Faculty Collaboration Database

Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial. Circulation 2012 May 01;125(17):2081-91

Date

03/30/2012

Pubmed ID

22456475

Pubmed Central ID

PMC3341507

DOI

10.1161/CIRCULATIONAHA.111.064113

Scopus ID

2-s2.0-84860337259 (requires institutional sign-in at Scopus site)   282 Citations

Abstract

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<0.001). Neither PDI nor MDI score was associated with type of Norwood shunt. Independent predictors of lower PDI score (R(2)=26%) were clinical center (P=0.003), birth weight <2.5 kg (P=0.023), longer Norwood hospitalization (P<0.001), and more complications between Norwood procedure discharge and age 12 months (P<0.001). Independent risk factors for lower MDI score (R(2)=34%) included center (P<0.001), birth weight <2.5 kg (P=0.04), genetic syndrome/anomalies (P=0.04), lower maternal education (P=0.04), longer mechanical ventilation after the Norwood procedure (P<0.001), and more complications after Norwood discharge to age 12 months (P<0.001). We found no significant relationship of PDI or MDI score to perfusion type, other aspects of vital organ support (eg, hematocrit, pH strategy), or cardiac anatomy.

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.

Author List

Newburger JW, Sleeper LA, Bellinger DC, Goldberg CS, Tabbutt S, Lu M, Mussatto KA, Williams IA, Gustafson KE, Mital S, Pike N, Sood E, Mahle WT, Cooper DS, Dunbar-Masterson C, Krawczeski CD, Lewis A, Menon SC, Pemberton VL, Ravishankar C, Atz TW, Ohye RG, Gaynor JW, Pediatric Heart Network Investigators

Author

Kathleen Mussatto Ph.D. Associate Professor in the School of Nursing department at Milwaukee School of Engineering

MESH terms used to index this publication - Major topics in bold

Abnormalities, Multiple
Blalock-Taussig Procedure
Brain Damage, Chronic
Child
Child, Preschool
Developmental Disabilities
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