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Background: The purpose of this study was to determine whether a clinical outcome score derived from early postoperative events is associated with Bayley-III scores at 18 to 24 months among infants undergoing cardiopulmonary bypass surgery., Methods: Included were infants aged 6 weeks or less who underwent surgery between 2005 and 2009, all of whom were referred for neurodevelopmental evaluation at 18 to 24 months. We excluded children with chromosomal abnormalities. The prespecified clinical outcome score had a range of 0 to 7. Lower scores indicated a more rapid postoperative recovery. Patients requiring extracorporeal life support were assigned a score of 7., Results: One hundred and ninety-nine subjects were included. Surgical procedures were arterial switch (72), Norwood (60), repair of total anomalous pulmonary venous connection (29), and other (38). Nine subjects had postoperative extracorporeal life support. Mean clinical outcome score in the Norwood group was 4.0 ± 1.4 versus the arterial switch group (2.6 ± 1.5, p < 0.001), total anomalous pulmonary venous connection group (2.8 ± 1.8, p < 0.01), and other group (4.0 ± 1.8, p = not significant). Among children who had a clinical outcome score of 4 or greater, there was a decrease in Bayley-III cognitive score of 5.7 (95% confidence interval: 1.5 to 9.9, p = 0.009), a decrease in language score of 10.0 (95% confidence interval: 4.9 to 15.1, p < 0.001), and a decrease in motor score of 9.7 (95% confidence interval: 4.8 to 14.5, p < 0.001). Time until lactate of 2.0 mmol/L or less and highest 24-hour inotrope score increased with increasing clinical outcome score (p < 0.0001)., Conclusions: Clinical outcome scores of 4 or greater were associated with significantly lower Bayley-III scores at 18 to 24 months. This score may be valuable as an endpoint when evaluating novel potential therapies for this high-risk population., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Survival after heart transplant has improved and more attention is focused on developmental outcomes. We aimed to determine the survival, morbidity, and developmental outcomes of young children after heart transplant., Methods: All children under 6 years of age having a heart transplant in Edmonton between 1999 and 2006 were included in this inception cohort study. Demographics, pretransplant, transplant, and posttransplant variables were collected. The association of potentially predictive variables with neurodevelopmental outcomes at least 12 months posttransplant were determined by univariate and multiple regression analyses., Results: Thirty-three children had a heart transplant; 18 with congenital heart disease (CHD) and 15 with cardiomyopathy-myocarditis (non-CHD). Mortality during 19 (8) months of follow-up was 12% (95% confidence interval [CI] 3% to 28%). Survivors had frequent low weight (28%) and height (31%), and delay in language (41%), motor (52%), mental (34%), and general adaptive composite (48%) scores. Only CHD was associated with death-disability-mental delay on multiple regression (odds ratio 7.94; 95% CI 1.6 to 39.4, p=0.011). The CHD was also associated with mental and language delay on multiple regressions. Mental delay occurred in 8 (53%) with CHD and 2 (14%) with non-CHD (p=0.05). Mental score of 85 or greater was found in 13.4% of patients with CHD compared with 50% with non-CHD (p=0.05)., Conclusions: In this single-center inception cohort study, adverse neurodevelopmental outcomes in survivors of heart transplant before the age 6 years were common, particularly in those with CHD. Careful pretransplant and posttransplant counseling are needed, and close follow-up with early intervention for these high-risk children is imperative., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Red blood cell transfusions may or may not improve oxygen delivery to the tissues. Some studies suggest transfusion might contribute to adverse outcomes. This study investigated the association between hemoglobin concentration and transfusion with outcome in cyanotic neonates undergoing Norwood operations., Methods: Infants 6 weeks old or younger with hypoplastic left heart syndrome undergoing staged Norwood operations between September 1996 and July 2005 were included. Demographics and preoperative, operative, and postoperative variables were collected prospectively. Hemoglobin concentration, transfusion, fluid balance, and chest tube losses were collected retrospectively. The association of variables with outcomes, including early and 2-year mortality, mental and psychomotor developmental indices at 18 to 24 months of age, and ventilator days were determined by univariate and multiple regression analyses., Results: Ninety-four patients had Norwood operations. Excluded were 10 requiring postoperative extracorporeal life support and 2 with chromosomal abnormalities. By multiple regression analysis, only a higher nadir hemoglobin on days 2 to 5 postoperatively was associated with higher early mortality (odds ratio, 2.09; 95% confidence interval [CI], 1.14 to 3.87; p = 0.018), and the highest preoperative dopamine dose and highest epinephrine dose on day 2 to 5 postoperatively were associated with 2-year mortality; however, neither hemoglobin concentration nor number of transfusions were associated with mental or psychomotor developmental indices. The number of transfusions on day 2 to 5 postoperatively was associated with ventilator days in multiple variable analysis (effect size, 1.85; 95% CI, 0.33 to 3.36; p = 0.018)., Conclusions: This single-center cohort study found transfusion was not associated with improved outcomes in neonates undergoing Norwood operations., (Copyright (c) 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)