Objective: For most newborns, congenital heart defects (CHD) appear to be isolated anomalies and the brain is presumed to have normal developmental potential. Most studies of neurodevelopmental ...outcomes have focused on operative management strategies. Methods: Infants with complex CHD and no identified syndromes other than 22q11 microdeletions enrolled in a study of apolipoprotein E (APOE) polymorphisms and developmental outcome were evaluated at one year of age; including genetic evaluation and the Bayley Scales of Infant Development-II mental (MDI) and psychomotor developmental indices (PDI). Results: Five hundred and fifty infants enrolled and 359 (20 with 22q11) of 501 survivors (72%) returned. Mean MDI was 90 ± 15 and PDI was 78 ± 18. Genetic syndromes not identified at birth were confirmed in 28 (8.1%) and suspected in 51 (15.0%). By multivariable analysis, suspected/confirmed genetic syndromes and APOE ɛ2 allele predicted lower MDI and PDI, all p ≪ 0.04. Lower birth weight (p ≪ 0.001) and preoperative intubation (p = 0.012) predicted lower MDI. Higher hematocrit during the initial operation was associated with higher MDI (p = 0.007). Longer postoperative length of stay was predictive of lower PDI (p = 0.002). Additional operations with cardiopulmonary bypass were associated with lower MDI and PDI (both p ≪ 0.002), but use of deep hypothermic circulatory arrest was not. Conclusions: Patient factors (birth weight and preoperative status) are significant determinants of neurodevelopmental outcomes as opposed to operative management strategies. In this cohort, genetic syndromes unsuspected at birth were surprisingly common and correlate with poor neurodevelopmental outcomes. Without multiple congenital anomalies, syndromes may be missed in infancy. Genetic evaluation should be considered in all infants with CHD.
Objectives To determine whether a cardiac diagnosis is a predictor of neurodevelopmental outcomes after infant cardiac surgery. Methods Infants with ventricular septal defect (VSD), tetralogy of ...Fallot (TOF), transposition of the great arteries (TGA), and hypoplastic left heart syndrome (HLHS) in a study of apolipoprotein E ( APOE ) polymorphisms, and neurodevelopmental outcome underwent neurodevelopmental and genetic evaluation at 4 years of age. The domains tested included cognition, language, speech, memory, executive function, visual-motor, fine motor, and reading and math skills. Results Testing was completed in 178 patients with normal genetic evaluations: VSD (n = 26), TOF (n = 44), TGA (n = 41), and HLHS (n = 67). No differences were found in gestational age, ethnicity, APOE genotype, socioeconomic status, or maternal education among groups. Patient age at the first surgery was significantly lower for patients with TGA and HLHS compared with those with TOF and VSD. The postoperative length of stay was significantly longer for HLHS than all other groups and for TGA compared with TOF and VSD. HLHS correlated significantly with the use of deep hypothermic circulatory arrest and multiple operations. The mean scores for each domain were within normal limits for all groups. Compared with the other patients, those with HLHS had significantly lower scores for cognition, fine motor skills, executive function, and math skills. No significant differences were found among the TGA, TOF or VSD patients for any domain. Significant impairments in at least 1 domain were identified in 8% (2/25) of patients with VSD, 20% (8/41) with TOF, 17% (7/41) with TGA, and 18% (12/65) with HLHS. After correction for the demographic, preoperative, and operative variables, no significant differences were found among the groups for any domain. Conclusions The mean scores for the neurodevelopmental outcomes domains tested were in the normal range for preschool children with no recognized genetic syndromes after surgery for VSD, TOF, TGA, and HLHS. In each diagnostic group, the number of children with impairments in at least 1 domain increased compared with the general population. Unadjusted neurodevelopmental outcomes for HLHS were lower for cognition, fine motor skills, executive function, and math skills compared with the other patients. After correction for the demographic, preoperative, and operative variables, no significant differences were found among the groups for any domain. The specific cardiac diagnosis determines a large portion of the variation in these covariates. Therefore, although HLHS did predict for poorer outcomes in some domains, it did not add predictive power to the other factors considered.
