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Background Management of patients with hypoplastic left heart syndrome has benefited from advancements in medical and surgical care. Outcomes have improved, although survival and long-term functional and cognitive deficits remain a concern. Methods and Results This is a cohort study of all consecutive patients with hypoplastic left heart syndrome undergoing surgical palliation at a single center. We aimed to examine demographic and perioperative factors from each surgical stage for their association with survival and neurocognitive outcomes. A total of 117 consecutive patients from 1996 to 2010 underwent surgical palliation. Seventy patients (60%) survived to the Fontan stage and 68 patients (58%) survived to undergo neurocognitive assessment at a mean (SD) age of 56.6 months (6.4 months). Full-scale, performance, and verbal intelligence quotient, as well as visual-motor integration mean (SD) scores were 86.7 (16.1), 86.3 (15.8), 88.8 (17.2), and 83.2 (14.8), respectively. On multivariable analysis, older age at Fontan, sepsis peri-Norwood, lowest arterial partial pressure of oxygen postbidirectional cavopulmonary anastomosis, and presence of neuromotor disability pre-Fontan were strongly associated with lower scores for all intelligence quotient domains. Older age at Fontan and sepsis peri-Norwood remained associated with lower scores for all intelligence quotient domains in a subgroup analysis excluding patients with disability pre-Fontan or with chromosomal abnormalities. Conclusions Older age at Fontan and sepsis are among independent predictors of poor neurocognitive outcomes for patients with hypoplastic left heart syndrome. Further studies are required to identify the appropriate age range for Fontan completion, balancing a lower risk of acute and long-term hemodynamic complications while optimizing long-term neurocognitive outcomes.

Background: Clinicians often use information about developmental outcomes in decision-making around offering complex, life-saving interventions in children such as dialysis and renal transplant. This information in children with end-stage renal disease (ESRD) is limited, particularly when ESRD onset is in infancy or early childhood., Methods: Using data from an ongoing prospective, longitudinal, inception cohort study of children with renal transplant before 5 years of age, we evaluated (1) the risk of adverse neurocognitive and functional outcomes at 5 years of age and (2) predictors of developmental outcomes., Results: We found evidence of neurocognitive sequelae of ESRD in very young children; however, developmental outcomes appear remarkably better when compared with findings of two or three decades ago. Less time on dialysis predicted higher developmental scores, and hemodialysis was associated with poorer developmental outcomes., Conclusions: Our data suggest that renal replacement therapies in young children are associated with acceptable developmental outcome. Programs to identify those with developmental delays and provide early intervention may allow achievement of the child's full potential.

Background: We aimed to determine whether sepsis is associated with neurocognitive outcomes 4.5 years after congenital heart disease surgery in early infancy., Methods and Results: A secondary analysis from a prospective inception cohort included all children having congenital heart disease surgery done at ≤6 weeks of age with cardiopulmonary bypass at the Western Canadian referral center from 1996 to 2009. Follow-up at the referral center determined the primary outcomes at 4.5 years with full-scale, performance, and verbal intelligence quotients on the Wechsler Preschool and Primary Scale of Intelligence. Perioperative variables were collected prospectively, and confirmation of blood culture-positive sepsis was done retrospectively. Multiple linear regression models for neurocognitive outcomes and multiple Cox proportional hazards regression for mortality were determined. Sepsis occurred in 97 of 502 patients (19%) overall and in 76 of 396 survivors (19%) with 4.5-year follow-up. By 4.5 years, there were 91 (18%) deaths, and 396 of 411 survivors (96%) had follow-up completed. Extracorporeal membrane oxygenation was associated with worse scores on all neurocognitive outcomes on multivariable regression; the association between extracorporeal membrane oxygenation and full-scale intelligence quotient had a regression coefficient of -13.6 (95% CI -21.3 to -5.9; P=0.001). Sepsis perioperatively was associated with performance and verbal intelligence quotients, with a trend for full-scale intelligence quotient (P=0.058) on multivariable regression. The regression coefficient for sepsis was strongest for performance intelligence quotient (-5.31; 95% CI -9.84 to -0.78; P=0.022). Sepsis was not but extracorporeal membrane oxygenation was associated with mortality by 4.5 years., Conclusions: Perioperative sepsis and extracorporeal membrane oxygenation were associated with adverse neurocognitive outcomes on multivariable regression. Quality improvement to prevent sepsis has the potential to improve long-term neurocognitive outcomes in infants after surgery for congenital heart disease., (© 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)

