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

Robertson, Charlene M.T.; Sauvé, Reg; Joffe, Ari R.; Alton, Gwen Y.; Moddemann, Diane; Blakley, Patricia M.; Synnes, Anne; Dinu, Irina; Harder, James A.; Soni, Reeni; Bodani, Jaya; Kakadekar, Ashok; Dyck, John D.; Human, Derek G.; Ross, David B.; Rebeyka, Ivan M.

doi:10.4061/2011/965740

Cited by 57 publications

(76 citation statements)

References 48 publications

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“…12 Other specific pre-ECLS, ECLS, and post-ECLS variables were collected from the medical chart retrospectively (Table SI in the Data Supplement). Our ECLS program's policy is to maintain platelets >100 000 for infants <3 months and >80 000 for infants ≥3 months.…”

Section: Methodsmentioning

confidence: 99%

“…Physical measurements were obtained as has been described. 12 The family socioeconomic status (SES) was determined by the Blishen Index, 13 a formula considering the relative income, education level, and prestige factor of employment with a population mean and SD of 43 (13). Maternal education was indicated by years of schooling.…”

Section: Methodsmentioning

confidence: 99%

“…Hearing was evaluated by experienced certified pediatric audiologists in soundproof environments, as has been described. 12 Permanent hearing impairment was defined as bilateral loss of >25 dB hearing level at any frequency from 250 to 4000 Hz. Disability includes both motor and sensory disability.…”

Section: Methodsmentioning

confidence: 99%

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Survival and Neurocognitive Outcomes After Cardiac Extracorporeal Life Support in Children Less Than 5 Years of Age

Ryerson

Guerra

Joffe

et al. 2015

Circ: Heart Failure

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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.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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Survival and Neurocognitive Outcomes After Cardiac Extracorporeal Life Support in Children Less Than 5 Years of Age

Ryerson

Guerra

Joffe

et al. 2015

Circ: Heart Failure

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“…16 Infants were identified at the time of first CCS and followed prospectively. At the time of CCS, predetermined demographic, preoperative, intraoperative, and postoperative variables were collected.…”

Section: Methodsmentioning

confidence: 99%

“…16 Each child was seen by a developmental pediatrician; if CND was suspected, a pediatric neurologist confirmed diagnoses. The CND nature, typology (spastic, dyskinetic, ataxic, hypotonic), anatomic distribution (unilateralbilateral) of the motor disorder 17,18 ; functional motor abilities, (including oromotor involvement); accompanying impairments (intellectual disability; communication, vision, hearing impairments; epilepsy; autism spectrum disorder); and neuroimaging findings were recorded.…”

