The Alberta Infant Motor Scale as an Outcomes Measure of Gross Motor Abilities after Early Complex Cardiac Surgery.

To address the research hypothesis that the Alberta Infant Motor Scale (AIMS) completed following complex cardiac surgery (CCS) is a useful outcomes measure this study determined: (1) AIMS scores at age 8 months after CCS; (2) predictive validity of AIMS at 8 months for Bayley Scales of Infant and Toddler Development-III Gross Motor-scaled scores (GMSS) and diagnosis of cerebral palsy (CP) at 21 months; and (3) predictive demographic and surgical variables of AIMS scores. A prospective cohort study of 250/271 (92.3%) surviving children from Northern Alberta (born 2009-2020) who had CCS at age < 6 months determined AIMS scores at age mean (SD) 8.6 (2.4) and the GMSS at 21.9 (3.8) months. Gross motor delay was defined as AIMS < 5th percentile and GMSS as < 4 (-2SD). Predictions using multiple logistic regressions were expressed as Odds Ratios (OR) and 95% Confidence Interval (CI). Of children, 100/250 (40%) had AIMS < 5th predicting GMSS < 4 (n = 43); sensitivity, specificity, positive, and negative predictive values were 88%, 71%, 40%, and 97%. Hospitalization days were independently associated with AIMS < 5th, OR 1.02 (95% CI 1.007, 1.032; p = 0.005). Excluding hospital days, ventilation days independently predicted AIMS < 5th, OR 1.08 (95% CI 1.038, 1.125, p < 0.001. Gross motor delay determine by AIMS scores of < 5th percentile occurred in 40% of survivors with good prediction of continued delay. Delay determined by AIMS was predicted by longer hospitalization and ventilation; further investigations about the causes are required. AIMS results provide opportunity for early motor intervention.

Differences in gross motor and fine motor outcomes for toddlers after early complex cardiac surgery.

OBJECTIVES: To determine whether gross motor scores of toddlers after complex cardiac surgery were different from fine motor scores and were adequately represented by motor composite scores and, whether acute care predictors and chronic childhood health markers of gross motor scores differed from those of fine motor. METHODS: This prospective inception-cohort outcomes study included 171 toddlers after complex cardiac surgery with cardiopulmonary bypass at age <6 months, born in Northern Alberta from 2009 to 2019, and without known chromosomal abnormalities. At a mean (standard deviation) age of 21.7 (3.7) months, the Bayley Scales of Infant and Toddler Development-III determined motor composite and scaled scores (normative values, 100 (15), 10 (3), respectively). The same variables from surgery and assessment were analysed using multivariate regression to predict gross and fine motor scores; results expressed as effect size (95% confidence interval) with % variance. RESULTS: Composite, fine, and gross motor scores were 89.7 (14.2), 9.4 (2.5), and 7.2 (2.7), respectively. Predictive variables accounted for 21.2% of the variance for fine motor, and 36.9% for gross motor. Multivariate analysis for gross motor scores included toddlers need for cardiac medication, effect size (95% confidence interval) -0.801 (-1.62, -0.02), gastrostomy, -1.35 (-2.39, -0.319), and single ventricle, -0.93 (-1.71, -0.15). These same variables did not predict fine motor scores. CONCLUSION: Gross motor skills commonly were lower than fine motor skills for toddlers after complex cardiac surgery. Predictors for gross motor scores differed from fine motor scores. Separate reporting of gross motor scores could lead to improved identification of predictors of delay and to optimised early intervention.

Neurodevelopmental Outcomes of Infants after Neonatal Surgical Intervention for Tetralogy of Fallot or Pulmonary Atresia.

