Deterioration of functional abilities in children surviving the Fontan operation.

Functional abilities are needed for activities of daily living. In general, these skills expand with age. We hypothesised that, in contrast to what is normally expected, children surviving the Fontan may have deterioration of functional abilities, and that peri-Fontan stroke is associated with this deterioration. All children registered in the Western Canadian Complex Pediatric Therapies Follow-up Program who survived a Fontan operation in the period 1999-2016 were eligible for inclusion. At the age of 2 years (pre-Fontan) and 4.5 years (post-Fontan), the Adaptive Behavior Assessment System-II general adaptive composite score was determined (population mean: 100, standard deviation: 15). Deterioration of functional abilities was defined as ⩾1 standard deviation decrease in pre- to post-Fontan scores. Perioperative strokes were identified through chart review. Multivariable logistic regression analysis determined predictors of deterioration of functional abilities. Of 133 children, with a mean age at Fontan of 3.3 years (standard deviation 0.8) and 65% male, the mean (standard deviation) general adaptive composite score was 90.6 (17.5) at 2 years and 88.3 (19.1) at 4.5 years. After Fontan, deterioration of functional abilities occurred in 34 (26%) children, with a mean decline of 21.8 (7.1) points. Evidence of peri-Fontan stroke was found in 10 (29%) children who had deterioration of functional abilities. Peri-Fontan stroke (odds ratio 5.00 (95% CI 1.74, 14.36)) and older age at Fontan (odds ratio 1.67 (95% CI 1.02, 2.73)) predicted functional deterioration. The trajectory of functional abilities should be assessed in this population, as more than 25% experience deterioration. Efforts to prevent peri-Fontan stroke, and to complete the Fontan operation at an earlier age, may lead to reduction of this deterioration.

Early childhood language outcomes after arterial switch operation: a prospective cohort study.

BACKGROUND: Developmental and language outcomes at 2 years of age of children who had arterial switch operation (ASO) for transposition of the great arteries 2004-2010 are described. METHODS: In this prospective cohort study, 91/98 (93 %) children who underwent ASO were assessed at 2 years of age with the Bayley Scales of Infant & Toddler Development-3rd Edition. Outcomes were compared by patient and perioperative variables using bivariate and multivariate regression analyses to identify predictors of language delay. RESULTS: Infants without ventricular septal defect (VSD) (n = 60) were more likely to be outborn (73 vs 58 %, p = 0.038), require septostomy (80 vs 58 %, p = 0.026), have a shorter cross clamp time (min) (62.7 vs 73.0, p = 0.019), and a lower day 1 post-operative plasma lactate (mmol/L) (3.9 vs 4.8, p = 0.010). There were no differences in cognitive, motor and language outcomes based on presence of a VSD. Language delay (<85) of 29 % was 1.8 times higher than the normative sample; risk factors for this in multivariate analyses included <12 years of maternal education (AOR 19.3, 95 % CI 2.5-148.0) and cross-clamp time ≥70 min (AOR 14.5, 95 % CI 3.1-68.5). Maternal education <12 years was associated with lower Language Composite Scores (-20.2, 95 % CI -32.3 to -9.1). CONCLUSIONS: Outcomes at 2 years of age in children who undergo ASO are comparable to the normative sample with the exception of language. There is a risk of language delay for which maternal education and cross-clamp duration are predictors. These findings suggest that focused post-operative early language interventions could be considered.

Clinical and Functional Developmental Outcomes in Neonates Undergoing Truncus Arteriosus Repair: A Cohort Study.