Objective The occurrence of an electroencephalographic (EEG) seizure after surgery for complex congenital heart defects has been associated with worse neurodevelopmental (ND) outcomes. We previously ...identified postoperative seizures documented by 48-hour EEG monitoring in 11% of 178 neonates and infants. Evaluation at 1 year of age did not identify an adverse effect of an EEG seizure on ND outcomes. The current study was undertaken to determine if testing in the preschool period would identify deficits that become apparent as children develop. Methods The ND outcomes assessed at 4 years of age included cognition, language, attention, impulsivity, executive function, behavior problems, academic achievement, and visual and fine motor skills. Results Developmental evaluations were performed in 132 (87%) of 151 survivors. For the entire cohort, the Full-Scale IQ was 95.0 ± 18.5. IQ was 95.1 ± 18.7 for patients without a history of seizure and 93.6 ± 16.7 for those with a history of seizure. After covariate adjustment, occurrence of an EEG seizure was associated with worse executive function ( P = .037) and impaired social interactions/restricted behavior ( P = .05). Seizures were not significantly associated with worse performance for cognition, language, attention, impulsivity, academic achievement, or motor skills (all P > .1). Conclusions The occurrence of a postoperative seizure is a biomarker of brain injury. This study confirms that postoperative EEG seizures are associated with worse ND outcomes, characterized by impairments of executive function and a higher prevalence of deficits in social interactions and repetitive/restricted behaviors in preschool survivors of cardiac surgery in infancy. However, EEG seizures were not associated with worse cognitive, language, or motor skills.
Abstract Objectives We sought to assess the effect of congenital heart disease requiring infant surgery with cardiopulmonary bypass on neurodevelopmental outcomes and growth at 4 years of age, while ...matching for gestational age, socioeconomic status, maternal gestational conditions, home environment, and parental intelligence by studying multiple-gestation births. Methods We performed within-family comparison of 14 multiple-gestation births in which 1 child had congenital heart disease requiring surgery with cardiopulmonary bypass at ≤6 months of age. Between 4 and 5 years of age, a comprehensive neurodevelopmental assessment was performed. Paired comparisons were conducted between siblings with and without heart defects using a series of nonparametric tests. Results On average, the children qualified as late preterm (mean gestational age 35.4 ± 2.6 weeks). At an average age of 4.8 ± 0.1 years, children with congenital heart disease weighed less than their siblings (median weight for age z score −0.4 vs 0.1, P = .02) and had worse performance for cognition (median full-scale IQ 99 vs 109, P = .02) and fine motor skills (median Wide Range Assessment of Visual Motor Ability, Fine Motor score 94.5 vs 107.5, P < .01). Conclusions After controlling for socioeconomic status, home environment, parental intelligence, and gestational factors by using multiple-gestation births, congenital heart disease requiring surgery with cardiopulmonary bypass at ≤6 months of age is associated with lower weight, cognitive abilities and fine motor skills at 4 years of age.
Objective Evaluate the impact of near-term delivery on neurodevelopmental (ND) outcomes in children with congenital heart disease (CHD). Methods Secondary analysis of data from a study of genetic ...polymorphisms and ND outcomes after cardiac surgery in infants. The effect of gestational age (GA) as a continuous variable on ND outcomes was evaluated using general linear regression models. GA was also evaluated as a categorical variable to seek a threshold for better outcomes. ND domains tested at 4 years of age included cognition, language skills, attention, impulsivity, memory, executive function, social competence, visual-motor, and fine-motor skills. Results ND outcomes and GA were available for 378 infants. Median GA was 39 weeks (range, 28-42 weeks) with 351 born at 36 weeks or more (near-term/term). In univariate analysis of the near-term/term subgroup, older GA predicted better performance for cognition, visual-motor, and fine-motor skills. After covariate adjustment, older GA predicted better performance for fine-motor skills ( P = .018). Performance for cognition, language, executive function, social skills, visual-motor, and fine-motor skills was better for those born at 39 to 40 weeks of GA or more versus those born at less than 39 weeks (all P < .05). Conclusions These findings are consistent with the hypothesis that delivery before 39 to 40 weeks of GA is associated with worse outcomes in patients with CHD. Early delivery of a child with CHD is often indicated because of maternal or fetal health issues. In the absence of these concerns, these data suggest that elective (or spontaneous) delivery at 39 to 40 weeks of GA is associated with better ND outcomes.
