From vision to implementation: Building a national undergraduate paediatric curriculum.

Objective: There are many challenges in ensuring medical students learn paediatrics. Medical educators must develop and maintain curricula that meet learners' needs and accreditation requirements. Paediatricians and family physicians, practicing and teaching in busy clinical environments, require Canadian-relevant curricular guidance and resources to teach and assess learners. Students struggle with curricular cohesion, clear expectations, and resources. Recognizing these challenges and acknowledging the need to address them, the Paediatric Undergraduate Program Directors of Canada (PUPDOC) created canuc-paeds, a comprehensive competency-based undergraduate curriculum that teachers and students would actually use. Methods: Curriculum development included the following: utilization of best practices in curriculum development, an environmental scan, development of guiding principles, Delphi surveys, in-person meetings, and quality improvement. All Canadian paediatric undergraduate educator leaders and other stakeholders were invited to participate. Results: The curriculum, based on the RCPSC CanMEDS Framework, includes 29 clinical presentations, each with key conditions, foundational knowledge objectives, and learning resources. Essential paediatric-specific physical examination and procedural skills that graduating medical students are expected to perform are identified. Objectives specific to Intrinsic Roles of Collaborator, Communicator, Professional, Leader, Health Advocate and Scholar that can be assessed in the field of paediatrics at the undergraduate level are articulated. The national curriculum has been implemented widely at Canadian medical schools. Online, open-access clinical resources have been developed and are being used world-wide. Conclusion: This curriculum provides overarching Canadian-specific curricular guidance and resources for students and for the paediatricians and family physicians who are responsible for teaching and assessing undergraduate learners.

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.

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.

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.

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.

The registry and follow-up of complex pediatric therapies program of Western Canada: a mechanism for service, audit, and research after life-saving therapies for young children.

Newly emerging health technologies are being developed to care for children with complex cardiac defects. Neurodevelopmental and childhood school-related outcomes are of great interest to parents of children receiving this care, care providers, and healthcare administrators. Since the 1970s, neonatal follow-up clinics have provided service, audit, and research for preterm infants as care for these at-risk children evolved. We have chosen to present for this issue the mechanism for longitudinal follow-up of survivors that we have developed for western Canada patterned after neonatal follow-up. Our program provides registration for young children receiving complex cardiac surgery, heart transplantation, ventricular assist device support, and extracorporeal life support among others. The program includes multidisciplinary assessments with appropriate neurodevelopmental intervention, active quality improvement evaluations, and outcomes research. Through this mechanism, consistently high (96%) follow-up over two years is maintained.

Five-year neurocognitive and health outcomes after the neonatal arterial switch operation.

OBJECTIVES: We sought to assess the 5-year neurocognition and health of an interprovincial inception cohort undergoing the arterial switch operation for transposition of the great arteries. METHODS: Sixty-nine consecutive neonates had operations from 1996-2003 with full-flow cardiopulmonary bypass and selective deep hypothermic circulatory arrest. Outcomes were recorded at 58 +/- 9 months of age. Univariate and multivariate analyses were used to identify outcome predictors, including surgical subtype and preoperative, operative, and postoperative variables. RESULTS: There was 1 (1.5%) operative death. Two children were lost to follow-up, and 1 was excluded because of postdischarge meningitis. Outcomes are reported for 65 survivors. Two (3%) children have cerebral palsy, and 7 (11%) have language disorders, 4 of whom also meet the criteria for autism spectrum disorder. Two of the 4 children with autism have an affected older sibling. Of the 61 children without autism, scores approach those of peers, with a full-scale intelligence quotient of 97 +/- 16, a verbal intelligence quotient of 97 +/- 18, a performance intelligence quotient of 96 +/- 15, and a visual-motor integration score of 95 +/- 16. Mother's education, birth gestation or weight, and postoperative plasma lactate values account for 21% to 32% of the variance of these scores. Septostomy adds 7% to the variance of visual-motor integration scores. CONCLUSIONS: Most preschool children do well after surgical correction for transposition of the great arteries, including complex forms. Potentially modifiable variables include high preoperative plasma lactate levels and septostomy. A minority of children were given diagnoses of language disorders, including autism, in which familial factors likely contribute to outcome.

Neurocognitive, functional, and health outcomes at 5 years of age for children after complex cardiac surgery at 6 weeks of age or younger.

OBJECTIVE: This work provides neurocognitive, functional, and health outcomes for 5-year survivors of early infant complex cardiac surgery, including those with chromosomal abnormalities. PATIENTS AND METHODS: Of 85 children (22.4% mortality), 61 received multidisciplinary, individual evaluation and parental questionnaires at 5 years. Full-scale, verbal, and performance IQ scores were compared by using analysis of variance among children who received different surgeries (arterial switch, 20; Norwood for hypoplastic left heart syndrome, 14; simple total anomalous pulmonary venous connection, 6; miscellaneous, 21; and chromosomal abnormalities, 8). Predictions from mental scores at 2 years for IQ scores at 5 years were determined. RESULTS: Children with chromosomal abnormalities had lower full-scale and verbal IQs at 5 years than other survivors, with no differences found among the remaining groups. For children post-Norwood, performance IQ scores remained lower than for children after the arterial-switch operation. Prediction of full-scale IQ (<70) from 2-year mental scores for all 61 children were as follows: sensitivity, 87.5%; specificity, 88.1%; positive predictive value, 53.8%; and negative predictive value, 97.9%. For full-scale IQ of <85, predictions were 90.0%, 87.8%, 78.3%, and 94.7%, respectively. For those 53 without chromosomal abnormalities, full-scale IQ <70, respective predictions were 86.7%, 90.0%, 28.6%, and 97.8%, and for full-scale IQ <85, respective predictions were 85.7%, 89.7%, 75.0%, and 94.6%. Parental report indicated good health in 80% and adequate function in 67% to 88% of the children, although health-utilization numbers suggest that these reports are optimistic. CONCLUSIONS: Five-year full-scale and verbal IQs were similar among groups, excluding those with chromosomal abnormalities. Children with chromosomal abnormalities had the lowest scores. Excluding those with chromosomal abnormalities, the mean mental scores for the children as a group tended to increase from 2 to 5 years of age, with an overall high percentage of correct classifications at 2 years.