BACKGROUND: Truncus arteriosus (TA) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired. The objective of this study was to assess the clinical and developmental outcomes in a prospective cohort of patients who underwent TA repair. METHODS: All patients who underwent a TA repair between 1996 and 2012 were included. Follow-up clinical, neurologic, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Functional developmental outcomes were assessed at 21.1 ± 2.5 months of age with the Adaptive Behavior Assessment System-II, General Adaptive Composite (GAC) score. Survival and outcomes were compared between those with and without chromosomal abnormalities (CA). Survival and freedom from reintervention were assessed by Kaplan-Meier analysis. RESULTS: The study comprised 36 infants (19 male). CA was identified in 13, with 22q11.2 deletion in 10 patients. Patients underwent TA repair at a median age of 10 days; 5 patients underwent concomitant interrupted arch repair. There were 8 deaths, 2 of which occurred in the hospital. The 5-year survival was 79.4%. Survival was similar between those with and without CA. At 5 years, freedom from reoperation was 77.2%. The mean GAC was higher in the patients without CA (93.6 ± 12.8 vs 76.1 ± 13.1, p = 0.0016). CONCLUSIONS: Patients with surgically repaired TA continue to have significant postoperative mortality. Reoperation and cardiac catheterization are eventualities for a quarter of patients in the first 5 years of life. Functional developmental outcome in patients without CA is good, although it is significantly impaired in those with CA.

Acute kidney injury after heart transplant in young children: risk factors and outcomes.

BACKGROUND: Critical illness following heart transplantation can include acute kidney injury (AKI). Study objectives were to define the epidemiology of, risk factors for, or impact on outcomes of AKI after pediatric heart transplant. METHODS: Using data from a prospective study of 66 young children, we evaluated: (1) post-operative AKI rate (by pediatric modified RIFLE criteria); (2) pre, intra, and early post-operative AKI risk factors using stepwise logistic regression (3) effect of AKI on short-term outcomes (ventilation and length of pediatric intensive care unit (PICU) stay) using stepwise multiple regression. RESULTS: AKI occurred in 73 % of children. Pre-transplant ventilation and higher baseline estimated creatinine clearance (eCCl) were independent risk factors for AKI. Pre-operative inotrope use was associated with reduced risk of AKI. Tacrolimus level emerged as important in multivariable risk prediction. Children with AKI had a longer duration of ventilation and length of pediatric intensive care unit (PICU) stay, with AKI being an independent predictor. CONCLUSIONS: AKI was common after heart transplant and associated with more complicated early post-transplant course. Lower baseline eCCl was associated with lower incidence of AKI; this merits further investigation. The association of pre-operative inotropes with less AKI may reflect a pathophysiological mechanism or be a surrogate for clinical factors and management prior to transplant. Avoiding high tacrolimus levels may be a modifiable risk factor for AKI.

Screening for language delay after life-saving therapies in term-born infants.

BACKGROUND: Strong recommendations have been made for the periodic developmental surveillance, screening, and evaluation of children with CHD. This supports similar calls for all at-risk children in order to provide timely, structured early developmental intervention that may improve outcomes. The aim of this study was to determine the accuracy of screening for language delay after life-saving therapies using the parent-completed vocabulary screen of the language Development Survey, by comparing screening with the individually administered language scores of the Bayley Scales of Infant and Toddler Development, Third edition. METHOD: In total, 310 (92.5%) of 335 eligible term-born children, born between 2004 and 2011, receiving complex cardiac surgery, heart or liver transplantation, or extracorporeal membrane oxygenation in infancy, were assessed at 21.5 (2.8) months of age (lost, 25 (7.5%)), through developmental/rehabilitation centres at six sites as part of the Western Canadian Complex Pediatric Therapies Follow-up Group. RESULTS: Vocabulary screening delay was defined as scores ⩽15th percentile. Language delay defined as scores >1 SD below the mean was calculated for language composite score, receptive and expressive communication scores of the Bayley-III. Delayed scores for the 310 children were as follows: vocabulary, 144 (46.5%); language composite, 125 (40.3%); receptive communication, 98 (31.6%); and expressive communication, 124 (40%). Sensitivity, specificity, positive predictive values, and negative predictive values of screened vocabulary delay for tested language composite delay were 79.2, 75.7, 68.8, and 84.3%, respectively. CONCLUSION: High rates of language delay after life-saving therapies are concerning. Although the screening test appears to over-identify language delay relative to the tested Bayley-III, it may be a useful screening tool for early language development leading to earlier referral for intervention.

