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Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome
J H Kern1, V J Hinton, N E Nereo
1Columbia-Presbyterian Medical Center, Department of Pediatrics, New York, NY, USA.
Insights
Children with hypoplastic left heart syndrome (HLHS) often show low-normal intellectual and adaptive functioning. Longer circulatory arrest times during the Norwood procedure correlate with lower IQ scores in these patients.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Outcomes
- Congenital Heart Disease
Background:
- Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect requiring staged surgical palliation.
- The Norwood procedure is a critical initial surgical intervention for HLHS.
- Long-term neurodevelopmental outcomes in HLHS survivors are a significant clinical concern.
Purpose of the Study:
- To evaluate the intellectual and adaptive behavior of children with HLHS post-Norwood procedure.
- To compare neurodevelopmental outcomes in HLHS patients with family controls.
- To identify potential correlations between perioperative variables and cognitive function in HLHS.
Main Methods:
- A cohort of 14 children with HLHS (age >3 years) who completed at least two surgical stages of the Norwood procedure were assessed.
- Intelligence quotient (IQ) testing and parent-reported adaptive behavior assessments were conducted.
- Outcomes were compared to 10 family controls and analyzed for correlations with perioperative factors.
Main Results:
- HLHS patients demonstrated median full-scale IQ scores of 88 and adaptive behavior scores of 91 (normal range: 100 ± 15).
- One child met criteria for intellectual disability; statistically significant differences were noted in adaptive behavior compared to controls.
- A negative correlation was observed between the duration of circulatory arrest during Stage I surgery and full-scale IQ.
Conclusions:
- Children with HLHS typically function within the low-normal range for intelligence and adaptive behaviors.
- Prolonged circulatory arrest time during surgical palliation may negatively impact intellectual development in HLHS patients.
- These findings highlight the importance of monitoring neurodevelopmental trajectories in HLHS survivors and optimizing surgical techniques to minimize neurological risk.
Objective:
To assess intellect and adaptive behavior in children with hypoplastic left heart syndrome (HLHS) who had undergone at least two surgical stages of the Norwood procedure.
Methods:
Fourteen children with HLHS >3 years of age participated in the study. The patients underwent intelligence quotient (IQ) testing, and their parents were interviewed regarding their children's adaptive behavior. Results were compared with those of 10 family controls. Outcomes were studied for possible correlation with perioperative variables.
Results:
Among the HLHS patients, the median scores for full scale IQ and adaptive behavior were 88 and 91, respectively (normal = 100 +/- 15). One child met criteria for mental retardation. Family controls scored generally higher than did HLHS patients, but only differences in adaptive behavior were statistically significant. A negative correlation was found between stage I circulatory arrest time and full scale IQ.
Conclusions:
Children with HLHS most often function in the low-normal range of intelligence and adaptive behavior. A prolonged circulatory arrest time may result in decreased intellectual function.