OBJECTIVE: To determine the 2-year neurodevelopmental outcomes for survivors of neonatal cardiac surgery for the most common right ventricular outflow tract obstructive lesions: tetralogy of Fallot and pulmonary atresia with a ventricular septal defect. STUDY DESIGN: A single-center consecutive cohort of 77 children underwent neonatal surgery for tetralogy of Fallot or pulmonary atresia with a ventricular septal defect at ≤6 weeks of age between 2006 and 2017. The patients underwent a multidisciplinary neurodevelopmental assessment at 18-24 months of age. Survivor outcomes were compared by univariable and multivariable analyses. RESULTS: The 2-year mortality was 7.8% (6/77) with a postoperative in-hospital mortality of 3.9% (3/77). Freedom from reintervention by cardiac catheterization or surgical intervention at 2 years was 36%. Functional and neurodevelopmental assessment for 69 of 71 survivors was completed at a mean age of 22.6 ± 4.0 months using the Bayley Scales of Infant and Toddler Development III. The mean neurodevelopmental outcome scores were 83.4 ± 16.5 for cognitive skills, 82.2 ± 18.7 for language skills, and 81.4 ± 18.1 for motor skills. Cognitive, language, and motor delay, defined as a score of <70, was identified in 25%, 25%, and 23% of patients, respectively. Multivariable analyses for factors associated with worse neurodevelopmental outcomes identified chromosomal anomalies (P < .001) and postoperative complications (P < .03). CONCLUSIONS: Cyanotic tetralogy of Fallot and pulmonary atresia with ventricular septal defect requiring neonatal repair showed similar 2-year neurodevelopmental outcomes below normative values and a high prevalence of cognitive, language and motor delays.

Third Trimester Umbilical Arterial Pulsatility Index is Associated with Neurodevelopmental Outcomes at 2-Years in Major Congenital Heart Disease.

Major congenital heart disease (CHD) is associated with impaired neurodevelopment (ND), partly from prenatal insults. In this study we explore associations between 2nd and 3rd trimester umbilical (UA) and middle cerebral artery (MCA) pulsatility index (PI = systolic-diastolic velocities/mean velocity) in fetuses with major CHD and 2-year ND and growth outcomes. Eligible patients included those with a prenatal diagnosis of CHD from 2007 to 2017 without a genetic syndrome who underwent previously defined cardiac surgeries and 2-year biometric and ND assessments in our program. UA and MCA-PI Z-scores at fetal echocardiography were examined for relationships with 2-year Bayley Scales of Infant and Toddler Development and biometric Z-scores. Data from 147 children was analyzed. Second and 3rd trimester fetal echocardiograms were performed at 22.4 ± 3.7 and 34.7 ± 2.9 weeks (mean ± SD), respectively. Multivariable regression analysis showed an inverse relationship between 3rd trimester UA-PI for all CHD and cognitive - 1.98 (- 3.37, - 0.59), motor - 2.57 (- 4.15, - 0.99), and language - 1.67 (- 3.3, - 0.03) (effect size and 95th confidence interval) ND domains (p < 0.05), with the strongest relationships in the single ventricle and hypoplastic left heart syndrome subgroups. No association was found for 2nd trimester UA-PI or any trimester MCA-PI and ND or between UA or MCA-PI and 2-year growth parameters. Increased 3rd trimester UA-PI, reflecting an altered late gestation fetoplacental circulation, relates to worse 2-year ND in all domains.

A complicated Glenn procedure: risk factors and association with adverse long-term neurodevelopmental and functional outcomes.

OBJECTIVES: To determine potentially modifiable risk factors for a complicated Glenn procedure (cGP) and whether a cGP predicted adverse neurodevelopmental and functional outcomes. A cGP was defined as post-operative death, heart transplant, extracorporeal life support, Glenn takedown, or prolonged ventilation. METHODS: All 169 patients having a Glenn procedure from 2012 to 2017 were included. Neurodevelopmental assessments were performed at age 2 years in consenting survivors (n = 156/159 survivors). The Bayley Scales of Infant and Toddler Development-3rd Edition (Bayley-III) and the Adaptive Behavior Assessment System-2nd Edition (ABAS-II) were administered. Adaptive functional outcomes were determined by the General Adaptive Composite (GAC) score from the ABAS-II. Predictors of outcomes were determined using univariate and multiple variable linear or Cox regressions. RESULTS: Of patients who had a Glenn procedure, 10/169 (6%) died by 2 years of age and 27/169 (16%) had a cGP. Variables statistically significantly associated with a cGP were the inotrope score on post-operative day 1 (HR 1.04, 95%CI 1.01, 1.06; p = 0.010) and use of inhaled nitric oxide post-operatively (HR 7.31, 95%CI 3.19, 16.76; p < 0.001). A cGP was independently statistically significantly associated with adverse Bayley-III Cognitive (ES -10.60, 95%CI -17.09, -4.11; p = 0.002) and Language (ES -11.43, 95%CI -19.25, -3.60; p = 0.004) scores and adverse GAC score (ES -14.89, 95%CI -22.86, -6.92; p < 0.001). CONCLUSIONS: Higher inotrope score and inhaled nitric oxide used post-operatively were associated with a cGP. A cGP was independently associated with adverse 2-year neurodevelopmental and functional outcomes. Whether early recognition and intervention for risk of a cGP can prevent adverse outcomes warrants study.