This prospective inception cohort study determines kindergarten-entry neurocognitive abilities and explores their predictors following liver transplantation at age <3 yr. Of 52 children transplanted (1999-2008), 33 (89.2%) of 37 eligible survivors had psychological assessment at age 54.7 (8.4) months: 21 with biliary atresia, seven chronic cholestasis, and five acute liver failure. Neurocognitive scores (mean [s.d.], 100 [15]) as tested by a pediatric-experienced psychologist did not differ in relation to age group at transplant (≤12 months and >12 months): FSIQ, 93.9 (17.1); verbal (VIQ), 95.3 (16.5); performance (PIQ), 94.3 (18.1); and VMI, 90.5 (15.9), with >70% having scores ≥85, average or above. Adverse predictors from the pretransplant, transplant, and post-transplant (30 days) periods using univariate linear regressions for FSIQ were post-transplant use of inotropes, p = 0.029; longer transplant warm ischemia time, p = 0.035; and post-transplant highest serum creatinine, (p = 0.04). For PIQ, they were pretransplant encephalopathy, p = 0.027; post-transplant highest serum creatinine, p = 0.034; and post-transplant inotrope use, p = 0.037. For VMI, they were number of post-transplant infections, p = 0.019; post-transplant highest serum creatinine, p = 0.025; and lower family socioeconomic index, p = 0.039. Changes in care addressing modifiable predictors, including reducing acute post-transplant illness, pretransplant encephalopathy, transplant warm ischemia times, and preserving renal function, may improve neurocognitive outcomes., (© 2013 John Wiley & Sons A/S.)

Objective: Our goal was to determine if a clinical outcome score derived from early postoperative events is associated with 18- to 24-month Psychomotor Developmental Index (PDI) score among infants undergoing cardiopulmonary bypass surgery., Methods: We included infants aged ≤6 weeks who underwent surgery during 2002-2006, all of whom were referred for neurodevelopmental evaluation at age 18 to 24 months. We excluded children with chromosomal abnormalities, hearing loss, cerebral palsy, or a Bayley III assessment. The prespecified clinical outcome score had a range of 0 to 7. Lower scores indicated a more rapid postoperative recovery. Patients requiring extracorporeal membrane oxygenation were assigned a score of 7., Results: Ninety-nine subjects were included. Surgical procedures were arterial switch (n = 36), Norwood (n = 26), repair of total anomalous pulmonary venous connection (n = 16), and other (n = 21). Four subjects had postoperative extracorporeal membrane oxygenation. Clinical outcome scores were highest in the Norwood group (mean 4.1 ± 1.4) compared with the arterial switch group (1.9 ± 1.6) (P < .001), total anomalous pulmonary venous connection group (1.6 ± 2.0) (P < .001), and other group (3.3 ± 1.6, P = not significant). A mean decrease in PDI of 10.9 points (95% confidence interval, 4.9-16.9; P = .0005) was observed among children who had a clinical outcome score ≥3, compared with those with a clinical outcome score <3. Time until lactate ≤2.0 mmol/L increased with increasing clinical outcome score (P = .0003), as did highest 24-hour inotrope score (P < .0001)., Conclusions: Clinical outcome scores of ≥3 were associated with a significantly lower PDI at age 18 to 24 months. This score may be valuable as an end point when evaluating novel potential therapies for this high-risk population., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: The newest measure of neurodevelopmental outcomes, the Bayley Scales of Infant and Toddler Development, 3rd Edition (Bayley-III), gives higher-than-expected scores for preterm infants; results after cardiac surgery are unknown., Objectives: The goal of this study was to report Bayley-III scores after cardiac surgery and compare the results with those of the Bayley Scales of Infant Development, 2nd Edition (BSID-II) on a subset of the same children., Methods: In this prospective, inception cohort, neurodevelopmental outcome study after complex cardiac surgery in infants from 2004 to 2007, the Bayley-III was given to 110 survivors (68% boys) at a mean age of 21 months (SD: 4 months). Analysis of variance was used to compare intergroup differences. Results for both test editions on the same 25 children were compared by using paired-samples statistics., Results: Mean (SD) Bayley-III mean composite scores (CSs) for 110 children were as follows: cognitive, 95.9 (14.1); language, 90.8 (18.1); and motor, 93.7 (14.2), differentiating selected cardiac surgery groups. The average difference in mean CSs was 7.4 points higher than BSID-II scores for a previous cohort from this site and 7.2 points higher than a systematic review report. Direct comparison of BSID-II and Bayley-III revealed an average difference in mean CSs of 6.1 points, similar to normative results. Mean cognitive CSs increased by 10.0 (P <.001), language by 1.4 (P = .526), and motor by 6.9 points (P = .009)., Conclusions: Researchers should be careful attributing higher Bayley-III scores to changes in acute care. At-risk children who previously qualified for early developmental intervention may no longer do so. School-age longitudinal studies are needed to determine the accuracy of early developmental estimates using the Bayley-III.