Gastrostomy Tube Feeding after Neonatal Complex Cardiac Surgery Identifies the Need for Early Developmental Intervention.

OBJECTIVE: To compare the proportion of developmental delay in early complex cardiac surgery (CCS) survivors with and without gastrostomy tube feeding (GTF). To explore acute care predictors of GTF that might help improve care in CCS survivors. STUDY GROUP: This comparison study of 2 groups within an inception cohort included 334 CCS survivors after cardiopulmonary bypass at ≤6 weeks of age (2005-2012) who did not require extracorporeal membrane oxygenation or heart transplantation. Children were assessed at 21 ± 3 months with the Bayley Scales of Infant and Toddler Development-Third Edition and the Adaptive Behavior Assessment System-Second Edition: general adaptive composite score. Delay was determined by scores >2 SD below mean. The χ(2) test compared groups. Predictors of GTF were analyzed using multiple logistic regression analysis, results expressed as OR with 95% CI. RESULTS: Of the survivors, 67/334 (20%) had GTF any time before the 21-month assessment. Developmental delays in children with GTF were cognitive in 16 (24%), motor in 18 (27%), language in 24 (36%) vs without GTF in 7 (3%), 8 (3%), and 32 (12%), respectively (P < .001). Gastrostomy group had almost 8 times the number of children delayed on the general adaptive composite score. Independent OR for GTF are presence of a chromosomal abnormality, OR 4.6 (95% CI 1.8, 12.0) (P = .002), single ventricle anatomy, OR 3.4 (95% CI 1.7, 6.8) (P < .001), total postoperative days of open sternum, OR 1.15 (95% CI 1.1, 1.3) (P = .031), and total number of hospital days at CCS, OR 1.03 (95% CI 1.1, 1.04) (P = .002). CONCLUSIONS: GTF identifies CCS survivors at risk for delay, who would benefit from early developmental intervention. The described mostly nonmodifiable predictors may guide counseling of these children's families.

Sepsis After Cardiac Surgery Early in Infancy and Adverse 4.5-Year Neurocognitive Outcomes.

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.

Survival and neurocognitive outcomes in pediatric extracorporeal-cardiopulmonary resuscitation.

OBJECTIVE: Extracorporeal Cardiopulmonary Resuscitation (E-CPR) is the initiation of extracorporeal life support during active chest compressions. There are no studies describing detailed neurocognitive outcomes of this population. We aim to describe the survival and neurocognitive outcomes of children who received E-CPR. METHODS: Prospective cohort study. Children who received E-CPR at the Stollery Children's Hospital between 2000 and 2010 were included. Neurocognitive follow-up, including Wechsler Preschool and Primary Scales of Intelligence, was completed at the age of 4.5 years, and at a minimum of 6 months after the E-CPR admission. RESULTS: Fifty-five patients received E-CPR between 2000 and 2010. Children with cardiac disease had a 49% survival to hospital discharge and 43% survival at age 5-years, with no survivors (n=4) in those with non-cardiac disease. Pediatric E-CPR survivors had a mean (SD) Full Scale Intelligence quotient (FSIQ) score of 76.5 (15.9); with 4 children (24%) having intellectual disability (defined as FSIQ over 2 standard deviations below the population mean; i.e., <70). Multiple Cox regression analysis found that mechanical ventilation prior to E-CPR, open chest CPR, longer duration of CPR, low pH and more red blood cells given on the first day of ECMO, and longer time for lactate to normalize on ECMO were associated with higher mortality at age 5-years. CONCLUSION: Pediatric patients with cardiac disease who required E-CPR had 43% survival at age 5 years. Of concern, the intelligence quotient in E-CPR survivors was significantly lower than the population mean, with 24% having intellectual disability.

Clinical Outcome Score Predicts Adverse Neurodevelopmental Outcome After Infant Heart Surgery.