Comparison of motor outcomes between preschool children with univentricular and biventricular critical heart disease not diagnosed with cerebral palsy or acquired brain injury.

This comparison study of two groups within an inception cohort aimed to compare the frequency of motor impairment between preschool children with univentricular and biventricular critical congenital heart disease (CHD) not diagnosed with cerebral palsy/acquired brain injury, describe and compare their motor profiles and explore predictors of motor impairment in each group.Children with an intellectual quotient <70 or cerebral palsy/acquired brain injury were excluded. Motor skills were assessed with the Movement Assessment Battery for Children-2. Total scores <5th percentile indicated motor impairment. Statistical analysis included χ2 test and multiple logistic regression analysis.At a mean age of 55.4 (standard deviation 3.77) months, motor impairment was present in 11.8% of those with biventricular critical CHD, and 32.4% (p < 0.001) of those with univentricular critical CHD. The greatest difference between children with biventricular and univentricular CHD was seen in total test scores 8.73(2.9) versus 6.44(2.8) (p < 0.01) and in balance skills, 8.84 (2.8) versus 6.97 (2.5) (p = 0.001). Manual dexterity mean scores of children with univentricular CHD were significantly below the general population mean (>than one standard deviation). Independent odds ratio for motor impairment in children with biventricular critical CHD was presence of chromosomal abnormality, odds ratio 10.9 (CI 2.13-55.8) (p = 0.004); and in children with univentricular critical CHD odds ratio were: postoperative day 1-5 highest lactate (mmol/L), OR: 1.65 (C1.04-2.62) (p = 0.034), and dialysis requirement any time before the 4.5-year-old assessment, OR: 7.8 (CI 1.08-56.5) (p = 0.042).Early assessment of motor skills, particularly balance and manual dexterity, allows for intervention and supports that can address challenges during the school years.

Health-related quality of life after pediatric heart transplantation in early childhood.

BACKGROUND: There is limited information about HRQL after pediatric heart transplantation at a young age. METHODS: Prospective follow-up study of children who received a heart transplant at age ≤4 years. HRQL was assessed using the PedsQLTM 4.0 at age 4.5 years. This cohort was compared with healthy children, children with CHD, and with chronic conditions. Peri-operative factors associated with HRQL were also explored. RESULTS: Of 66 eligible patients, 15 (23%) died prior to the HRQL assessment and 2 (3%) were lost to follow-up, leaving 49 patients. Indication for transplantation was CHD in 27 (55%) and CMP in 22 (45%). Median age (IQR) at transplant was 9 (5-31) months. HRQL was significantly lower in transplanted children compared to population norms (65.3 vs 87.3, P < .0001), children with chronic conditions (65.3 vs 76.1, P = .001), and children with CHD (65.3 vs 81.1, P < .0001). Transplanted children with CHD had lower HRQL than those with a prior diagnosis of CMP (59.5 vs 72.5, P-value = .020). Higher creatinine pretransplant and higher lactate post-operatively were associated with lower HRQL. CONCLUSION: Children after heart transplant had significantly lower HRQL, as reported by their parents, than the normative population, children with chronic conditions, and children with CHD.

Kindergarten-age neurocognitive, functional, and quality-of-life outcomes after liver transplantation at under 6 years of age.

