With dramatic advances of neonatal repair of complex cardiac disease, the population of adults with congenital heart disease (CHD) has increased dramatically, and while studies have shown an increased risk of neurodevelopmental and psychological disorders in these patients, few studies have evaluated their cognitive and psychosocial outcomes. Now, a review of young adults who had an arterial switch operation for transposition of the great arteries in France has found that they have almost twice the rate of cognitive difficulties and more than triple the rate of cognitive impairment as healthy peers.
“Despite satisfactory outcomes in most adults with transposition of the great arteries (TGA), a substantial proportion has cognitive or psychologic difficulties that may reduce their academic success and quality of life,” said lead author David Kalfa, MD, PhD, of Morgan Stanley Children’s Hospital of New York-Presbyterian, Columbia University Medical Center and coauthors in the September issue of the Journal of Thoracic and Cardiovascular Surgery (
The study involved a review of 67 adults aged 18 and older born with TGA between 1984 and 1985 who had an arterial switch operation (ASO) at two hospitals in France: Necker Children’s Hospital in Paris and Marie Lannelongue Hospital in Le Plessis-Robinson. The researchers performed a matched analysis with 43 healthy subjects for age, gender, and education level.
The researchers found that 69% of the TGA patients had an intelligence quotient in the normal range of 85-115. The TGA patients had lower quotients for mean full-scale (94.9 plus or minus 15.3 vs. 103.4 plus or minus 12.3 in healthy subjects; P = 0.003), verbal (96.8 plus or minus 16.2 vs. 102.5 plus or minus 11.5; P =.033) and performance intelligence (93.7 plus or minus 14.6 vs. 103.8 plus or minus 14.3; P less than .001).
The TGA patients also had higher rates of cognitive difficulties, measured as intelligence quotient less than or equal to –1 standard deviation, and cognitive impairment, measured as intelligence quotient less than or equal to –2 standard deviation; 31% vs. 16% (P = .001) for the former and 6% vs. 2% (P = .030) for the latter.
TGA patients with cognitive difficulties had lower educational levels and were also more likely to repeat grades in school, Dr. Kalfa and coauthors noted. “Patients reported an overall satisfactory health-related quality of life,” Dr. Kalfa and coauthors said of the TGA group; “however, those with cognitive or psychologic difficulties reported poorer quality of life.” The researchers identified three predictors of worse outcomes: lower parental socioeconomic and educational status; older age at surgery; and longer hospital stays.
“Our findings suggest that the cognitive morbidities commonly reported in children and adolescents with complex CHD persist into adulthood in individuals with TGA after the ASO,” Dr. Kalfa and coauthors said. Future research should evaluate specific cognitive domains such as attention, memory, and executive functions. “This consideration is important for evaluation of the whole [adult] CHD population because specific cognitive impairments are increasingly documented into adolescence but remain rarely investigated in adulthood,” the researchers said.
Dr. Kalfa and coauthors reported having no financial disclosures.