Abstract
Neurodevelopmental sequelae are prevalent among patients with congenital heart defects (CHD). In a study of infants and children with repaired tetralogy of Fallot (TOF), we sought to identify those at risk for abnormal neurodevelopment and to test associations between socioeconomic and medical factors with neurodevelopment deficits. Single-center retrospective observational study of patients with repaired TOF that were evaluated at the institution’s Cardiac Kids Developmental Follow-up Program (CKDP) between 2012 and 2018. Main outcomes included neurodevelopmental test scores from the Bayley Infant Neurodevelopmental Screener (BINS), Peabody Developmental Motor Scale (PDMS), and Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III). Mixed effects linear regression and marginal logistic regression models tested relationships between patient characteristics and outcomes. Sub-analyses were conducted to test correlations between initial and later neurodevelopment tests. In total, 49 patients were included, predominantly male (n = 33) and white (n = 28), first evaluated at a median age of 4.5 months. Forty-three percent of patients (n = 16) had deficits in the BINS, the earliest screening test. Several socioeconomic parameters and measures of disease complexity were associated with neurodevelopment, independently of genetic syndrome. Early BINS and PDMS performed in infancy were associated with Bayley-III scores performed after 1 year of age. Early screening identifies TOF patients at risk for abnormal neurodevelopment. Socioeconomic factors and disease complexity are associated with abnormal neurodevelopment and should be taken into account in the risk stratification and follow-up of these patients. Early evaluation with BINS and PDMS is suggested for detection of early deficits.
Highlights
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect (CHD), with a yearly incidence of one per 2500 live births in the United States [1]
There were no differences in neighborhood-level characteristics between patients with follow-up in the Cardiac Kids Developmental Follow-up Program (CKDP) and those that were lost to follow up
We provide a preliminary analysis of the neurodevelopmental status of infants and children with TOF, and report on factors associated with neurodevelopmental outcomes
Summary
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect (CHD), with a yearly incidence of one per 2500 live births in the United States [1]. The Bayley Infant Neurodevelopmental Screener (BINS) has been used to detect early deficits in high-risk neonatal groups, including premature infants and those with other congenital anomalies, allowing for risk stratification and intervention. To our knowledge, these early tests have not been studied in patients with congenital heart disease, in particular in those with TOF [11, 12]. The association of social determinants of health with overall outcomes in children is well known, but the association between socioeconomic factors and neurodevelopment status has not been extensively studied in patients with TOF [13,14,15]
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