evaluated whether a simple additive model could explain the normally expected relation between scores on measures of single- and dual-dimension naming speed. The AQT color (C), form (F), and color-form (CF) naming tests were administered individually to 270 adults (ages 18 to 70 yr.). Paired-sample t tests mean comparisons of C+F and CF naming times for the total group and for three age cohorts (ages 18 to 34, 35 to 54, and 55 to 70), each with 90 adults, indicated significant mean differences between these processing-speed measures for the total group and for the 18- to 34- and 35- to 54-year age cohorts. Thus, a simple additive model does not explain the relationship between single- and dual-dimension naming speed across ages. To provide a better description, an additive model with "overhead" (a measure of processing efficiency) was proposed and norm-referenced for using the AQT tests to assess normal and atypical ranges for dual-dimension processing efficiency (overhead). ANOVA with post hoc analysis (Scheffé) compared AQT C + F, CF, and overhead means across age cohorts. The results indicated significant mean differences for the CF and overhead measures, but not for the C+F measure. Normative ranges for typical overhead sizes were established for each age cohort. In clinical practice, an overhead larger than typical or normal for a given age would suggest executive dysfunction, involving attention, visual working memory, and set shifting.

Parietal lobe activation in rapid, automatized naming by adults.

Three automatic naming tasks (Wiig & Nielsen, 1999) were administered to 60 normally functioning adults. The mean time required for naming 40 single-dimension (colors, forms, numbers, and letters) and 40 dual-dimension stimuli (color-form, color-number, and color-letter combinations) were compared in young (17-38 yr.) and older (40-68 yr.) men and women. Analysis of variance for the combined groups indicated significant naming-time differences for age but not for sex. There were no significant interaction effects. For men there was a significant naming time difference between age groups for forms, and for women for colors and forms. The sex-specific analyses indicated no significant differences in naming time based on age groups for color-form, color-number, or color-letter combinations. In a second study of adult subjects (n = 14), functional brain activity was measured with regional cerebral blood flow during the performance of the color, form, and color-form naming tasks. One subject was repeatedly measured during the performance of each task, whereas 13 subjects were measured during the performance of color-form naming. In comparison to normal reference values for rest and FAS verbal fluency, blood-flow measurements showed a consistent parietal-lobe activation during form and color-form naming, but only a slight activation during color naming. During all naming tasks, a significant frontal and frontotemporal flow decrease was seen in comparison to both rest and verbal fluency reference values. This functional brain activation pattern of a parietal increase and a frontotemporal decrease was consistently confirmed across subjects during the color-form naming task.