Shared reading with infants: SharePR a novel measure of shared reading quality.

BACKGROUND: The AAP recommends "shared" reading from early infancy for healthy development. However, many families are uncertain how to read most enjoyably and effectively with infants, especially from underserved backgrounds. Shared reading quality (interactivity) moderates benefits yet is challenging to measure. SHARE/STEP is a new model of shared reading quality at this age incorporating evidence-based behaviors. OBJECTIVE: To test the SharePR parent-report measure of caregiver-infant reading quality. METHODS: This study involved mother-infant dyads in two unrelated trials in an obstetric (0-2 months old) and pediatric (6-9 months old) clinic. SharePR is a 10-item measure based on the SHARE/STEP model. Analyses involved descriptive statistics, measures of psychometric integrity, and correlations with home literacy environment (HLE). RESULTS: There were 99 dyads in the younger (1.2 + 0.5 months) and 108 dyads in the older groups (6.6 + 1.1 months). A majority were of non-white race (73%, 96%) and low-socioeconomic status (56%, 44% in-poverty). SharePR administration time was under 2 min and scores were normally distributed at each age. Psychometric properties were strong in terms of internal consistency and reliability. Scores were positively correlated with HLE for the older group (p < 0.05). CONCLUSIONS: SharePR may be an efficient tool to quantify shared reading quality with infants, warranting further investigation. CLINICAL TRIALS: Data for these analyses were collected via two unrelated trials led by the lead author (J.S.H.). For the younger cohort, this is registered on the ClinicalTrials.gov website, ID# NCT04031235. For the older cohort, this is registered on the ClinicalTrials.gov website, ID# 2017-6856. IMPACT: The AAP recommends caregiver-child ("shared") reading beginning in infancy, yet many families are uncertain how to do so. Verbal and social-emotional interactivity during shared reading ("quality") moderates benefits and is often low in families from disadvantaged backgrounds, yet is challenging to measure. SharePR is a 10-item parent-report measure of shared reading quality based on a novel conceptual model incorporating evidence-based behaviors (SHARE/STEP). SharePR exhibited promising psychometric properties in two separate samples of mothers of younger and older infants. SharePR is a potentially useful measure of shared reading quality at this formative age, for research and to frame early reading guidance.

Psychometric properties of the ScreenQ for measuring digital media use in Portuguese young children.

AIM: Digital media use is prevalent among children and linked to potential developmental and health risks, but validated measures of children's digital media use are lacking. The aim of this study was to validate the Portuguese version of the ScreenQ with three distinct children's age groups. METHODS: Parents of children living in Portugal completed an online survey including the 16-item version of the ScreenQ and items related to home activities and digital media use. A combination of classical and modern theory (Rasch) methods was used for analysis. RESULTS: A total of 549 mothers and 51 fathers of 325 girls and 322 boys from 6 months to 9 years and 11 months old responded to the survey. Point-measure correlations were all positive and endorsement of item values were within acceptable ranges. Cronbach's coefficient α was acceptable for a new measure, and test-retest reliability was high. Statistically significant correlations were found between ScreenQ total scores and relevant demographic, play-related, parenting and digital media use items. CONCLUSION: The Portuguese version of the ScreenQ exhibited sound psychometric properties, including internal consistency and concurrent validity referenced to external items. Higher ScreenQ scores were correlated with higher digital media multitasking, lower parent-child interaction, and higher concerns regarding child's learning and behaviour.

Chronic foetal hypoxaemia does not cause elevation of serum markers of brain injury.