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.

Survival and neurocognitive outcomes after cardiac extracorporeal life support in children less than 5 years of age: a ten-year cohort.

BACKGROUND: Survival after pediatric cardiac extracorporeal life support (ECLS) is guarded, and neurological morbidity varies widely. Our objective is to report our 10-year experience with cardiac ECLS, including survival and kindergarten entry neurocognitive outcomes; to identify predictors of mortality or adverse neurocognitive outcomes; and to compare 2 eras, before and after 2005. METHODS AND RESULTS: From 2000 to 2009, 98 children had venoarterial cardiac ECLS. Sixty-four patients (65%) survived to hospital discharge, and 50 (51%) survived ≤5 years of age. Neurocognitive follow-up of survivors was completed at mean (SD) age of 52.9 (8) months using Wechsler Preschool and Primary Scale of Intelligence. Logistic regression analysis found the longer time (hours) for lactate to fall below 2 mmol/L on ECLS (hazard ratio, 1.39; 95% confidence interval, 1.05, 1.84; P=0.022), and the amount of platelets (mL/kg) given in the first 48 hours (hazard ratio, 1.18; 95% confidence interval, 1.06, 1.32; P=0.002) was independently associated with higher in-hospital mortality. Receiving ECLS after the year 2005 was independently associated with lower risk of in-hospital mortality (hazard ratio, 0.36; 95% confidence interval, 0.13, 0.99; P=0.048). Extracorporeal cardiopulmonary resuscitation was not independently associated with mortality or neurocognitive outcomes. Era was not independently associated with neurocognitive outcomes. The full-scale intelligence quotient of survivors without chromosomal abnormalities was 79.7 (16.6) with 25% below 2 SD of the population mean. CONCLUSIONS: Mortality has improved over time; time for lactate to fall on ECLS and volume of platelets transfused are independent predictors of mortality. Extracorporeal cardiopulmonary resuscitation and era were not independently associated with neurocognitive outcomes.

Neurocognitive outcomes at kindergarten entry after surgical repair of total anomalous pulmonary venous connection in early infancy.

The objective of this study was to determine neurocognitive outcomes 4.5 years after surgery for TAPVC in infancy and predictors of these outcomes. A cohort having TAPVC repair at age ≤6 weeks between 1998 and 2007 were followed by the Complex Pediatric Therapies Follow-up Program at 4.5 years. Outcomes include mortality, full-scale intelligence quotient (FSIQ), verbal IQ (VIQ), performance IQ (PIQ), visual motor integration (VMI), and general adaptive composite of the Adaptive Behavior Assessment System (GAC). There were 51 infants with simple TAPVC [4 year mortality 4 (8%)], and 16 with complex TAPVC [4 year mortality 7 (44%)], hazard ratio (HR) 7.02 (95% CI 2.05-24.07, p = 0.002). Of the 47 survivors after simple TAPVC, FSIQ (SD) was 92 (17), VIQ 92 (17), PIQ 94 (15), VMI 92 (15), and GAC 92 (15). Independent predictors of neurocognitive outcome included father's socioeconomic status, mother's years of schooling, gender, post-operative base deficit, and deep hypothermic circulatory arrest (DHCA) time. Complex TAPVC was associated on univariate analysis only with PIQ [81.9 (10.2) vs. 93.6 (15.4); p = 0.012] and FSIQ [80.7 (10.1) vs. 92.0 (17.7); p = 0.017]. Original peoples accounted for 25/51 (49%) of simple and 3/16 (19%) of complex TAPVC. Original peoples race was associated with 4-year mortality [HR 6.85 (95% CI 2.15, 21.76, p = 0.001)]. Survivors of TAPVC repair in early infancy have encouraging neurocognitive outcomes. Few independent predictors of neurocognitive outcome were found, with post-operative acidosis and DHCA time being potentially modifiable. Original peoples account for an unexpected proportion of patients (42%) and have a higher mortality.

