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Early Neurodevelopmental Outcomes in Children with Hypoplastic Left Heart Syndrome and Related Anomalies After Hybrid
1The Heart Center, Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH, 43205, USA. omar.khalid@nationwidechildrens.org.
Insights
The hybrid strategy for single ventricle palliation shows significant neurodevelopmental impairment in 46% of infants by 12 months, primarily affecting motor skills. No differences were observed between hypoplastic left heart syndrome and non-HLHS groups.
Area of Science:
- Pediatric Cardiology
- Neonatal Surgery
- Developmental Neuroscience
Background:
- Single ventricle heart defects require complex surgical palliation.
- The hybrid strategy is an alternative to the Norwood procedure for single ventricle palliation.
- Neurodevelopmental outcomes following neonatal cardiac surgery are a significant concern.
Purpose of the Study:
- To report 12-month neurodevelopmental outcomes in infants undergoing neonatal hybrid palliation for single ventricle.
- To identify clinical factors associated with neurodevelopmental outcomes in this cohort.
- To compare neurodevelopment between infants with hypoplastic left heart syndrome (HLHS) and non-HLHS single ventricle.
Main Methods:
- Retrospective study of infants with single ventricle undergoing neonatal hybrid procedure.
- Two cohorts: HLHS and non-HLHS single ventricle.
- Neurodevelopment assessed at 12 months using Bayley Scales of Infant and Toddler Development, Third Edition (Bayley III).
- Statistical analysis included parametric and non-parametric tests.
Main Results:
- Abnormal neurodevelopment was identified in 11 of 24 infants (46%), predominantly motor impairment (46%).
- No significant differences in neurodevelopment were found between HLHS and non-HLHS groups.
- Higher lactate levels correlated with lower cognitive scores.
- Fewer mechanical ventilation days, shorter ICU and hospital stays, and fewer complications were associated with better neurodevelopmental scores.
- Higher weight at 12 months was associated with better cognitive and language scores.
Conclusions:
- Significant neurodevelopmental impairment is present in a substantial proportion of infants treated with neonatal hybrid palliation for single ventricle.
- The hybrid approach did not show differential neurodevelopmental outcomes between HLHS and non-HLHS infants.
- Further multicenter trials are warranted to compare neurodevelopmental outcomes between the hybrid and Norwood approaches.
Abstract:
The hybrid strategy is one approach to single ventricle palliation. In this study, we reported neurodevelopment at 12 months for two cohorts of children managed with the hybrid and clinical factors associated with neurodevelopment in the entire sample. We performed a retrospective study of children with single ventricle who had undergone a neonatal hybrid procedure. One group included infants with hypoplastic left heart syndrome (HLHS); another group included infants with non-HLHS single ventricle. Neurodevelopment was assessed with 12-month Bayley III. Parametric and non-parametric statistics were used for analysis. Nine infants with HLHS and 15 with non-HLHS were identified. Abnormal neurodevelopment was identified in 11 of 24 (46%), primarily motor (46%). Development did not differ between groups. In the whole sample, higher lactate levels were associated with lower cognitive scores (p = 0.04). Fewer mechanical ventilation days were associated with higher cognitive scores (p = 0.05) after Stage 1 and higher motor scores after Stage 2. Shorter ICU length of stay (p = 0.01), shorter hospital length of stay (p = 0.01), and fewer complications (p = 0.01) after stage 2 were associated with higher motor scores. Higher cognitive (p = 0.02) and language (p = 0.002) scores were associated with higher weight at 12 months. In the largest cohort of single ventricle children treated with neonatal hybrid palliation yet reported, significant neurodevelopmental impairment was identified. No differences in neurodevelopment were found between children with HLHS and those with non-HLHS variants. A multicenter trial is needed to test differences in neurodevelopment between hybrid and Norwood approaches.
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