OBJECTIVES: The objective of this study was to investigate changes in serum biomarkers of acute brain injury, including white matter and astrocyte injury during chronic foetal hypoxaemia. We have previously shown histopathological changes in myelination and neuronal density in fetuses with chronic foetal hypoxaemia at a level consistent with CHD. METHODS: Mid-gestation foetal sheep (110 ± 3 days gestation) were cannulated and attached to a pumpless, low-resistance oxygenator circuit, and incubated in a sterile fluid environment mimicking the intrauterine environment. Fetuses were maintained with an oxygen delivery of 20-25 ml/kg/min (normoxemia) or 14-16 ml/kg/min (hypoxaemia). Myelin Basic Protein and Glial Fibrillary Acidic Protein serum levels in the two groups were assessed by ELISA at baseline and at 7, 14, and 21 days of support. RESULTS: Based on overlapping 95% confidence intervals, there were no statistically significant differences in either Myelin Basic Protein or Glial Fibrillary Acidic Protein serum levels between the normoxemic and hypoxemic groups, at any time point. No statistically significant correlations were observed between oxygen delivery and levels of Myelin Basic Protein and Glial Fibrillary Acidic Protein. CONCLUSION: Chronic foetal hypoxaemia during mid-gestation is not associated with elevated serum levels of acute white matter (Myelin Basic Protein) or astrocyte injury (Glial Fibrillary Acidic Protein), in this model. In conjunction with our previously reported findings, our data support the hypothesis that the brain dysmaturity with impaired myelination found in fetuses with chronic hypoxaemia is caused by disruption of normal developmental pathways rather than by direct cellular injury.

Deep hypothermic circulatory arrest in cyanotic piglets is associated with increased neuronal necrosis.

BACKGROUND: The contribution of neonatal cyanosis, inherent to cyanotic congenital heart disease, to the magnitude of neurologic injury during deep hypothermic circulatory arrest has not been fully delineated. This study investigates the impact of cyanosis and deep hypothermic circulatory arrest on brain injury. METHODS: Neonatal piglets were randomised to placement of a pulmonary artery to left atrium shunt to create cyanosis or sham thoracotomy. At day 7, animals were randomised to undergo deep hypothermic circulatory arrest or sham. Arterial oxygen tension and haematocrit were obtained. Neurobehavioural performance was serially assessed. The animals were sacrificed on day 14. Brain tissue was assessed for neuronal necrosis using a 5-point histopathologic score. RESULTS: Four experimental groups were analysed (sham, n = 10; sham + deep hypothermic circulatory arrest, n = 8; shunt, n = 9; shunt + deep hypothermic circulatory arrest, n = 7). Cyanotic piglets had significantly higher haematocrit and lower partial pressure of oxygen at day 14 than non-cyanotic piglets. There were no statistically significant differences in neurobehavioural scores at day 1. However, shunt + deep hypothermic circulatory arrest piglets had evidence of greater neuronal injury than sham animals (median (range): 2 (0-4) versus 0 (0-0), p = 0.02). DISCUSSION: Cyanotic piglets undergoing deep hypothermic circulatory arrest had increased neuronal injury compared to sham animals. Significant injury was not seen for either cyanosis or deep hypothermic circulatory arrest alone relative to shams. These findings suggest an interaction between cyanosis and deep hypothermic circulatory arrest and may partially explain the suboptimal neurologic outcomes seen in children with cyanotic heart disease who undergo deep hypothermic circulatory arrest.

A novel, composite measure of screen-based media use in young children (ScreenQ) and associations with parenting practices and cognitive abilities.

BACKGROUND: Screen-based media use is prevalent in children and is associated with health risks. American Academy of Pediatrics (AAP) recommendations involve access to screens, frequency, content, and co-viewing. The aim of this study was to test the ScreenQ, a composite measure of screen-based media use. METHODS: ScreenQ is a 15-item parent report measure reflecting AAP recommendations. Range is 0-26, higher scores reflecting greater non-adherence. With no "gold standard" available, four validated measures of skills and parenting practices cited as influenced by overuse were applied as the external criteria, including expressive language, speed of processing, emergent literacy, and cognitive stimulation at home. Psychometric analyses involved Rasch methods and Spearman's ρ correlations. RESULTS: Sixty-nine families were administered ScreenQ. Child age ranged from 36 to 63 months old (52 ± 8; 35 girls). Mean ScreenQ score was 9.6 (±5.0; 1-22). Psychometric properties were strong (rCo-α = 0.74). ScreenQ scores were negatively correlated with CTOPP-2 (Comprehensive Test of Phonological Processing, Second Edition) (rρ = -0.57), EVT-2 (Expressive Vocabulary Test, Second Edition) (rρ = -0.45), GRTR (Get Ready to Read!) (rρ = -0.30) and StimQ-P (rρ = -0.42) scores (all p ≤ 0.01). CONCLUSIONS: ScreenQ shows potential as a composite measure of screen-based media use in young children in the context of AAP recommendations. ScreenQ scores were correlated with lower executive, language and literacy skills, and less stimulating home cognitive environment.

