Inpatient Screening for Early Identification of Developmental Risk in Infants with Congenital Heart Defects.

OBJECTIVE: To assess the utility of an inpatient standardized developmental screener for early identification of developmental risk in infants with a congenital heart defect (CHD). STUDY DESIGN: This was a retrospective, observational study with convenience sample of postoperative infants with CHD (aged 3-12 months) who underwent neurodevelopmental screening with the Bayley Scales of Infant and Toddler Development Screening Test, Third Edition (Bayley-III Screener) just before discharge. Follow-up testing included outpatient Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) (12-42 mo). RESULTS: The Bayley-III Screener was administered to 325 infants at a median of 5 months, 8 days (IQR 3 months, 28 days, to 7 months, 17 days). Infants scored below age expectations on the Gross Motor (79%), Fine Motor (63%), Receptive Communication (50%), Expressive Communication (38%), and Cognitive (38%) domains. In each domain, children with CHD had greater rates of scores below expectations than the normative sample (each P <.001). The odds of scoring in a greater risk category were increased for infants with genetic syndromes and longer length of hospital stay across all domains. The outpatient Bayley-III (n = 74, 23% follow-up) was completed at a median of 19 months, 9 days (IQR: 17 months, 3 days, to 23 months, 37 days). Individuals falling in greater-risk categories on their initial Bayley-III Screener were significantly more likely to have worse performance on their follow-up outpatient Bayley-III (each domain P < .01). CONCLUSIONS: Inpatient standardized neurodevelopmental screening provides important clinical utility in identifying infants at risk for developmental concern, allows for provision of recommendations for developmental services, and potentially overcomes barriers often noted in returning for outpatient post-discharge assessments.

Sleep Patterns in Young Children with Congenital Heart Disease.

Sleep patterns of 419 toddlers with congenital heart disease were comparable with the normative population except for increased likelihood across the cohort of sleeping in parents' room and increased disrupted sleep in children aged 18-23 months. Disrupted sleep patterns were associated with lower maternal education and increased medical complexity.

Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome.

OBJECTIVE: To characterize neurodevelopmental outcomes after fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome and determine the risk factors for adverse neurodevelopment. STUDY DESIGN: Questionnaires were mailed to families of children who underwent fetal aortic valvuloplasty from 2000 to 2012, and medical records were reviewed retrospectively. The primary outcome was the General Adaptive Composite score of the Adaptive Behavior Assessment System Questionnaire-Second Edition. Other questionnaires included the Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, and Pediatric Quality of Life Inventory. RESULTS: Among 69 eligible subjects, 52 (75%) completed questionnaires at median age of 5.5 (range 1.3-12) years; 30 (58%) had biventricular status circulation. The General Adaptive Composite mean score (92 ± 17) was lower than population norms (P < .001) and similar to published reports in patients with hypoplastic left heart syndrome without fetal intervention; scores in the single ventricular versus biventricular group were 97 ± 19 vs 89 ± 14, respectively (P = .10). On multivariable analysis, independent predictors of a lower General Adaptive Composite score were total hospital duration of stay in the first year of life (P = .001) and, when forced into the model, biventricular status (P = .02). For all other neurodevelopmental questionnaires (Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, Pediatric Quality of Life Inventory), most subscale scores for patients with biventricular and single ventricular status were similar. CONCLUSION: Children who underwent fetal aortic valvuloplasty have neurodevelopmental delay, similar to patients with hypoplastic left heart syndrome without fetal intervention. Achievement of biventricular circulation was not associated with better outcomes. We infer that innate patient factors and morbidity during infancy have the greatest effect on neurodevelopmental outcomes.

Impact of Tight Glycemic Control on Neurodevelopmental Outcomes at 1 Year of Age for Children with Congenital Heart Disease: A Randomized Controlled Trial.

OBJECTIVE: To assess the association of postoperative tight glycemic control and hypoglycemia in children undergoing cardiac surgery with neurodevelopmental outcomes at 1 year of age. STUDY DESIGN: A 2-center, prospective, randomized trial of postoperative tight glycemic control vs standard care was conducted in 980 children undergoing cardiac surgery. Neurodevelopmental outcomes were assessed at nine to 18 months using the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III), the Adaptive Behavior Assessment System, Second Edition, the Ages and Stages Questionnaire, Third Edition, and the Brief Infant Toddler Social-Emotional Assessment. RESULTS: Neurodevelopmental follow-up was performed on 237 patients with a mean age of 13 months. No significant treatment group differences were found in the Bayley-III and Adaptive Behavior Assessment System, Second Edition composite scores or percentage at risk based on the Ages and Stages Questionnaire, Third Edition and the Brief Infant Toddler Social-Emotional Assessment. Patients who experienced moderate to severe hypoglycemia (n = 8) had lower Bayley-III composite scores compared with patients with no to mild hypoglycemia, even after controlling for factors known to be associated with poorer neurodevelopmental outcomes. CONCLUSION: For infants undergoing cardiac surgery, tight glycemic control did not impact neurodevelopmental outcomes compared with standard care. These data suggest a possible association between moderate to severe hypoglycemia and poorer neurodevelopmental outcomes at 1 year of age. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00443599.