BACKGROUND: We aimed to describe school-entry age neurocognitive, functional, and HRQL outcomes and their predictors after liver transplant done at age <6 years. METHODS: A prospective cohort of all (n = 69) children surviving liver transplant from 1999 to 2014 were assessed at age 55.4 (SD 7.2) months and 38.6 (12.4) months after transplant. Assessment included: the Wechsler Preschool and Primary Scales of Intelligence, Beery-Buktenica Developmental Test of VMI, Adaptive Behavior Assessment System caregiver-completed questionnaire, and PedsQL 4.0 Generic Core Scales. Univariate and multiple linear regression determined predictors of outcomes at P < .05. RESULTS: Neurocognitive and functional outcomes were on average within 1 SD of population norms, although shifted to the left (P ≤ .03), with more patients than expected having scores >2 (3.7-5.9 times more, P ≤ .007) SD below population norms. Total and Summary HRQL scores were statistically significantly lower than the healthy normative population (P ≤ .02) and a congenital heart disease group (P ≤ .02), but similar to children with other chronic health conditions; differences often exceeded the MCID and were lowest in the School functioning domain. There were few predictors on multiple linear regressions, and we could not confirm previous studies that suggested various inconsistent predictors of outcomes. Neurocognitive and functional outcomes scores were highly correlated with HRQL scores except for the School functioning domain, but did not fully explain them. CONCLUSIONS: Long-term follow-up of this vulnerable population is important in order to facilitate support for the patient and family, and early intervention for any difficulties identified.

Neurocognitive and functional outcomes at 5 years of age after renal transplant in early childhood.

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.

Avoiding Furosemide Ototoxicity Associated With Single-Ventricle Repair in Young Infants.

OBJECTIVE: To reduce bilateral delayed-onset progressive sensory permanent hearing loss using a systems-wide quality improvement project with adherence to best practice for the administration of furosemide. DESIGN: Prospective cohort study with regular audiologic follow-up assessment of survivors both before and after a 2007-2008 quality improvement practice change. SETTING: The referral center in Western Canada for complex cardiac surgery, with comprehensive multidisciplinary follow-up by the Complex Pediatric Therapies Follow-up Program. PATIENTS: All consecutive patients having single-ventricle palliative cardiac surgery at age 6 weeks old or younger. INTERVENTIONS: A 2007-2008 quality improvement practice change consisted of a Parenteral Drug Monograph revision indicating slow IV administration of furosemide, an educational program, and an evaluation. MEASUREMENTS AND MAIN RESULTS: The outcome measure was the prevalence of permanent hearing loss by 4 years old. Firth multiple logistic regression compared pre (1996-2008) to post (2008-2012) practice change occurrence of permanent hearing loss, adjusting for confounding variables, including all hospital days, extracorporeal membrane oxygenation, cardiopulmonary bypass time, age at first surgery, dialysis, and sepsis. From 1996 to 2012, 259 infants had single-ventricle palliative surgery at age 6 weeks old or younger, with 173 (64%) surviving to age 4 years. Of survivors, 106 (61%) were male, age at surgery was 11.6 days (9.0 d), and total hospitalization days by age 4 years were 64 (42); 18 (10%) had cardiopulmonary resuscitation and 38 (22%) had sepsis at any time. All 173 (100%) had 4-year follow-up. Pre- to postpractice change permanent hearing loss dropped from 17/100 (17%) to 0/73 (0%) of survivors. On Firth multiple logistic regression, the only variable statistically associated with permanent hearing loss was the pre- to postpractice change time period (odds ratio, 0.03; 95% CI, 0-0.35; p = 0.001). CONCLUSIONS: A practice change to ensure slow IV administration of furosemide eliminated permanent hearing loss. Centers caring for critically ill infants, particularly those with single-ventricle anatomy or hypoxia, should review their drug administration guidelines and adhere to best practice for administration of IV furosemide.

Prevalence of Childhood Permanent Hearing Loss after Early Complex Cardiac Surgery.