Section: Childhood Clinical Assessmentsmentioning

confidence: 99%

Chronic Neuromotor Disability After Complex Cardiac Surgery in Early Life

Ricci¹,

Andersen

Joffe

et al. 2015

Pediatrics

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BACKGROUND AND OBJECTIVES: Little is known about chronic neuromotor disability (CND) including cerebral palsy and motor impairments after acquired brain injury in children surviving early complex cardiac surgery (CCS). We sought to determine the frequency and presentation of CND in this population while exploring potentially modifiable acute care predictors.METHODS: This prospective follow-up study included 549 children after CCS requiring cardiopulmonary bypass at #6 weeks of age. Groups included those with only 1 CCS, mostly biventricular CHD, and those with .1 CCS, predominantly single ventricle defects. At 4.5 years of age, 420 (94.6%) children received multidisciplinary assessment. Frequency of CND is given as percentage of assessed survivors. Predictors of CND were analyzed using multiple logistic regression analysis.RESULTS: CND occurred in 6% (95% confidence interval [CI] 3.7%-8.2%) of 4.5-year survivors; for 1 CCS, 4.2% (CI 2.3%-6.1%) and .1, 9.8% (CI 7%-12.6%). CND presentation showed: hemiparesis, 72%; spasticity, 80%; ambulation, 72%; intellectual disability, 44%; autism, 16%; epilepsy, 12%; permanent vision and hearing impairment, 12% and 8%, respectively. Overall, 32% of presumed causative events happened before first CCS. Independent odds ratio for CND are age (days) at first CCS, 1.08 (CI 1.04-1.12; P , .001); highest plasma lactate before first CCS (mmol/L), 1.13 (CI 1.03-1.23; P = 0.008); and .1 CCS, 3.57 (CI 1.48-8.9; P = .005).CONCLUSIONS: CND is not uncommon among CCS survivors. The frequency of associated disabilities characterized in this study informs pediatricians caring for this vulnerable population. Shortening the waiting period and reducing preoperative plasma lactate levels at first CCS may assist in reducing the frequency of CND. WHAT'S KNOWN ON THIS SUBJECT:Neurodevelopmental outcomes after cardiac surgery in early life provide critical information for understanding and improving care. Studies show these children are at risk for arterial ischemic stroke and acquired brain injury; further characterization of motor impairment is needed. WHAT THIS STUDY ADDS:This study focuses on the presence of chronic neuromotor disabilities including cerebral palsy and motor impairments after acquired brain injury in children surviving early complex cardiac surgery, providing information on the frequency, characteristics, and predictors that may assist in prevention.

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Pre‐school neurocognitive and functional outcomes after liver transplant in children with early onset urea cycle disorders, maple syrup urine disease, and propionic acidemia: An inception cohort matched‐comparison study

et al. 2020

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Background Urea cycle disorders (UCD) and organic acid disorders classically present in the neonatal period. In those who survive, developmental delay is common with continued risk of regression. Liver transplantation improves the biochemical abnormality and patient survival is good. We report the neurocognitive and functional outcomes post‐transplant for nine UCD, three maple syrup urine disease, and one propionic acidemia patient. Methods Thirteen inborn errors of metabolism (IEM) patients were individually one‐to‐two matched to 26 non‐IEM patients. All patients received liver transplant. Wilcoxon rank sum test was used to compare full‐scale intelligence‐quotient (FSIQ) and Adaptive Behavior Assessment System‐II General Adaptive Composite (GAC) at age 4.5 years. Dichotomous outcomes were reported as percentages. Results FSIQ and GAC median [IQR] was 75 [54, 82.5] and 62.0 [47.5, 83] in IEM compared with 94.5 [79.8, 103.5] and 88.0 [74.3, 97.5] in matched patients (P‐value <.001), respectively. Of IEM patients, 6 (46%) had intellectual disability (FSIQ and GAC <70), 5 (39%) had autism spectrum disorder, and 1/13 (8%) had cerebral palsy, compared to 1/26 (4%), 0, 0, and 0% of matched patients, respectively. In the subgroup of nine with UCDs, FSIQ (64[54, 79]), and GAC (56[45, 75]) were lower than matched patients (100.5 [98.5, 101] and 95 [86.5, 99.5]), P = .005 and .003, respectively. Conclusion This study evaluated FSIQ and GAC at age 4.5 years through a case‐comparison between IEM and matched non‐IEM patients post‐liver transplantation. The neurocognitive and functional outcomes remained poor in IEM patients, particularly in UCD. This information should be included when counselling parents regarding post‐transplant outcome.

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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

Cited by 57 publications

References 48 publications

Survival and Neurocognitive Outcomes After Cardiac Extracorporeal Life Support in Children Less Than 5 Years of Age

Survival and Neurocognitive Outcomes After Cardiac Extracorporeal Life Support in Children Less Than 5 Years of Age

Chronic Neuromotor Disability After Complex Cardiac Surgery in Early Life

Pre‐school neurocognitive and functional outcomes after liver transplant in children with early onset urea cycle disorders, maple syrup urine disease, and propionic acidemia: An inception cohort matched‐comparison study

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