Published online December 15, 2005
PEDIATRICS Vol. 117 No. 1 January 2006, pp. e90-e97 (doi:10.1542/peds.2005-0575)
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Relationship of Surgical Approach to Neurodevelopmental Outcomes in Hypoplastic Left Heart Syndrome

William T. Mahle, MDa, Karen J. Visconti, PhDb, M. Catherin Freier, PhDc, Stephen M. Kanne, PhDd, William G. Hamilton, PhDa, Angela M. Sharkey, MDd, Richard E. Chinnock, MDc, Kathy J. Jenkins, MDb, Peter K. Isquith, PhDe, Thomas G. Burns, PsyDa and Pamela C. Jenkins, MD, PhDe

a Children's Healthcare of Atlanta, Atlanta, Georgia
b Children's Hospital, Boston, Massachusetts
c Loma Linda University Children's Hospital, Loma Linda, California
d St Louis Children's Hospital, St Louis, Missouri
e Dartmouth Medical School, Hanover, New Hampshire

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.

Key Words: hypoplastic left heart syndrome • neurodevelopmental outcome • transplantation • Norwood procedure

Abbreviations: BASC—Behavior Assessment System for Children • BRIEF—Behavior Rating Inventory of Executive Function • CELF—Clinical Evaluation of Language Fundamentals • DHCA—deep hypothermic circulatory arrest • HLHS—hypoplastic left heart syndrome • VMI—Visual-Motor Integration • WASI—Wechsler Abbreviated Scale of Intelligence • WIAT—Wechsler Individual Achievement Test

Accepted Jun 30, 2005.




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