Changes in diet quality and home food environment in preschool children following weight management.

BACKGROUND: Family-based obesity treatment interventions can successfully reduce energy intake in preschoolers. An implicit goal of obesity treatment interventions is to improve diet quality, but diet quality has been less examined as a treatment outcome in studies of preschoolers. The purpose of this study was to conduct a secondary analysis comparing the change in diet quality and home food environment in preschoolers assigned to a behavioral family-based obesity intervention (LAUNCH), motivational interviewing (MI) condition, or standard care (STC) condition. METHODS: Three 24-h dietary recalls were completed at baseline and 6-months and were analyzed using NDS-R software; diet quality was assessed using the Healthy Eating Index-2010 (HEI-2010). Availability of foods and beverages in the home was assessed through direct observation using the Home Health Environment tool that classifies foods and beverages as 'red' or 'green' based upon fat and sugar content. Repeated measures linear mixed effects models were used to examine changes in diet quality and home food environment between conditions (LAUNCH, MI, STC). RESULTS: At 6-months, preschoolers in the LAUNCH condition had a higher HEI-2010 total score (62.8 ± 13.7) compared to preschoolers in the MI (54.7 ± 13.4, P = 0.022) and STC (55.8 ± 11.6, P = 0.046) conditions. Regarding the home food environment, families in LAUNCH had significantly less 'red' foods in their home at 6-months (12.5 ± 3.4 'red' foods) compared to families in MI (14.0 ± 3.7 'red' foods, P = 0.030), and STC (14.3 ± 3.4 'red' foods, P = 0.006). There were no statistically significant differences across home food environments for number of 'green' foods. CONCLUSION: Family-based obesity treatment interventions for preschoolers can improve overall diet quality and alter the home food environment through reductions in 'red' foods. TRIAL REGISTRATION: Clinicaltrials.gov, NCT01546727 . Registered March 7, 2012.

Risk factors for mortality in paediatric cardiac ICU patients managed with extracorporeal membrane oxygenation.

BACKGROUND: Veno-arterial extracorporeal membrane oxygenation is frequently used in patients with cardiac disease. We evaluated short-term outcomes and identified factors associated with hospital mortality in cardiac patients supported with veno-arterial extracorporeal membrane oxygenation. METHODS: A retrospective review of patients supported with veno-arterial extracorporeal membrane oxygenation at a university-affiliated children's hospital was performed. RESULTS: A total of 253 patients with cardiac disease managed with extracorporeal membrane oxygenation were identified; survival to discharge was 48%, which significantly improved from 39% in an earlier era (1995-2001) (p=0.01). Patients were categorised into surgical versus non-surgical groups on the basis of whether they had undergone cardiac surgery before or not, respectively. The most common indication for extracorporeal membrane oxygenation was extracorporeal cardiopulmonary resuscitation: 96 (51%) in the surgical group and 45 (68%) in the non-surgical group. In a multiple covariate analysis, single-ventricle physiology (p=0.01), duration of extracorporeal membrane oxygenation (p<0.01), and length of hospital stay (p=0.03) were associated with hospital mortality. Weekend or night shift cannulation was associated with mortality in non-surgical patients (p=0.05). CONCLUSION: We report improvement in survival compared with an earlier era in cardiac patients supported with extracorporeal membrane oxygenation. Single-ventricle physiology continues to negatively impact survival, along with evidence of organ dysfunction during extracorporeal membrane oxygenation, duration of extracorporeal membrane oxygenation, and length of stay.

Clinic and Home-Based Behavioral Intervention for Obesity in Preschoolers: A Randomized Trial.

