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Updated: Nov 21, 2025
Assessment and Evaluation of the High Risk Neonate: The NICU Network Neurobehavioral Scale
Published on: August 25, 2014
Six-Year Neurodevelopmental Outcomes for Children With Single-Ventricle Physiology
Renee Sananes1, Caren S Goldberg2, Jane W Newburger3
1Department of Pediatrics and Labatt Family Heart Centre, Hospital for Sick Children, University of Toronto, Toronto, Canada; renee.sananes@sickkids.ca.
Insights
Early developmental scores do not reliably predict adaptive skills in children with single-ventricle physiology at age 6. Serial evaluations are crucial for identifying neurodevelopmental deficits in these children.
Area of Science:
- Pediatric Cardiology
- Developmental Pediatrics
- Neurodevelopmental Disorders
Background:
- Children with single-ventricle physiology often experience neurodevelopmental deficits.
- Early identification of these deficits is critical for timely intervention and improved outcomes.
Purpose of the Study:
- To assess if neurodevelopmental deficits in single-ventricle physiology change with age.
- To determine if early developmental scores predict 6-year outcomes in this population.
Main Methods:
- Utilized data from the Single Ventricle Reconstruction Trial.
- Administered Bayley Scales of Infant Development at 14 months.
- Conducted annual Behavior Assessment System for Children, Second Edition (BASC-2) from ages 2 to 6 years.
Main Results:
- A significant proportion of participants scored at risk or impaired on the BASC-2 Adaptive Skills Index at age 6.
- 14-month Psychomotor Development Index showed 79% sensitivity for predicting 6-year adaptive skill deficits.
- Positive predictive values for early scores were low, indicating limited predictive accuracy for later adaptive skills.
Conclusions:
- Many children with hypoplastic left heart syndrome and low adaptive skills at age 6 are missed by early screening.
- Emphasizes the necessity of ongoing, serial developmental evaluations for children with critical congenital heart disease throughout their development.
Objectives:
To determine if neurodevelopmental deficits in children with single-ventricle physiology change with age and early developmental scores predict 6-year outcomes.
Methods:
In the Single Ventricle Reconstruction Trial, Bayley Scales of Infant Development, Second Edition, were administered at 14 months of age, and parents completed the Behavior Assessment System for Children, Second Edition (BASC-2) annually from the ages of 2 to 6 years. Scores were classified as average, at risk, or impaired. We calculated sensitivities, specificities, and positive and negative predictive values of earlier tests on 6-year outcomes.
Results:
Of 291 eligible participants, 244 (84%) completed the BASC-2 at 6 years; more Single Ventricle Reconstruction participants than expected on the basis of normative data scored at risk or impaired on the BASC-2 Adaptive Skills Index at that evaluation (28.7% vs 15.9%; P < .001). Children with Adaptive Skills Composite scores <2 SD below the mean at the age of 6 were more likely to have had delayed development at 14 months, particularly on the Psychomotor Development Index (sensitivity of 79%). However, the positive predictive value of the 14-month Mental Development Index and Psychomotor Development Index for 6-year BASC-2 Adaptive Scores was low (44% and 36%, respectively). Adaptive Skills Composite score impairments at the age of 6 were poorly predicted by using earlier BASC-2 assessments, with low sensitivities at the ages of 3 (37%), 4 (48%), and 5 years (55%).
Conclusions:
Many children with hypoplastic left heart syndrome who have low adaptive skills at the age of 6 years will not be identified by screening at earlier ages. With our findings, we highlight the importance of serial evaluations for children with critical congenital heart disease throughout development.