Aim: To examine survival and neurodevelopmental outcomes in neonates having post-operative cardiopulmonary resuscitation (CPR)., Methods: This inception cohort study included all neonates (6 weeks old or less) who received postoperative CPR (Group 1) after cardiac surgery from 1996 to 2005, matched for heart defect, year of surgery, chromosomal abnormality, and socioeconomic status to two neonates who did not receive postoperative CPR (Group 2). Two-year neurodevelopment was prospectively assessed with Bayley Scales of Infant Development II and Adaptive Behavior Assessment System II. Pre-, intra-, and post-operative variables were collected prospectively. Cardiac arrest variables were collected retrospectively. Predictors of mortality were analyzed by univariate analysis and conditional multiple logistic regression., Results: There were 29 patients in Group 1, and 58 patients in Group 2. In survivors, there were no significant differences between Groups 1 and 2 in outcomes [mean (SD)] of mental developmental index [84.5 (12.2) vs. 81.0 (18.9)], psychomotor developmental index [82.8 (13.8) vs. 80.1 (21.9)], General Adaptive Composite [84.6 (15.3) vs. 84.3 (19.2)], Motor scale [8.4 (3.2) vs. 8.0 (3.8)], or delay on any of these scales. Two-year mortality [58.6% Group 1; 8.6% Group 2], was associated on conditional multiple logistic regression with CPR (OR 26.6; 95% CI, 5.4, 129.5). In Group 1, on multiple logistic regression, 2-year mortality was associated with minutes of chest compressions (OR 1.04, 95% CI, 1.01, 1.08)., Conclusions: Among neonates having cardiac surgery, CPR is associated with greater mortality. There is no evidence that CPR survivors have different 2-year neurodevelopmental outcomes than those neonates not having CPR., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Objective: This study determined neurodevelopmental outcomes of survivors of neonatal cardiac surgery for interrupted aortic arch through an interprovincial program and explored preoperative, intraoperative, and postoperative outcome predictors., Methods: Children who underwent neonatal cardiac surgery for interrupted aortic arch at 6 weeks old or younger between 1996 and 2006 had a multidisciplinary neurodevelopmental assessment at 18 to 24 months old (mental and psychomotor developmental indices as mean +/- SD and delay [score <70]). Survivor outcomes were compared by univariate and multivariate analyses and compared between children with and without chromosomal abnormality., Results: Outcomes were available for all 26 survivors (mortality, 3.7%). Mental and psychomotor developmental indices were 75.8 +/- 17.1 and 72.3 +/- 16.9, respectively, with significantly lower scores for children with chromosomal abnormalities, which accounted for 29% of the variance in developmental indices. For the remaining 17 children without chromosomal abnormalities, mental and psychomotor developmental indices were 82.7 +/- 14.5 and 79.1 +/- 14.3, respectively, with deep hypothermic circulatory arrest time and Apgar score at 5 minutes contributing 46% of the variance in mental developmental index., Conclusions: The neurodevelopmental indices of children who have undergone neonatal cardiac surgery for interrupted aortic arch are below normative values; those of children with chromosomal abnormalities are even lower. For children without a chromosomal abnormality, longer deep hypothermic circulatory arrest times and low Apgar scores predict lower mental developmental indices at 18 to 24 months of age.