OBJECTIVE: To test the hypotheses that an innovative skills-based behavioral family clinic and home-based intervention (LAUNCH) would reduce body mass index z score (BMIz) compared with motivational interviewing and to standard care in preschool-aged children with obesity. STUDY DESIGN: Randomized controlled trial with children between the ages of 2 and 5 years above the 95th percentile for body mass index for age and sex recruited from 27 pediatrician offices across 10 recruitment cycles between March 12, 2012 and June 8, 2015. Children were randomized to LAUNCH (an 18-session clinic and home-based behavioral intervention), motivational interviewing (delivered at the same frequency as LAUNCH), or standard care (no formal intervention). Weight and height were measured by assessors blinded to participant assignment. The primary outcome, BMIz at month 6 after adjusting for baseline BMIz, was tested separately comparing LAUNCH with motivational interviewing and LAUNCH with standard care using regression-based analysis of covariance models. RESULTS: A total of 151 of the 167 children randomized met intent-to-treat criteria and 92% completed the study. Children were 76% White and 57% female, with an average age of 55 months and BMI percentile of 98.57, with no demographic differences between the groups. LAUNCH participants demonstrated a significantly greater decrease in BMIz (mean = -0.32, SD = ±0.33) compared with motivational interviewing (mean = -0.05, SD = ±0.27), P < .001, ω2 = 0.74 and compared with standard care (mean = -0.13, SD = ±0.31), P < .004, ω2 = 0.75. CONCLUSIONS: In preschool-age children, an intensive 6-month behavioral skills-based intervention is necessary to reduce obesity. TRIAL REGISTRATION: Clinicaltrials.gov NCT01546727.

Hearing Loss after Cardiac Surgery in Infancy: An Unintended Consequence of Life-Saving Care.

OBJECTIVES: To investigate the prevalence of hearing loss after cardiac surgery in infancy, patient and operative factors associated with hearing loss, and the relationship of hearing loss to neurodevelopmental outcomes. STUDY DESIGN: Audiologic and neurodevelopmental evaluations were conducted on 348 children who underwent repair of congenital heart disease at the Children's Hospital of Philadelphia as part of a prospective study evaluating neurodevelopmental outcomes at 4 years of age. A prevalence estimate was calculated based on presence and type of hearing loss. Potential risk factors and the impact of hearing loss on neurodevelopmental outcomes were evaluated. RESULTS: The prevalence of hearing loss was 21.6% (95% CI, 17.2-25.9). The prevalence of conductive hearing loss, sensorineural hearing loss, and indeterminate hearing loss were 12.4% (95% CI, 8.8-16.0), 6.9% (95% CI, 4.1-9.7), and 2.3% (95% CI, 0.6-4.0), respectively. Only 18 of 348 subjects (5.2%) had screened positive for hearing loss before this study and 10 used a hearing aid. After adjusting for patient and operative covariates, younger gestational age, longer postoperative duration of stay, and a confirmed genetic anomaly were associated with hearing loss (all P < .01). The presence of hearing loss was associated with worse language, cognition and attention (P <.01). CONCLUSIONS: These findings suggest that the prevalence of hearing loss in preschool children after heart surgery in infancy may be 20-fold higher than in the 1% prevalence seen in the general population. Younger gestational age, presence of a genetic anomaly, and longer postoperative duration of stay were associated with hearing loss. Hearing loss was associated with worse neurodevelopmental outcomes.

Are Preschoolers Meeting the Mark? Comparing the Dietary, Activity, and Sleep Behaviors of Preschoolers With Obesity to National Recommendations.