Abstract Objective To explore barriers to early intervention (EI) evaluation among referred infants and toddlers. Methods We conducted semistructured interviews with parents of children referred for ...EI services and with EI staff. We purposively sampled families according to whether they received an EI evaluation. Families were recruited from a randomized controlled trial testing implementation of developmental screening. Parents filled out demographic surveys. Interviews were recorded, transcribed, and coded. We identified themes within and across respondent groups using modified grounded theory. Results We reached thematic saturation after interviewing 22 parents whose child was evaluated by EI, 22 not evaluated, and 14 EI employees. Mean child age at first referral was 16.7 months, and 80% were referred as the result of language concerns. We identified 5 primary themes: (1) Parents reported communication problems with their pediatrician, including misinterpreting reassurance and not understanding the referral process; (2) Many parents saw themselves as experts on their child's development and felt they should decide whether their child pursues EI services; (3) Some families preferred to wait for the developmental concern to resolve or work with their child on their own prior to seeking EI services; (4) For ambivalent parents, practical obstacles especially limited completion of evaluation, but highly motivated parents overcame obstacles; and (5) EI employees perceived that families avoid evaluation because they mistake EI for child protective services. Conclusions Communication between pediatricians and families that addresses practical logistics, families' perceptions of their child's development and EI, and motivation to address developmental concerns may improve the completion of EI referrals.
The goal was to evaluate polymorphisms of the APOE gene as modifiers of neurobehavioral outcomes for preschool-aged children with congenital heart defects, after cardiac surgery.
A prospective ...observational study with neurodevelopmental evaluation between the fourth and fifth birthdays was performed. Attention and behavioral skills were assessed through parental report.
Parents of 380 children completed the neurobehavioral measures. Child Behavior Checklist scores for the pervasive developmental problem scale were in the at-risk or clinically significant range for 15% of the cohort, compared with 9% for the normative data (P < .00001). Attention problem scores were in the at-risk or clinically significant range for 12% of the cohort, compared with 7% for the normative data (P = .0002). The Attention-Deficit/Hyperactivity Disorder Rating Scale-IV, Preschool Version, was completed for 378 children; 30% scored in the clinically significant range for inattention and 22% for impulsivity. After adjustment for covariates, the APOE epsilon2 allele was significantly associated with higher scores (worse problems) for multiple Child Behavior Checklist indices, including somatic complaints (P = .009), pervasive developmental problems (P = .032), and internalizing problems (P = .009). In each case, the epsilon4 allele was associated with a better outcome. APOE epsilon2 carriers had impaired social skills, compared with epsilon4 carriers (P = .009).
For preschool-aged children with congenital heart defects requiring surgery, parental rating scales showed an increased prevalence of restricted behavior patterns, inattention, and impaired social interactions. The APOE epsilon2 allele was associated with increased behavior problems, impaired social interactions, and restricted behavior patterns.
To determine if early literacy promotion, which consisted of board books and reading promotion beginning with newborns, is more effective than standard literacy promotion beginning at 6 months.
...Hybrid type 1 randomized controlled implementation trial of Medicaid-eligible newborns. Prior to 6 months of age, early literacy promotion participants received board books and reading promotion at well visits plus weekly text messages on reading, while standard literacy promotion participants only received weekly text messages on safety. Both groups received board books and reading promotion at well visits after 6 months as part of Reach Out and Read. Measures included proportion who received board books to assess implementation and StimQ Read Subscale (SQRS) scores and Preschool Language Scale-Fifth Edition (PLS-5) scores at 6 and 24 months to assess outcomes. Differences in measures were assessed using intention-to-treat analysis.
