Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial.

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.

• Authors: Jane W Newburger, Lynn A Sleeper, David C Bellinger, Caren S Goldberg, Sarah Tabbutt, Minmin Lu, Kathleen A Mussatto, Ismee A Williams, Kathryn E Gustafson, Seema Mital, Nancy Pike, Erica Sood, William T Mahle, David S Cooper, Carolyn Dunbar-Masterson, Catherine Dent Krawczeski, Alan Lewis, Shaji C Menon, Victoria L Pemberton, Chitra Ravishankar, Teresa W Atz, Richard G Ohye, J William Gaynor, Pediatric Heart Network Investigators
• Journal: Circulation (Circulation) Vol. 125 Issue 17 Pg. 2081-91 (May 01 2012) ISSN: 1524-4539 [Electronic] United States
• PMID: 22456475 (Publication Type: Journal Article, Multicenter Study, Randomized Controlled Trial, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)

Topics

• Abnormalities, Multiple (epidemiology)
• Blalock-Taussig Procedure
• Brain Damage, Chronic (epidemiology, etiology, prevention & control, rehabilitation)
• Child
• Child, Preschool
• Developmental Disabilities (epidemiology, etiology, rehabilitation)
• Early Intervention, Educational
• Female
• Heart Ventricles (surgery)
• Humans
• Hypoplastic Left Heart Syndrome (complications, surgery)
• Hypoxia, Brain (etiology, psychology)
• Infant
• Infant, Newborn
• Male
• Neuropsychological Tests
• Norwood Procedures
• Postoperative Complications (epidemiology, etiology, rehabilitation)
• Prospective Studies
• Psychomotor Disorders (epidemiology, etiology, rehabilitation)
• Pulmonary Artery (surgery)
• Risk Factors
• Socioeconomic Factors
• Vascular Grafting (methods)