Objective: National health organizations and expert committees have issued recommendations for health behaviors related to obesity risk. Behavioral and family-based weight management interventions for preschoolers often target improving adherence to these recommendations, but it is unknown how the health behaviors of preschoolers with obesity enrolled in weight control treatments (WCTs) compare with these guidelines. In this study, the dietary intake, activity, and sleep behaviors of preschoolers with obesity enrolled in a family-based behavioral WCT are described and compared with national health behavior recommendations. Methods: Health behaviors of 151 preschoolers with obesity (M age = 4.60, SD = 0.93) enrolled in a clinical trial of a weight management program were measured at baseline through caregiver-report questionnaires, three 24-hr dietary recalls, and accelerometers. Results: In total, 70% of the sample exceeded daily caloric recommendations, only 10 and 5% met recommendations for fruit and vegetable intake, respectively, and only 30% met the recommendation of consuming no sugar-sweetened beverages. The majority of the sample met the daily recommendations for 60 min of moderate-to-vigorous activity (80%), < 2 hr of screen time (68%), and sleep duration (70%). Conclusions: Behavioral weight management interventions for preschoolers with obesity should target the health behaviors where children are not meeting recommendations.

Shared reading quality assessment by parental report: preliminary validation of the DialogPR.

BACKGROUND: The American Academy of Pediatrics (AAP) recommends shared reading beginning as soon as possible after birth to promote healthy development. Shared reading quality can strongly influence outcomes, especially in children from low-SES households. Dialogic reading is a method developed to enhance verbal interactivity and engagement through book sharing, advocated by the AAP and clinic-based programs such as Reach Out and Read. There is no brief, validated, caregiver report measure of dialogic reading or shared reading quality currently available. METHODS: This cross-sectional study involved 49 healthy mother-child dyads (mean child age 4.5 yrs., SD = 0.6 yrs.) from 2 separate MRI-based studies. The DialogPR was administered by trained research coordinators following MRI, along with the READ subscale of the validated StimQ-P measure of home cognitive environment. The DialogPR consists of eight items developed in consultation with experts in early literacy, based on the PEER/CROWD dialogic reading conceptual model. Estimated reading level is 6th grade. Descriptive statistics were computed at both the item and scale levels. Modern theory Rasch methods were used to analyze all eight DialogPR items along with preliminary estimates of reliability and validity. RESULTS: Our combined sample involved 15 boys and 34 girls, and was diverse in terms of age, household income, and maternal education. DialogPR administration time was less than 2 min, with no problems reported. The DialogPR demonstrated strong internal consistency and reliability (Cronbach's alpha = 0.82), and criterion-related validity with the StimQ-P READ (Spearman's rho coefficient = 0.53). Rasch analysis revealed strong psychometric properties in terms of reliability, variability in item difficulty, and inter-item and item-measure correlations. CONCLUSIONS: Preliminary evidence suggests that the DialogPR may be an efficient means to assess shared reading quality and dialogic reading via caregiver report for clinical and research purposes, warranting further investigation.

Investigation of the Use of a Family Health History Application in Genetic Counseling.

The paper-based pedigree is the current standard for family health history (FHH) documentation in genetic counseling. Several tools for electronic capture of family health data have been developed to improve re-use and accessibility, data quality and standardization, ease of updating, and integration with electronic medical records. One such tool, the tablet-based Proband application, provides a flexible approach to data capture in dynamic and diverse clinical settings. This study compared Proband FHH collection to paper-based methods and investigated the usability of Proband in a clinical setting. After one use by 23 genetic counselors and students, Proband had 91% accuracy with a FHH audio scenario, which was significantly less (p < 0.001) than paper's 96% accuracy. These differences were attributed to incorrect or missing ages of grandparents (p < 0.001) and great-aunts/uncles (p = 0.012) and missing documentation of consanguinity (p < 0.001). Possible explanations for these differences include greater experience with paper FHH documentation and pre-populated prompts for consanguinity on the paper template used. Proband's perceived usability increased with use, with individual System Usability Scores increasing between first and last use (p = 0.033). We conclude that tools for dynamic, provider-driven FHH documentation such as Proband show promise for improving risk assessment accuracy and quality patient care.

Long-Term Outcomes of Pediatric Cardiac Patients Supported by Extracorporeal Membrane Oxygenation.