Objective: Neonatal complex cardiac surgery carries a significant risk for adverse neurodevelopmental outcome. We hypothesized this risk to be higher in patients with deletion 22q11.2., Methods: From 1996 to 2004, neonates who had complex cardiac surgery at age 6 weeks or less had multisite, multidisciplinary health and neurodevelopmental outcomes (Bayley Scales of Infant Development II; mental and psychomotor developmental indices [MDI, PDI] as mean [SD] and delay [<70]) assessed at 18 to 24 months of age. All 16 patients with deletion 22q11.2 (group 1) were compared with 16 patients without deletion 22q11.2 (group 2) having undergone neonatal complex cardiac surgery at the same center and matched for cardiac lesion, socioeconomic status, and year of operation. Outcomes were compared by univariate and multivariate analyses., Results: Heart lesions in each group consisted of 6 (37.5%) cases of interrupted aortic arch, 6 (37.5%) cases of truncus arteriosus, and 4 (25%) cases of tetralogy of Fallot. Outcomes were available for all survivors. Mortality was 3 (19%) and 1 (6%) in groups 1 and 2, respectively (P = .6). MDI and PDI scores were 66.1 (10.6) and 55.0 (9.4) for group 1 and 86.3 (14.6) and 82.3 (14.3) for group 2 (P < .001). Only deletion 22q11.2 was significant in stepwise multiple regression to predict MDI and PDI scores. Mental delay occurred in 8 (61.5%) of 13 in group 1 and 2 (13.8%) of 15 in group 2 (P = .016). Psychomotor delay occurred in 11 (84.6%) of 13 in group 1 and 1 (6.7%) of 15 in group 2 (P < .001)., Conclusion: Neonates affected by deletion 22q11.2 and having neonatal complex cardiac surgery have significantly worse neurodevelopmental outcome than do those without deletion 22q11.2.

Objective: This interprovincial inception cohort study explores early childhood outcomes and their operative and perioperative predictors after total anomalous pulmonary venous connection repair, simple (patent ductus arteriosus, atrial septal defect, or both) or complex (any associated major cardiac anomalies)., Methods: From 1996 through 2004, a total of 41 consecutive neonates with total anomalous pulmonary venous connection underwent complete repair with deep hypothermic circulatory arrest. Multidisciplinary health and neurodevelopmental outcomes (Bayley Scales of Infant Development II [1993], Mental and Psychomotor Developmental Indices) were assessed at 18 to 24 months of age. Regression analyses explored potentially modifiable predictors of outcome., Results: Survival after simple total anomalous pulmonary venous connection repair was 31 of 32 (97%); that after complex repair was 3 of 9 (33%). Relative risk of death was higher for complex (21.3) and obstructive (8.4) total anomalous pulmonary venous connections. Those who died had longer deep hypothermic circulatory arrest times (P < .001). For 34 survivors, Mental Developmental Index was 87 +/- 16 and Psychomotor Developmental Index was 89 +/- 13. Among survivors, 24.6% of variability in Mental Developmental Index was explained by its linear relationship with socioeconomic status, whereas 35.5% of variability in Psychomotor Developmental Index was explained by its linear relationship with weight at surgery and highest plasma lactate level after surgery. North American native peoples made up 38% of survivors, a higher than expected number., Conclusion: Mean developmental scores were in the low average range of normative data. Socioeconomic factors predicted mental outcome. High postoperative lactate level on day 1 predicted low motor scores. Other potentially modifiable variables will need to be explored. Further epidemiologic study relating race to occurrence of total anomalous pulmonary venous connection is suggested. Early neurodevelopmental assessment is recommended.

Objectives: An observational cohort study was conducted in infants less than 6 weeks of age undergoing intracardiac surgery to examine the predictive value of serial postoperative lactate determination on survival and early childhood neurodevelopment., Methods: A total of 85 infants with congenital heart disease underwent intracardiac surgery between 1996 and 1999. Differences in serial lactate concentrations after surgery among 3 outcome groups were compared. The predictive value of plasma lactate concentration on outcome (1) at discharge from initial hospitalization and (2) 18 to 24 months postnatal age was examined., Results: Compared with survivors, the nonsurvivors had higher lactate concentrations on admission to the pediatric intensive care unit at day 1 peak and area under the curve of the lactate profile than those of adverse and intact survivors (all P < .001, analysis of variance). Significant differences in the time for lactate concentrations to return to 2 mmol/L or less during the first postoperative day were observed among the groups: nonsurvivors > adverse survivors > intact survivors. Lactate concentrations of less than 7 mmol/L on admission or less than 8 mmol/L at day 1 peak predicted survival with 82% sensitivity and 83% specificity, and positive and negative predictive values of 97% and 43%, respectively (P < .001, chi2). Plasma lactate concentrations were associated with adverse outcome but had lower predictive values compared with that for nonsurvival., Conclusions: Serial lactate determination accurately predicts survival and may help differentiate survivors with adverse outcome from those with intact neurodevelopment in early childhood.

Objectives: To determine 18-month neurodevelopmental outcome of survivors of complex open heart surgery at