Prediction of preschool functional abilities after early complex cardiac surgery.

BACKGROUND: It is important to identify early predictors of functional limitations in children after congenital heart surgery to optimise their independence as they prepare for school. The purpose of this study is to determine potentially modifiable predictor variables of functional abilities in pre-school children who underwent complex cardiac surgery at 6 weeks of age or earlier. METHODS: This prospective inception cohort study comprised a sample of 165 survivors (63% boys) who had complex cardiac surgery (75% biventricular repairs) at Stollery Children's Hospital, Edmonton, Alberta. We excluded children with chromosomal abnormalities. When children were 4-5 years of age, the parents completed the Adaptive Behavioral Assessment System II. Regression analysis was used to assess the association between multiple risk factors and each of the four continuous composite scores. RESULTS: The mean scores for the practical domain and general adaptive composite score of the Adaptive Behavioural Assessment System were lower than the conceptual and social domains, with 13.3% of the children having a delay in the practical domain. There was a significant association between the general adaptive (p=0.003; 0.012), conceptual (p=0.0004; 0.042), social (p=0.0007; 0.028), and the practical (p=0.046; 0.003) domain composite scores with the mother's education and preoperative plasma lactate, respectively. CONCLUSION: Maternal education may be a marker for the social context of children, and warrants societal attention to improve functional outcomes. Preoperative lactate as a potentially modifiable variable may warrant increased attention to early diagnosis and aggressive resuscitation of young infants with congenital heart disease.

Health-related quality of life in pediatric cardiac extracorporeal life support survivors.

OBJECTIVE: To assess the health-related quality of life of children who received cardiac extracorporeal life support. We hypothesized that extracorporeal life support survivors have lower health-related quality-of-life scores when compared with a healthy sample, with children with chronic conditions, and with children who had surgery for congenital heart disease and did not receive extracorporeal life support. DESIGN: Prospective cohort study. SETTING: Stollery Children's Hospital and Complex Pediatric Therapies Follow-up Program clinics. PATIENTS: Children less than or 5 years old with diagnosis of cardiac disease (congenital or acquired) who received extracorporeal life support at the Stollery Children's Hospital from 1999 to 2009. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Health-related quality of life was assessed using the PedsQL 4.0 Generic Core Scales completed by the children's parents at the time of follow-up. Forty-seven cardiac extracorporeal life support survivors had their health-related quality of life assessed at a median age of 4 years. Compared with a healthy sample, children who received venoarterial extracorporeal life support have significantly lower PedsQL (64.9 vs 82.2; p < 0.0001). The PedsQL scores of children who received extracorporeal life support were also significantly lower than those of children with chronic health conditions (64.9 vs 73.1; p = 0.007). Compared with children with congenital heart disease who underwent cardiac surgery early in infancy and who did not receive extracorporeal life support, extracorporeal life support survivors had significantly lower PedsQL scores (64.9 vs 81.1; p < 0.0001). Multiple linear regression analysis found an independent association between both higher inotrope score in the first 24 hours of extracorporeal life support and longer hospital length of stay, with lower PedsQL scores. CONCLUSIONS: Pediatric cardiac extracorporeal life support survivors showed lower health-related quality of life than healthy children, children with chronic conditions, and children with congenital heart disease who did not receive extracorporeal life support.

Neurotoxicity of sedative and analgesia drugs in young infants with congenital heart disease: 4-year follow-up.