OBJECTIVE: Extracorporeal membrane oxygenation is an important form of short-term mechanical support in children with cardiac disease, but information on long-term outcomes and quality of life is limited. The primary objective of this study was to determine the long-term outcomes of children previously supported by extracorporeal membrane oxygenation for cardiac etiologies. DESIGN: A retrospective analysis was performed on patients with cardiac disease managed with extracorporeal membrane oxygenation between January 1, 1995, and December 31, 2012, at the Children's Hospital of Philadelphia. Survivors completed patient- and parent-reported verbal and written surveys, and univariate analyses assessed risk factors for long-term outcomes. SETTING: Tertiary-care children's hospital. PATIENTS: Patients with cardiac disease managed with extracorporeal membrane oxygenation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Over 18 years, 396 patients were managed with extracorporeal membrane oxygenation with 43% survival to discharge. The median age at cannulation was 78 days. The majority had congenital heart disease (86%), surgery prior to extracorporeal membrane oxygenation (71%), and cardiopulmonary arrest as the primary extracorporeal membrane oxygenation indication (53%). With 6-year median follow-up, 66% are known to be deceased, including 38 deaths after hospital discharge. Among survivors at discharge, 65 (38%) completed the phone survey, and 33 (19%) completed the written survey. Negative clinical outcomes, defined as having at least significant physical limitations or "fair" or "poor" health, were present in 18% of patients. No patient- or extracorporeal membrane oxygenation-related variables were associated with negative outcomes in univariate analyses. There were significantly lower self-reported and parent-reported written Pediatric Quality of Life Inventory quality of life scores in children compared with healthy individual normative data but no differences in adolescents. CONCLUSIONS: In this series of pediatric cardiac patients supported by extracorporeal membrane oxygenation, mortality was 66% with 6-year median follow-up. The majority reported positive outcomes with respect to health and physical limitations, but children reported lower quality of life compared with healthy individuals.

High Levels of Decisional Conflict and Decision Regret When Making Decisions About Biologics.

OBJECTIVES: The aim of the study was to understand the association between parents' perceptions of the decision process and the decision outcomes in decisions about the use of biologics in pediatric chronic conditions. METHODS: We mailed surveys to parents of children with inflammatory bowel disease or juvenile idiopathic arthritis who had started treatment with biologics in the prior 2 years and were treated at either of 2 children's hospitals. The survey included measures of the decision process, including decision control and physician engagement, and decision outcomes, including conflict and regret. We used means and frequencies to assess the response distributions. General linear models were used to test the associations between decision process and decision outcomes. RESULTS: We had 201 respondents (response rate 54.9%). Approximately 47.0% reported using shared decision making. Each physician engagement behavior was experienced by the majority of parents, with the highest percentage reporting that their child's physician used language they understood and listened to them. Approximately 48.5% of parents had decisional conflict scores of 25 or greater, indicating high levels of conflict. Approximately 28.2% had no regret, 31.8% had mild regret, and the remaining 40.0% had moderate to severe regret. Shared decision making was not associated with improved decisional conflict, but physician engagement behaviors were associated with both decisional conflict and regret. CONCLUSIONS: Improving decision outcomes will require more than just focusing on who parents perceive as controlling the final decision. Developing interventions that facilitate specific physician engagement behaviors may decrease parents' distress around decision making and improve decision outcomes.

Analysis of patient-reported anterior knee pain scale: implications for scale development in children and adolescents.

PURPOSE: The purpose of this investigation was to estimate and document the reliability and validity of the Anterior Knee Pain Scale (AKPS) and to estimate its relative prediction accuracy of anterior knee pain in young females. METHODS: Data from a prospective, epidemiologic study to diagnose patellofemoral knee pain among female athletes (n = 499) using the Anterior Knee Pain Scale (AKPS). Data were treated in 4 phases (descriptive phase, reliability phase, scale refinement phase) and a final validation stage that was focused on an effort to test and document the validation of the AKPS short form and perform head-to-head comparisons of the 6-item short form with the original, 13-item form. RESULTS: The AKPS was reduced from 13 items (αCoeff = 0.77, σSEM = 0.004) to 6 items (αCoeff = 0.78, σSEM = 0.004). Point-biserial correlations with patellofemoral pain diagnosis were comparable: r [498] = 0.70 (R(2) = 0.49, short form) and r [498] = 0.71 (R(2) = 0.51, long form), as was sensitivity: 84% (short form) and 80% (long form), and specificity: 89% (short form) and 90% (long form; AUC = 0.94 both). CONCLUSION: The current analyses indicate that a subset of measures from the AKPS is responsive to patellofemoral pain symptoms and may support screening for related diagnoses. A simpler and quicker scale optimized for diagnostic accuracy could reduce the demand on patients, clinicians and research teams focused on the identification and management of patellofemoral pain. LEVEL OF EVIDENCE: III.