OBJECTIVES: To estimate the prevalence of childhood permanent hearing loss (PHL) after early cardiac surgery. STUDY DESIGN: This prospective observational (1996-2015) study after complex cardiac surgery with cardiopulmonary bypass at ≤6 weeks of life reports audiology follow-up by registered pediatric-experienced audiologists at 6-8 months postsurgery, age 2 years, and as required throughout and thereafter to complete diagnoses. PHL at any frequency (500-4000 Hz) is defined as responses of >25-decibel hearing level in either ear. PHL was evaluated by type (conductive or sensorineural), pattern (flat or sloping), and severity (mild to profound). RESULTS: Survival rate was 83.4% (706 of 841 children) with a 97.9% follow-up rate (691 children); 41 children had PHL, 5.9% (95% CI 4.3%, 8.0%). By cardiac defect, prevalence was biventricular, 4.0% (95%CI 2.5%, 6.1%); single ventricle, 10.8% (95%CI 6.8%, 16.1%). Eighty-seven (12.6%) of 691 had syndromes/genetic abnormalities with known association with PHL; of these, 17 (41.5%) had PHL. Of 41 children, 4 had permanent conductive, moderate to severe loss (1 bilateral); 37 had moderate to profound sensorineural loss (29 bilateral with 20 sloping and 9 flat), 6 with cochlear implant done or recommended. CONCLUSIONS: Infants surviving complex cardiac surgery are at high risk for PHL. Over 40% with PHL have known syndromes/genetic abnormalities, but others do not have easily identifiable risk indicators. Early cardiac surgery should be considered a risk indicator for PHL.

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.

Prevalence Estimate of Cerebral Palsy in Northern Alberta: Births, 2008-2010.

OBJECTIVES: The objectives of this study were to determine prevalence estimates of cerebral palsy (CP) among 5-year-old children in northern Alberta; to provide congenital, gestational age- and birth weight-specific, and postneonatal CP rates; and to describe motor subtypes and function. METHODS: This population-based prevalence estimate study, part of the Canadian Cerebral Palsy Registry, reports confirmed CP diagnoses at age 5 years made by pediatric rehabilitation and child neurology specialists. Prevalence rates with 95% confidence intervals (CIs) used Alberta government denominators of same-age children and live births. RESULTS: The Northern Alberta CP rate (birth years, 2008-2010) for 173 5-year-old children is 2.22 (95% CI 2.12, 2.32) per 1000 5-year-old children. The congenital CP rate is 1.99 (95% CI, 1.89-2.09) per 1000 live births; unilateral congenital CP, 1.0 (95% CI, 0.64-1.36) per 1000 live births; and postneonatal CP, 0.12 (95% CI, 0.1-0.14) per 1000 live births. Gestational age-specific rates are similar: age <28 weeks, 27.2 (95% CI, 23.05-31.35) and 28 to 31 weeks, 29.5 (95% CI, 25.78-33.22). Motor subtypes for 169 children (data missing, 4; male, 97; postnatal, 9) are: spastic, 148 (87.6%) including 31 (20.9%) with diplegia, 10 (6.8%) triplegia, 33 (22.2%) quadriplegia, 74 (50%) hemiplegia/monoplegia); and dyskinetic, 18 (10.6%) and ataxic, 3 (1.8%). A total of 107 (63.3%) ambulate without assistive devices and 111(65.7%) handle most objects with their hands independently. CONCLUSIONS: This is the fourth Canadian CP prevalence study; one from Quebec used a similar case ascertainment approach and two 1980s studies from Alberta and British Columbia used administrative databases. Northern Alberta CP rates are comparable with other developed countries. The hemiplegic subtype is the most common. Rates among preterm children have declined but are similar for the <28 and 28 to 31 gestation-week groups.

Pilot randomized controlled trial on early and late remote ischemic preconditioning prior to complex cardiac surgery in young infants.

