In this issue of Pediatrics, Sananes et al1  leverage the Pediatric Heart Network Single Ventricle Reconstruction Trial to describe developmental and behavioral concerns in a cohort of children with hypoplastic left heart syndrome and related single right ventricle conditions. The Pediatric Heart Network recognized the importance of conducting developmental screening as recommended by the American Academy of Pediatrics (AAP)2,3  in this population at high risk for neurodevelopmental concerns.

The researchers evaluate how well a diagnostic developmental evaluation at 14 months of age, with the Bayley Scales of Infant Development-II,4  and developmental screening at 3, 4, and 5 years of age, with the Ages & Stages Questionnaire (ASQ),5  predicted the outcome of “developmental functioning” as measured by the Adaptive Score on the Behavior Assessment System for Children-2 (BASC-2)6  at 6 years of age.

There were some correlations between early developmental evaluations and later outcomes at age 6 years, such as a “failure” (scoring 2 SDs below the mean) on the Psychomotor Developmental Index of the Bayley Scales of Infant Development-II at age 14 months having a relatively good sensitivity (0.79) to identify lower adaptive skills at 6 years of age. In general, however, researchers in this study found that scores on the Bayley Scales of Infant Development-II at 14 months had a low positive predictive value for the BASC-2 Adaptive Scores at age 6 years. Some domains of the ASQ, including the personal-social, problem-solving, and communication scales, were correlated with 6-year-old BASC-2 Adaptive Scores, with “fail” ratings for problem-solving and personal-social domains at age 4 to 5 years having PPVs ranging from 75 to 93. Additionally, rates of parent-reported behavior problems on the BASC-2 were significantly higher at age 6 than at younger ages.

It is helpful to understand specific domains and tasks that assessment tools measure. The BASC-2 is a broad-based behavioral rating scale and measures different constructs than the Bayley Scales of Infant Development-II and ASQ and differs from other measures of adaptive functioning.

A signature developmental profile described in children with congenital heart disease (CHD) involves a “low severity, high prevalence” pattern of deficits.7  Thus, children may have mild deficits across numerous domains of development and behavior that subsequently culminate in impairments in day-to-day functioning. More subtle challenges in attention regulation and executive functioning are common in children with CHD and may not present functional impact until school-age. These concepts underscore the importance of in depth, multidisciplinary diagnostic evaluations at critical time points in development as recommended in the AAP-approved American Heart Association scientific statement on neurodevelopmental evaluation and treatment7  and guidelines for incorporating formal neurodevelopmental follow-up programs into cardiac care.8  Targeted neurodevelopmental evaluations should be conducted in addition to ongoing AAP-recommended developmental screening and surveillance for all children.2,3 

In this study, it was reassuring that more than three-quarters of children who passed the ASQ screening at younger ages subsequently scored within the average range on BASC-2 at age 6 years. Authors of this study raise important issues about developmental screening, surveillance, and evaluation in children with CHD as well as other high-risk pediatric populations, including those with conditions and risk factors associated with neurologic injury and developmental delay or disability). These issues include the following:

  • 1) The challenge of measuring developmental outcomes: which developmental domains to measure, with which tools, and at what ages can be challenging questions when development is an evolving process and mild deficits may not become apparent until the ability to integrate more complex, abstract tasks is expected.

  • 2) The advantages and disadvantages of developmental screening as compared with diagnostic assessment due to the variable predictive values of screening tools within high-risk populations.

  • 3) The potential to modify developmental trajectories when early identification of and intervention for deficits is achieved through robust recognition of developmental patterns.

  • 4) The importance of advocating for early intervention and educational support services for children with high-risk conditions who may not meet “traditional” definitions for eligibility based on standardized score cutoffs, when deficits may be mild but multiple.

The work of Sananes et al1  has implications for pediatric and surgical specialties. Implementing developmental screening within populations known to be at high risk can serve as a touchpoint to discuss developmental implications of chronic diseases as well as offer hope and support. When children have been diagnosed with developmental delays or are known to be at risk for delays, a proactive developmental support approach is important. A blended approach including developmental surveillance, screening, and diagnostic evaluations is logical. Unlike with many medical conditions, a named diagnosis is not required for a child to access and benefit from developmental interventions such as physical, occupational, and speech-language therapy and Part C Early Intervention programming.

Opinions expressed in these commentaries are those of the author and not necessarily those of the American Academy of Pediatrics or its Committees

FUNDING: No external funding.

COMPANION PAPER: A companion to this article can be found online at www.pediatrics.org/cgi/doi/10.1542/2020-014589.

AAP

American Academy of Pediatrics

ASQ

Ages & Stages Questionnaire

BASC-2

Behavior Assessment System for Children-2

CHD

congenital heart disease

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Competing Interests

POTENTIAL CONFLICT OF INTEREST: Dr Anixt receives funding as part of the National Pediatric Cardiology Quality Improvement Collaborative as consultant for the neurodevelopmental learning laboratory; Dr Wiley has indicated she has no potential conflicts of interest to disclose.

FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.