Six-Year Neurodevelopmental Outcomes for Children With Single-Ventricle Physiology

Six-Year Neurodevelopmental Outcomes for Children With Single-Ventricle Physiology.

Sananes R, Goldberg CS, Newburger JW, Hu C, Trachtenberg F, Gaynor JW, Mahle WT, Miller T, Uzark K, Mussatto KA, Pizarro C, Jacobs JP, Cnota J, Atz AM, Lai WW, Burns KM, Milazzo A, Votava-Smith J, Brosig CL; PHN investigators.

Pediatrics. 2021 Feb;147(2):e2020014589. doi: 10.1542/peds.2020-014589. Epub 2021 Jan 13.

PMID: 33441486


Take Home Points:

  • There is an increase in externalizing behavioral problems and decreased adaptive skills in school age children with HLHS.
  • Many children with HLHS who have low adaptive skills at 6 years of age will not be identified by screening at earlier ages.
  • Serial neurodevelopmental evaluations will be necessary to diagnose deficits as these single ventricle patients get older and advance in school.

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Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: Neurodevelopmental deficits are one of the most common long term issues that face children with HLHS. Early identification could theoretically allow for implementation of therapies that could improve long-term educational and behavioral outcomes. This extension study to the Single Ventricle Reconstruction (SVR) Trial studied the original cohort who had a 14-month mental development index (MDI) and psychomotor development index (PDI) via annual developmental (ASQ) and behavioral (externalizing and internalizing)/adaptive assessments (BASC-2) based on parental questionnaires from ages 3-6. The ASQ measures the child’s development in communication, gross motor, fine motor, problem solving and personal social skills, and the BASC-2 measures adaptive skills and problem behaviors in the community and home settings. Adaptive functioning refers to how a child copes with demands of everyday life and their personal independence. Scores were compared to population norms and classified as at least average (< 1 SD), at risk (< 1-2 SC) and impaired (< 2 SD). 249 patients completed the assessments.


The greatest change in proportion of children at being at risk or impaired occurred between ages 3 and 4, but this was largely due to a lower rate of reported problem behaviors at age 3 than the population norms. However, by age 6, many more had externalizing behavior challenges (e.g. hyperactivity, aggression, and rule breaking). There was not an increase in internalizing behaviors or differences compared to population norms. The most significant deviations were noted on the adaptive skills portion of BASC-2, with most differences occurring between ages 5 and 6. At age 3, 87% were age-appropriate, but by age 6, this dropped to only 71%. 22% (vs. 14% expected) were classified as at risk and 7% (vs. 2%) as impaired. See figure 1 and table 2. The authors note that this finding may not be a regression, but rather the parents now having increased opportunities for social comparison now that the children are in school and begin to act more independently. Parent ratings of deficits in problem solving skills and communication were strongly related to future poor adaptive skills, with ~44% of children at risk/impaired on the 14 month MDI and ~ 36% of children at risk/impaired on the 14 month PDI having impaired adaptive skills at age 6. As a “half glass full” observation, the authors highlight that a significant proportion (77-85%) who do not show early impairments remain unimpaired at age 6.


Limitations of the study were that only parental assessments were made, a selection bias was possibly present given that participants had fewer risk factors for impaired neurodevelopment and higher socioeconomic status than non-participants, and the inability to measure impact of access to early intervention services on outcome scores. A follow up study at 10-12 years is already underway to allow for further understanding of a relationship with later school age outcomes and standardized testing.