Of 120 newborns enrolled, most were African American, resided with a single parent, or had a parent with ≤high school education. Overall 82% of early literacy promotion participants received books/counseling at well visits <6 months old. Children in the early literacy promotion arm had greater SQRS scores (11.0 vs 9.4, P = .006) but similar PLS-5 scores at 6 months, but there were no differences in SQRS or PLS-5 scores between groups at 24 months.
Implementation of a literacy promotion program early in infancy was associated with richer home reading environments at 6 months but did not improve language development. Although an early literacy program was feasible, additional study may be needed to assess other potential benefits.
Clinicaltrials.gov: NCT02713659.
Abstract Objective To identify parent, child, community, and health care provider characteristics associated with early intervention (EI) referral and multidisciplinary evaluation (MDE) by EI. ...Methods We conducted a mixed methods secondary analysis of data from a randomized controlled trial of a developmental screening program in 4 urban primary care practices. Children <30 months of age not currently enrolled in EI and their parents were included. Using logistic regression, we tested whether parent, child, community, and health care provider characteristics were associated with EI referral and MDE completion. We also conducted qualitative interviews with 9 pediatricians. Interviews were recorded, transcribed, and coded. We identified themes using modified grounded theory. Results Of 2083 participating children, 434 (21%) were identified with a developmental concern. A total of 253 children (58%) with a developmental concern were referred to EI. A total of 129 children (30%) received an MDE. Failure in 2 or more domains on developmental assessments was associated with EI referral (adjusted odds ratio AOR 3.15, 95% confidence interval CI 1.89–5.24) and completed MDE (AOR 2.16, 95% CI 1.19–3.93). Faxed referral to EI, as opposed to just giving families a phone number to call was associated with MDE completion (AOR 2.94, 95% CI 1.48–5.84). Pediatricians reported that office processes, family preference, and whether they thought parents understood the developmental screening tool influenced the EI referral process. Conclusions In an urban setting, one third of children with a developmental concern were not referred to EI, and two thirds of children with a developmental concern were not evaluated by EI. Our results suggest that practice-based strategies that more closely connect the medical home with EI such as electronic transmission of referrals (eg, faxing referrals) may improve completion rates of EI evaluation.
Introduction Despite many recommended strategies for obesity prevention during infancy, effectively delivering recommendations to parents in clinical settings is challenging, especially among ...high-risk populations. This study describes and compares mothers’ and clinicians’ priorities for obesity prevention during infancy, to facilitate more-effective obesity prevention messaging. Methods A discrete choice experiment using maximum difference scaling was administered in 2013 and analyzed in 2013−2014. Twenty-nine low-income, obese mothers of infants and 30 pediatric clinicians from three urban primary care practices rated the relative importance of 16 items relevant to obesity prevention during infancy, in response to this question: Which topic would be most helpful for new mothers to learn about to prevent your their child from becoming overweight? Response options encompassed the domains of feeding, sleep, parenting (including physical activity and screen time), and maternal self-care. Results Mothers (all Medicaid-enrolled and black; mean age, 27 years; mean BMI, 35 kg/m2 ) and clinicians (97% female, 87% pediatricians, 13% nurse practitioners) both highly prioritized recognizing infant satiety and hunger cues, and appropriate feeding volume. Mothers rated infant physical activity and maintaining regular routines as 3.5 times more important than clinicians did ( p <0.001). Clinicians rated breastfeeding as 3.4 times more important than mothers did ( p <0.001). Neither group prioritized learning about screen time or maternal self-care. Conclusions Low-income, obese, black mothers of infants highly prioritized learning about many effective obesity prevention strategies, including recognizing hunger and satiety cues, promoting infant activity, and maintaining regular routines. Clinicians may frame preventive guidance to be responsive to these priorities.
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