Congenital heart disease and neurodevelopmental evaluation: National guidelines vs. single center utilization

Background: Children with congenital heart defects have a higher risk of acquiring developmental disabilities. Routine developmental evaluations throughout childhood increase the chances of identifying these deficits, prompting referral to Early Intervention services. Objectives: We hypothesized that referral rates to Early Intervention and/or neurodevelopmental evaluation were low despite national guidelines. We also sought to investigate the relationship between type of congenital heart disease and results of neurodevelopmental evaluation. Methods: A retrospective chart review was performed for congenital heart disease patients requiring cardiac surgery within their first year of life. Variables assessed included type of congenital heart disease, prenatal diagnosis, number of surgeries in the first year of life, and neurodevelopmental referral, evaluation, and quantitative assessment. Results: 151 patients met inclusion criteria from 1/1/13 through 12/31/18. 64 (42%) were referred to neurodevelopmental services, of which 60 patients accessed services. Single ventricle patients had an increased rate of neurodevelopmental referral (88% vs 31, p < 0.05) for Early Intervention. Hypoplastic right heart syndrome patients scored lowest on the Bayley cognitive subscale with a median score of 7 (p < 0.01) as compared to norms for the general pediatric population. Hypoplastic left heart syndrome and hypoplastic right heart syndrome patients scored lowest on gross motor skills, at medians of 3 and 5 respectively (p < 0.01). Conclusions: Only 42% of congenital heart disease patients were referred to neurodevelopmental services, despite known risks. Most congenital heart disease patients had low to average neurodevelopment scores. Excluding single ventricle patients, there was no difference between congenital heart disease type and neurodevelopmental outcomes. Single ventricle patients scored particularly low in cognition and gross motor. Ultimately, 67% of patients who underwent initial evaluation received additional Early Intervention services, demonstrating the significant importance of initial neurodevelopmental evaluation.