Neurodevelopmental Deficits in Children With Single-Ventricle Physiology Change With Age

By Megan Brooks

January 15, 2021

NEW YORK (Reuters Health) - Neurodevelopmental deficits in children with single-ventricle physiology change as children age, and early developmental scores are not a good predictor of functioning when they reach school age, new research shows.

The findings from the Single Ventricle Reconstruction (SVR) Extension Study (SVR-II) highlight the importance of serial evaluations and routine follow-up for children with congenital heart disease, researchers say.

Neurodevelopmental and behavioral deficits are the most common long-term problems faced by children with hypoplastic left heart syndrome (HLHS) and other complex congenital heart defects.

In the Pediatric Heart Network Single Ventricle Reconstruction (SVR) Trial, newborns with HLHS and other related single right ventricular defects were prospectively enrolled and randomly assigned to a Norwood procedure by using either a modified Blalock-Taussig shunt or a right ventricle-to-pulmonary artery shunt.

As part of the trial, parents completed the Bayley Scales of Infant Development, Second Edition, (BSID-II) at 14 months of age and the Behavior Assessment System for Children, Second Edition (BASC-2) annually up to age six years.

Of 291 eligible children from the original cohort, 244 (84%) completed the BASC-2 at six years.

With the SVR-II extension study, "we wanted to see whether or not we can sort of plot a trajectory from the very earliest assessments at about 14 months of age up until six years of age. What we found was that early assessments really don't have strong predictive value," Dr. Renee Sananes of the Department of Pediatrics and Labatt Family Heart Center, Hospital for Sick Children, University of Toronto, in Canada, told Reuters Health by email.

She emphasized, however, that early assessments, at around 14 months of age, are valuable. "They tell us how a child is doing at that time. And they also tell us if that child needs early intervention, which is really important, because we know that when early intervention is accessed and used properly, it does make a difference," Dr. Sananes said.

In SVR-II, by parent report of behavior symptoms, children were rated as having fewer externalizing behavior problems at age 3 and 4 years, relative to population norms, but more externalizing behavior challenges at age six years.

"This is consistent with findings reported by others revealing increased externalizing difficulties in children with age and in association with the experience of learning challenges at school," Dr. Sananes and colleagues note in Pediatrics.

In terms of adaptive functioning, parent ratings revealed a significant decrease in these skills with age, such that by six years of age significantly more children were rated as falling within the at-risk or impaired range in adaptive functioning compared to population norms.

This is not surprising, Dr. Sananes told Reuters Health, and may reflect the fact that parents have an increased opportunity for social comparison with peers as their children enter school.

"With our findings, we further highlight the importance of considering how challenges may emerge and change with development and the expectations of different environments at different ages, therefore emphasizing the importance of serial evaluations and routine follow-up for children with congenital heart disease throughout the life span," the researchers write.

It's also important to note that a significant proportion of children (77% to 85%) who do not show early impairments remain unimpaired at the age of six years. "This finding should provide some important reassurance for parents as they are counseled to continue to promote their child's development," they add.

Evaluation of the SVR cohort (SVR-III trial) of children at 10 to 12 years of age is currently underway and will examine the relationship between findings at six years of age to later school-aged outcomes.

SOURCE: Pediatrics, online January 13, 2021.