BACKGROUND: Remote ischemic preconditioning involves providing a brief ischemia-reperfusion event to a tissue to create subsequent protection from a more severe ischemia-reperfusion event to a different tissue/organ. The few pediatric remote ischemic preconditioning studies in the literature show conflicting results. AIM: We conducted a pilot randomized controlled trial to determine the feasibility of conducting a larger trial and to gather provisional data on the effect of early and late remote ischemic preconditioning on outcomes of infants after surgery for congenital heart disease. METHODS: This single-center, double-blind randomized controlled trial of remote ischemic preconditioning vs control (sham-remote ischemic preconditioning) in young infants going for surgery for congenital heart disease at the Stollery Children's Hospital. Remote ischemic preconditioning was performed at 24-48 h preoperatively and immediately prior to cardiopulmonary bypass. Remote ischemic preconditioning stimulus was performed with blood pressure cuffs around the thighs. Primary outcomes were feasibility and peak blood lactate level on day 1 postoperatively. RESULTS: Fifty-two patients were randomized but seven patients became ineligible after randomization leaving 45 patients included in the study. In the included patients, 7 (15%) had protocol deviations (five infants did not have the preoperative intervention and two did not receive the intervention in the operating room). From a comfort point of view, only one subject in the control group and two in the Remote ischemic preconditioning group received sedation during the preoperative intervention. There were no study-related adverse events and no complications to the limbs subjected to preconditioning. There were no significant differences between the Remote ischemic preconditioning group and the control group in the highest blood lactate level on day 1 postoperatively (mean difference, 1.28; 95%CI, -0.22, 2.78; P-value = 0.093). CONCLUSION: In infants who underwent surgery for congenital heart disease, our pilot randomized controlled trial on early and late remote ischemic preconditioning proved to be feasible but did not find any significant difference in acute outcomes. A larger trial may be necessary.

Hypoplastic Left Heart Syndrome is not Associated with Worse Clinical or Neurodevelopmental Outcomes Than Other Cardiac Pathologies After the Norwood-Sano Operation.

There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood-Sano operation between 2005 and 2014 were included. Follow-up clinical, neurological, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Developmental outcomes were assessed at 2 years of age using the Bayley Scales of Infant and Toddler Development (Bayley-III). Survival was assessed using Kaplan-Meier analysis. Baseline characteristics, survival, and neurodevelopmental outcomes were compared between those with HLHS and those with non-HLHS anatomy (non-HLHS). The study comprised 126 infants (75 male), 87 of whom had HLHS. Five-year survival was the same for subjects with HLHS and those with non-HLHS (HLHS 71.8%, non-HLHS 76.9%; p = 0.592). Ninety-three patients underwent neurodevelopmental assessment including Bayley-III scores. The overall mean cognitive composite score was 91.5 (SD 14.6), language score was 86.6 (SD 16.7) and overall mean motor composite score was 85.8 (SD 14.5); being lower than the American normative population mean score of 100 (SD 15) for each (p-value for each comparison, <0.0001). None of the cognitive, language, or motor scores differed between those with HLHS and non-HLHS (all p > 0.05). In the generalized linear models, dominant right ventricle anatomy (present in 117 (93%) of patients) was predictive of lower language and motor scores. Comparative analysis of the HLHS and non-HLHS groups undergoing single ventricle palliation including a Norwood-Sano, during the same era, showed comparable 2-year survival and neurodevelopmental outcomes.

Goal setting within a tertiary-level early developmental intervention program.

OBJECTIVES: To determine the domains of parent-therapist collaboratively set goals for intervention within a family-centered, tertiary-level early developmental intervention (EDI) program. To report changes in ratings for performance and satisfaction of performance pre- and postintervention for the set goals. METHODS: This study includes all children with complex developmental disabilities (30 ± 3 months of age, 61% boys) registered in a tertiary-level EDI program from 2009 to 2012 whose parents completed the Canadian Occupational Performance Measure (COPM) to evaluate goal setting. The International Classification of Functioning, Disability and Health: Children & Youth version (ICF-CY) domain for each goal was recorded. Pre- and postintervention ratings for individual and averaged goals per child were calculated. Positive changes in ratings were determined by an increase of ≤2 and presented as a proportion and as means (standard deviation). Student t test, Fisher Exact and chi-square tests compared groups. RESULTS: Of 483 individual goals set for 168 children, 65.4% were in the ICF-CY activity domain, 16.4% participation, 10.3% body function, 7.4% environment and 0.4% body structure. Positive changes in ratings for performance occurred in 70%; for satisfaction, 68%, with no difference in relation to domain. Positive changes in average ratings per child showed: performance, 67%; satisfaction, 66%. CONCLUSION: Intervention goals for disabled children within tertiary-level EDI are primarily those of activity, not participation as ICF-CY recommends; two-thirds of goals show positive change. These results suggest the need for further evaluation of goal setting within EDI programs with the expectation of increasing goals of participation and improving ratings for performance and satisfaction.

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.

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.

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.