OBJECTIVES/AIM: To determine whether sedation/analgesia drugs used before, during, and after infant cardiac surgery are associated with neurocognitive and functional outcomes. BACKGROUND: Some animal models suggest neurotoxic effects of anesthetic drugs on the developing brain; however, potential human effects are unknown. Whether these results can be extrapolated to humans is unknown. METHODS/MATERIALS: Prospective follow-up project of all infants ≤6 weeks old having surgery for congenital heart disease between 04/03 and 12/06. Demographic, perioperative, and sedation/analgesia variables were collected. Outcomes at kindergarten age were Wechsler Preschool and Primary Scale of Intelligence-III, Beery-Buktenica Developmental Test of Visual Motor Integration (VMI-V), and General Adaptive Composite (GAC) of the Adaptive Behavior Assessment System-II. Multivariable linear regression was used to identify predictor variables. RESULTS: From 135 infants who underwent heart surgery, 19 died, 17 were excluded, 8 were lost to follow-up, leaving 91 children for analysis. Multiple linear regression found days on chloral hydrate [3.5 (3.7) days] was associated with lower performance intelligence quotient (PIQ) (Effect size -1.03; 95% CI -1.96, -0.10; P = 0.03), and cumulative dose [54.2 (60.3) mg·kg(-1) ] of benzodiazepines was associated with lower VMI scores (Effect size -0.07; 95% CI -0.12, -0.01; P = 0.026). No other associations were found between sedation/analgesia variables and full-scale IQ, PIQ, Verbal IQ, VMI, or GAC. CONCLUSION: Assessment of this cohort at kindergarten age found a small statistically significant association between days on chloral hydrate and PIQ, and benzodiazepine cumulative dose and lower VMI. No other association between sedation/analgesia drugs and outcomes was found.

Health-related quality of life experienced by children with chromosomal abnormalities and congenital heart defects.

Long-term outcomes are fundamental in advising parents about the potential future of their children with congenital heart disease (CHD). No published reports have described the health-related quality of life (HRQL) experienced by children with chromosomal abnormalities who had surgery in early infancy for CHD. A study was undertaken to assess HRQL among children with chromosomal abnormalities and CHD. The authors hypothesized that these children have a worse HRQL than healthy children or a cohort of children matched for CHD diagnosis. Infants with chromosomal abnormalities undergoing cardiac surgery for CHD at 6 weeks of age or younger at the Stollery Children's Hospital between July 2000 and June 2005 were included in the study. The HRQL of these infants was assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales completed by their parents at a 4-year follow-up evaluation. The study compared the scores for 16 children with normative data. The children with chromosomal abnormalities and CHD had significantly lower mean total PedsQL (71.3 vs. 87.3; p < 0.0001), Psychosocial Summary (70.3 vs. 86.1; p < 0.0001), and Physical Summary (74.3 vs. 89.2; p = 0.0006) scores. Compared with the matched children, those with chromosomal abnormalities had a significantly lower median total PedsQL (75.0 vs. 84.6; p = 0.03), Physical Summary (79.5 vs. 96.9; p = 0.007), and School Functioning (68.5 vs. 83.0; p = 0.03) scores. A better understanding of the mechanisms and determinants of HRQL in these children has the potential to yield important implications for clinical practice including clarity for treatment decision making as well as determination of targeted supports and services to meet the needs of these children and their families differentially.

Neurocognitive outcomes at kindergarten entry after liver transplantation at <3 yr of age.

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.

Quality of life 4 years after complex heart surgery in infancy.

OBJECTIVE: To determine the health-related quality of life at 4 years of age in children who had undergone cardiac surgery for congenital heart disease in early infancy. METHODS: A prospective cohort study of infants undergoing cardiac surgery at 6 weeks of age or younger from July 2000 to June 2005 at the Stollery Children's Hospital. The quality of life was assessed using the Pediatric Quality of Life Inventory, version 4.0, generic core scales, and compared with normative values for the same age. The association between the perioperative variables and health-related quality of life was explored. RESULTS: A total of 242 infants underwent complex heart surgery during the study period. Of the 166 eligible survivors, 130 were included. No significant differences were present between the children with single ventricle versus biventricular repairs, except for lower physical health summary scores in the single ventricle patients (P = .007). Compared with the normative data, the children with biventricular repair had lower total Pediatric Quality of Life Inventory, version 4.0, scores (P = .001) and psychosocial health summary scores (P < .001). The children with single ventricle repair also had lower physical health summary scores (P = .003). Older age at surgery and markers of postoperative low cardiac output syndrome were associated with worse health-related quality of life, and greater socioeconomic status was associated with better quality of life. CONCLUSIONS: At 4 years of age, health-related quality of life was significantly lower in children who had undergone surgery for congenital heart disease in early infancy. An association was found between age at surgery and postoperative low cardiac output and socioeconomic status and quality of life.

