NEUROLOGICAL AND DEVELOPMENTAL OUTCOME IN TOF

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. AIM 1. To compare TOF and control groups on academic achievement, cognition and behavior. Hypothesis: Children with repaired TOF who do not possess 22q11 microdeletion will demonstrate academic, cognitive, and behavioral deficiencies relative to normal matched controls. The primary outcome will be Total Composite Score on the Wechsler Individual Achievement Test (WIAT). Academic success, as assessed by the WIAT, represents the outcome of factors including intelligence, neuropsychological strengths and weaknesses, and mental health. Therefore, secondary outcomes will include the following: (1) ability, measured by the Full-Scale, Verbal, and Performance IQ on the Wechsler Intelligence Scale for Children-Third Edition (WISC-III); (2) learning disability, defined as a significant discrepancy between a child's ability and achievement within particular academic domains; (3) performance in specific neuropsychologic domains, including language, memory, attention, executive functions, and visual-spatial/motor integration; and (4) mental health, including quality-of-life and global emotional functioning, as well as specific psychiatric diagnoses including mood, anxiety, attention-deficit/disruptive behavior, and psychotic disorders. AIM 2. To compare TOF and control groups on measures of brain structure. Hypothesis: Children with repaired TOF who do not possess the 22q11 deletion will have findings on structural MRI that differ from matched controls. The primary outcome using quantitative volumetric MRI imaging and segmentation will be total brain tissue volume. Secondary outcomes will be volumes of gray matter (total, cortical, and subcortical gray matter), white matter (myelinated and unmyelinated white matter), and cerebrospinal fluid. The primary outcome variable using diffusion tensor MRI imaging will be regional white matter microstructure, as measured by relative anisotropy (RA).