Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial.
PMCID: PMC3341507 DOI: 10.1161/CIRCULATIONAHA.111.064113
BACKGROUND: Survivors of the Norwood procedure may experience neurodevelopmental impairment. Clinical trials to improve outcomes have focused primarily on methods of vital organ support during cardiopulmonary bypass.
METHODS AND RESULTS: In the Single Ventricle Reconstruction trial of the Norwood procedure with modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery shunt, 14-month neurodevelopmental outcome was assessed by use of the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. We used multivariable regression to identify risk factors for adverse outcome. Among 373 transplant-free survivors, 321 (86%) returned at age 14.3 ± 1.1 (mean ± SD) months. Mean PDI (74 ± 19) and MDI (89 ± 18) scores were lower than normative means (each P
CONCLUSIONS: Neurodevelopmental impairment in Norwood survivors is more highly associated with innate patient factors and overall morbidity in the first year than with intraoperative management strategies. Improved outcomes are likely to require interventions that occur outside the operating room.
CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.
Abnormalities, Multiple; Blalock-Taussig Procedure; Brain Damage, Chronic; Child; Child, Preschool; Developmental Disabilities; Early Intervention (Education); Female; Heart Ventricles; Humans; Hypoplastic Left Heart Syndrome; Hypoxia, Brain; Infant; Infant, Newborn; Male; Neuropsychological Tests; Norwood Procedures; Postoperative Complications; Prospective Studies; Psychomotor Disorders; Pulmonary Artery; Risk Factors; Socioeconomic Factors; Vascular Grafting
Newburger, J. W., Sleeper, L. A., Bellinger, D. C., Goldberg, C. S., Tabbutt, S., Lu, M., Mussatto, K. A., Williams, I. A., Gustafson, K. E., Mital, S., Pike, N., Sood, E., Mahle, W. T., Cooper, D. S., Dunbar-Masterson, C., Krawczeski, C. D., Lewis, A., Menon, S. C., Pemberton, V. L., Ravishankar, C., Atz, T. W., Ohye, R. G., Gaynor, J. W., ., Shirali, G. S. Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial. Circulation 125, 2081-2091 (2012).
Cardiology Commons, Cardiovascular System Commons, Congenital, Hereditary, and Neonatal Diseases and Abnormalities Commons, Pediatrics Commons