Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Randomized Controlled Trial
. 2014 Sep;165(3):490-496.e8.
doi: 10.1016/j.jpeds.2014.05.019. Epub 2014 Jun 19.

Factors associated with neurodevelopment for children with single ventricle lesions

Collaborators, Affiliations
Randomized Controlled Trial

Factors associated with neurodevelopment for children with single ventricle lesions

Caren S Goldberg et al. J Pediatr. 2014 Sep.

Abstract

Objective: To measure neurodevelopment at 3 years of age in children with single right-ventricle anomalies and to assess its relationship to Norwood shunt type, neurodevelopment at 14 months of age, and patient and medical factors.

Study design: All subjects in the Single Ventricle Reconstruction Trial who were alive without cardiac transplant were eligible for inclusion. The Ages and Stages Questionnaire (ASQ, n = 203) and other measures of behavior and quality of life were completed at age 3 years. Medical history, including measures of growth, feeding, and complications, was assessed through annual review of the records and phone interviews. The Bayley Scales of Infant Development, Second Edition (BSID-II) scores from age 14 months were also evaluated as predictors.

Results: Scores on each ASQ domain were significantly lower than normal (P < .001). ASQ domain scores at 3 years of age varied nonlinearly with 14-month BSID-II. More complications, abnormal growth, and evidence of feeding, vision, or hearing problems were independently associated with lower ASQ scores, although models explained <30% of variation. Type of shunt was not associated with any ASQ domain score or with behavior or quality-of-life measures.

Conclusion: Children with single right-ventricle anomalies have impaired neurodevelopment at 3 years of age. Lower ASQ scores are associated with medical morbidity, and lower BSID-II scores but not with shunt type. Because only a modest percentage of variation in 3-year neurodevelopmental outcome could be predicted from early measures, however, all children with single right-ventricle anomalies should be followed longitudinally to improve recognition of delays.

Trial registration: ClinicalTrials.gov NCT00115934.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Single Ventricle Reconstruction Extension Trial Neurodevelopmental Assessment at 3 Years
Figure 2
Figure 2. Relationship of Ages and Stages Domain Scores at 3 Years to 14-Month Bayley Scales of Infant Development-II
Three-year ASQ domains versus A, 1-month Psychomotor Development Index (PDI) and B, 14-month Mental Development Index (MDI). The solid line represents the locally weighted scatterplot smoothing curve. The dashed line denotes the fitted piecewise linear regression model, using a cut-point of A, PDI=70 and B, MDI=85. The R2 assesses the percentage of variability in the ASQ domain explained by PDI score modeled with a piecewise linear regression fit.
Figure 2
Figure 2. Relationship of Ages and Stages Domain Scores at 3 Years to 14-Month Bayley Scales of Infant Development-II
Three-year ASQ domains versus A, 1-month Psychomotor Development Index (PDI) and B, 14-month Mental Development Index (MDI). The solid line represents the locally weighted scatterplot smoothing curve. The dashed line denotes the fitted piecewise linear regression model, using a cut-point of A, PDI=70 and B, MDI=85. The R2 assesses the percentage of variability in the ASQ domain explained by PDI score modeled with a piecewise linear regression fit.

References

    1. Tabbutt S, Nord AS, Jarvik GP, Bernbaum J, Wernovsky G, Gerdes M, et al. Neurodevelopmental outcomes after staged palliation for hypoplastic left heart syndrome. Pediatrics. 2008;121:476–483. - PubMed
    1. Mahle WT, Clancy RR, Moss EM, Gerdes M, Jobes DR, Wernovsky G. Neurodevelopmental outcome and lifestyle assessment in school aged and adolescent children with hypoplastic left heart syndrome. Pediatrics. 2000;105:1082–89. - PubMed
    1. Goldberg CS, Schwartz EM, Brunberg JA, Mosca RS, Bove EL, Schork MA, et al. Neurodevelopmental outcome of patients after the Fontan operation: a comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions. J Pediatr. 2000;137:646–52. - PubMed
    1. Fuller S, Nord AS, Gerdes M, Wernovsky G, Jarvik GP, Bernbaum J, et al. Predictors of impaired neurodevelopmental outcomes at one year of age after infant cardiac surgery. Eur J Cardiothorac Surg. 2009;36(1):40–48. - PubMed
    1. Newburger JW, Sleeper LA, Bellinger DC, Goldberg CS, Tabbutt S, Lu M, et al. Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: The Single Ventricle Reconstruction Trial. Circulation. 2012;125:2081–91. - PMC - PubMed

Publication types

Associated data