Cognitive Functioning

Neuropsychiatric impairment in CHD has been well documented since the first assessments over 50 years ago (Bret and Kohler 1956). Assessments using a variety of standardized cognitive tests (e.g., Bayley Scales of Infant Development, Cattell Infant Intelligence Scale, McCarthy Scales of Children's Abilities, Stanford-Binet Intelligence Scale, Wechsler Intelligence Scale for Children—Revised) have shown lower mean IQ scores for youngsters with cyanotic heart lesions compared with physically healthy children and those with acyanotic heart lesions (DeMaso et al. 1990; Gonzalez-Pardo et al. 1981; Kramer et al. 1989; Morris et al. 1993; Sil-bert et al. 1969). Cyanotic heart lesions have been associated with developmental and neurological abnormalities in as many as 25% of child survivors of cardiac surgery (Ferry 1987).

The current trend of early surgical correction of heart lesions in infancy is strongly supported by studies showing that IQ scores were inversely associated with age at surgical intervention (Newburger et al. 1984; O'Dougherty et al. 1983). Despite improved surgical techniques and repairs in children under age 3 months, children with transposition of the great arteries continue to demonstrate mean IQs that are below population norms (Bellinger et al. 1999). Of note, lower mean IQ scores found in all of the above studies were consistently within the normal range of intelligence.

Neuropsychiatric deficits in motor, speech, and language functioning have been identified as particularly problematic in children with cyanotic heart disease. Perceptual motor and gross motor abilities are significantly lower in children with cyanotic lesions than in children with acyanotic abnormalities (Newburger et al. 1984; Silbert et al. 1969). Children with transposition of the great arteries have performed below expectation on visual-motor integration, motor function, oromotor control, and expressive language measures (Bellinger et al. 1997, 1999). Similar neuropsychiatric deficits have been found even with the innovative development of the Fontan repair of complex heart defects (Uzark et al. 1998; Wernovsky et al. 2000). Overall, children with heart disease, particularly those with cyanotic heart lesions, are at significant risk for adverse motor and language deficits that may lead to adverse academic performance and the need for assessment and intervention in the school setting (Bellinger et al. 1995, 1999; Miatton et al. 2007; Newburger et al. 1984; Wray and Radley-Smith 2006).

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