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Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome
William T Mahle1, Karen J Visconti, M Catherin Freier
1Children's Healthcare of Atlanta, Atlanta, GA 30322-1062, USA. mahlew@kidsheart.com
Insights
Children with hypoplastic left heart syndrome (HLHS) show neurodevelopmental deficits regardless of surgical strategy. Prolonged hospital stays after surgery are linked to poorer neurodevelopmental outcomes in school-aged children.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Congenital Heart Disease Surgery
Background:
- Hypoplastic left heart syndrome (HLHS) requires complex surgical management in infants.
- Current surgical strategies include primary heart transplantation and the Norwood procedure.
- Understanding long-term neurodevelopmental outcomes is crucial for patient care.
Purpose of the Study:
- To compare neurodevelopmental outcomes at school age between infants with HLHS treated with heart transplantation versus the Norwood procedure.
- To identify factors associated with neurodevelopmental outcomes in this population.
Main Methods:
- A multicenter, cross-sectional study involving 47 school-aged children (mean age 12.4 years) with HLHS.
- Participants underwent comprehensive neuropsychological testing, including IQ, language, academic achievement, and visual-motor integration assessments.
- An intention-to-treat analysis compared outcomes based on initial surgical strategy (transplantation vs. Norwood procedure).
Main Results:
- Overall neurocognitive test results were significantly below population norms (mean full-scale IQ: 86 ± 14).
- No significant association was found between surgical strategy (transplantation vs. Norwood) and neurodevelopmental outcomes.
- Longer hospital stay post-surgery was significantly associated with lower IQ scores (verbal, performance, full-scale).
- Aortic valve atresia correlated with lower math achievement scores.
Conclusions:
- Neurodevelopmental deficits are common in school-aged children with HLHS, irrespective of the surgical approach.
- Complications leading to prolonged hospitalization are associated with poorer neurodevelopmental status.
- These findings highlight the importance of minimizing perioperative complications to optimize long-term neurodevelopmental outcomes.
Objective:
Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure. We sought to determine how these 2 surgical approaches influence neurodevelopmental outcomes at school age.
Methods:
A multicenter, cross-sectional study of neurodevelopmental outcomes among school-aged children (>8 years of age) with HLHS was undertaken between July 2003 and September 2004. Four centers enrolled 48 subjects, of whom 47 completed neuropsychologic testing. Twenty-six subjects (55%) had undergone the Norwood procedure and 21 (45%) had undergone transplantation, with an intention-to-treat analysis. The mean age at testing was 12.4 +/- 2.5 years. Evaluations included the Wechsler Abbreviated Scale of Intelligence, Clinical Evaluation of Language Fundamentals, Wechsler Individual Achievement Test, and Beery-Buktenica Developmental Test of Visual-Motor Integration.
Results:
The mean neurocognitive test results were significantly below population normative values. The mean full-scale IQ for the entire cohort was 86 +/- 14. In a multivariate model, there was no association of surgical strategy with any measure of developmental outcome. A longer hospital stay, however, was associated significantly with lower verbal, performance, and full-scale IQ scores. Aortic valve atresia was associated with lower math achievement test scores.
Conclusions:
Neurodevelopmental deficits are prevalent among school-aged children with HLHS, regardless of surgical approach. Complications that result in prolonged hospitalization at the time of the initial operation are associated with neurodevelopmental status at school age.
