Cognitive function and age at repair of transposition of the great arteries in children
Insights
Delayed surgical repair in children with d-transposition of the great arteries (dTGA) is linked to worse cognitive outcomes. Early intervention is crucial for preserving cognitive function in these complex congenital heart disease patients.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Congenital Heart Disease Research
Background:
- Chronic hypoxemia in infants can impact neurodevelopment.
- d-transposition of the great arteries (dTGA) is a cyanotic congenital heart defect requiring surgical correction.
- The timing of surgical intervention may influence long-term cognitive outcomes.
Purpose of the Study:
- To evaluate the relationship between the duration of chronic hypoxemia and cognitive function in children with dTGA.
- To determine if the age at corrective surgery affects cognitive performance in dTGA patients.
Main Methods:
- Studied 38 children with dTGA and intact ventricular septum, undergoing surgery between 6 months and 6 years.
- Administered the Wechsler Preschool and Primary Scale of Intelligence (WPPSI) and Illinois Test of Psycholinguistic Abilities (ITPA) subtests.
- Analyzed cognitive scores in relation to age at surgical repair, controlling for social index.
Main Results:
- Age at repair was inversely associated with WPPSI intelligence quotient (IQ) scores (P < 0.01).
- Later repair correlated with lower scores on visual-association (P < 0.01) and auditory-association (P < 0.1) ITPA subtests.
- Age at repair showed poor correlation with cognitive function in acyanotic congenital heart defects (e.g., ventricular septal defect).
Conclusions:
- Postponing surgical repair in dTGA is associated with progressive cognitive impairment.
- Earlier surgical correction may mitigate negative effects of chronic hypoxemia on cognitive development in dTGA.
- Findings highlight the importance of timely intervention for cyanotic congenital heart disease.
Abstract:
To assess the effect of the duration of chronic hypoxemia on cognitive function, we studied 38 children with d-transposition of the great arteries and an intact ventricular septum who underwent corrective surgery at six months to six years of age (median, 1.6 years). Tests included the Wechsler Preschool and Primary Scale of Intelligence (WPPSI) in 33 children and the visual-association and auditory-association subtests of the Illinois Test of Psycholinguistic Abilities in 38 and 37 children, respectively. When controlled for social index, age at repair was inversely associated with the WPPSI intelligence-quotient score (P less than 0.01), the visual-association subtest score (P less than 0.01), and the auditory-association subtest score (P less than 0.1). In contrast, age at repair correlated poorly with cognitive function in children with ventricular septal defect, an acyanotic congenital heart defect. These data suggest that postponing repair of a cyanotic congenital heart disease, such as transposition of the great arteries, is associated with progressive impairment of cognitive function.