Clinical outcome score predicts the need for neurodevelopmental intervention after infant heart surgery.

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.

Risk factors for and outcomes of acute kidney injury in neonates undergoing complex cardiac surgery.

OBJECTIVE: To characterize the epidemiology of and identify risk factors for neonatal cardiac surgery-associated acute kidney injury (CS-AKI) and determine its impact on clinical outcomes. STUDY DESIGN: Using secondary analysis of data from an ongoing multiprovincial prospective cohort study, we studied 264 neonates undergoing complex cardiac repair. CS-AKI was defined based on the Acute Kidney Injury Network (AKIN) definition. We used regression modeling and survival analysis (adjusting for covariates) to evaluate associations. RESULTS: CS-AKI occurred in 64% of the neonates in our study cohort. Lower age, longer cardiopulmonary bypass time, hypothermic circulatory arrest, type of repair, lower preoperative serum creatinine (SCr) level, lower gestational age, and preoperative ventilation were independent risk factors for developing CS-AKI. Neonates with CS-AKI had longer times to extubation, intensive care discharge, and hospital discharge, after adjusting for covariates. Mortality was significantly increased in neonates with AKIN stage 2 or higher CS-AKI. The neonates with CS-AKI had a lower z-score for height at 2-year follow-up and were seen by more specialists. CONCLUSION: Neonatal CS-AKI is common and independently predicts important clinical outcomes, including mortality. Many risk factors are similar to those in older children, but some are unique to neonates. The observation that lower baseline SCr predicts CS-AKI merits further study. The AKIN definition, based on preoperative SCr value, is a reasonable method for defining CS-AKI in neonates. Many previous studies of CS-AKI have excluded neonates; we suggest that future intervention studies on approaches to reducing CS-AKI incidence and improving outcomes should include neonates.

Bilateral sensory permanent hearing loss after palliative hypoplastic left heart syndrome operation.

BACKGROUND: Bilateral sensory permanent hearing loss (PHL) has been reported after neonatal respiratory failure but has rarely been noted in survivors after cardiac operations. We report the prevalence and severity of PHL after Norwood right ventricular-pulmonary artery shunt for hypoplastic left heart syndrome (HLHS), document progressive loss, and explore markers of acute illness and ototoxic medications for PHL. METHODS: This interprovincial longitudinal outcome study after neonatal complex cardiac operations at Stollery Children's Hospital, Edmonton, Alberta, Canada, 2002 to 2007, completed repeated diagnostic audiologic assessments for all survivors by registered pediatric-experienced audiologists. Demographic, surgical, and perisurgical variables, including ototoxic medications, were collected. The association of potentially predictive variables with PHL and its severity were determined by univariate analysis and multiple logistic and linear regression analysis. RESULTS: At an age older than 3.5 years, progressive PHL was present in 12 of 42 survivors (28.6%, 95% confidence interval, 16.2% to 44.8%; mortality, 20.9%). Overall lowest partial pressure of arterial oxygen (odds ratio, 1.315; 95% confidence interval, 1.051 to 1.506), and cumulative dose of furosemide given as bolus (odds ratio, 1.062; 95% confidence interval, 1.018 to 1.109) combined to predict PHL and gave 39% of the variance of PHL severity. Antibiotics and neuromuscular blockers were not associated with PHL. CONCLUSIONS: Monitoring outcomes of neonates after HLHS surgery revealed unexpected PHL associated with hypoxia and bolus administration of furosemide. As survival improves, close follow-up is necessary to identify outcomes and seek modifiable predictive variables. Changes in the mode of furosemide administration may prevent this complication.