Congenital Heart Disease Early Developmental Outcome in Children With Hypoplastic Left Heart Syndrome and Related Anomalies The Single Ventricle Reconstruction Trial

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 14.3Ϯ1.1 (meanϮSD) months. Mean PDI (74Ϯ19) and MDI (89Ϯ18) scores were lower than normative means (each PϽ0.001). Neither PDI nor MDI score was associated with type of Norwood shunt. Independent predictors of lower PDI score (R 2 ϭ26%) were clinical center (Pϭ0.003), birth weight Ͻ2.5 kg (Pϭ0.023), longer Norwood hospitalization (PϽ0.001), and more complications between Norwood procedure discharge and age 12 months (PϽ0.001). Independent risk factors for lower MDI score (R 2 ϭ34%) included center (PϽ0.001), birth weight Ͻ2.5 kg (Pϭ0.04), genetic syndrome/anomalies (Pϭ0.04), lower maternal education (Pϭ0.04), longer mechanical ventilation after the Norwood procedure (PϽ0.001), and more complications after Norwood discharge to age 12 months (PϽ0.001). 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. S urvival to adulthood is becoming a reality for patients with hypoplastic left heart syndrome (HLHS) and other single right ventricle anomalies treated with staged repair from the Norwood operation to the Fontan procedure. This remarkable progress has exposed a high prevalence of neu-rodevelopmental impairment in survivors, 1–5 affecting their educational achievement, employability, and quality of life. 6,7 Potential risk factors for adverse neurodevelopmental out-The Single Ventricle Reconstruction (SVR) trial compared outcomes in subjects with HLHS or related anomalies palliated by using the Norwood procedure with either a modified Blalock-Taussig (MBT) shunt or the right-ven-tricular-to-pulmonary-artery (RV-to-PA) shunt. The primary outcome was freedom from death or cardiac trans-plantation by 12 months postrandomization. 25 In this article, we report an important prespecified secondary trial outcome, neurodevelopment assessed by in-person evaluation at 14 months after randomization. We evaluated the influence of shunt …