Regional circumferential strain is a biomarker for disease severity in duchenne muscular dystrophy heart disease: a cross-sectional study.

The aim of this study is to determine the contribution of strain ε cc in mid left ventricular (LV) segments to the reduction of composite LV circumferential ε cc in assess severity of duchenne muscular dystrophy (DMD) heart disease as assessed by cardiac magnetic resonance imaging (CMR). DMD patients and control subjects were stratified by age, LV ejection fraction, and late gadolinium enhancement (LGE) status. Tagged CMR images were analyzed for global ventricular function, LGE imaging, and composite and segmental ε cc. The relationship between changes in segmental ε cc changes and LGE across patient groups was assessed by a statistical step-down model. LV ε cc exhibited segmental heterogeneity; in control subjects and young DMD patients, ε cc was greatest in LV lateral free wall segments. However, with increasing age and cardiac disease severity as demonstrated by decreased EF and development of myocardial strain the segmental differences diminished. In subjects with advanced heart disease as evidenced by reduced LV ejection fraction and presence of LGE, very little segmental heterogeneity was present. In control subjects and young DMD patients, ε cc was greatest in LV lateral free wall segments. Increased DMD heart disease severity was associated with reduced composite; ε cc diminished regional ε cc heterogeneity and positive LGE imaging. Taken together, these findings suggest that perturbation of segmental, heterogeneous ε cc is an early biomarker of disease severity in this cross-section of DMD patients.

The dynamic and diverse experience of adults with brachial plexus birth injury: a collective case study.

PURPOSE: To explore brachial plexus birth injury (BPBI)-affected adults' health-related quality of life (HRQoL) experiences informed by the theoretical framework of the International Classification of Functioning, Disability, and Health. METHODS: This study applied a qualitative collective case study design. Twelve participants who participated in a prior survey study were recruited using maximum variation sampling. One-on-one semi-structured interviews focused on gaining in-depth understanding of participants' experiences with HRQoL. Interviews were recorded verbatim and thematically analysed. RESULTS: We identified two themes. First, the theme biopsychosocial dimensions of the experience comprised the ways BPBI affects physical and emotional health and how BPBI is intertwined with affected individuals' identities, activities/participation, and social environments. Second, the theme lifelong and variable experience encompassed how BPBI is a chronic condition that changes over time and varies among affected individuals. The cross-case analysis derived a conceptual model of BPBI HRQoL to describe the BPBI experience through the lifespan. In this model, multiple dimensions of BPBI HRQoL expand through the lifespan while BPBI-specific health resources' availability contract. CONCLUSIONS: By providing insight into the many ways that BPBI affects and is affected by an individual's functioning and personal, social, and healthcare environmental factors, these findings underscore lifelong individualized care for BPBI-affected persons is needed.

Perioperative exposure to volatile organic compounds in neonates undergoing cardiac surgery.

OBJECTIVE: Volatile organic compounds (VOCs) are used in the sterilization and manufacture of medical equipment. These compounds have high vapor pressures with low water solubility and are emitted as gases from solids or liquids. They can be mutagenic, neurotoxic, genotoxic, and/or carcinogenic. Safe limits of exposure are not known for neonates. This study examined determinants of exposure in newborns undergoing cardiac surgery. METHODS: Twenty metabolites of 16 VOCs (eg, xylene, cyanide, acrolein, acrylonitrile, N, N-dimethylformamide, 1,3-butadiene, styrene, and benzene) were measured as metabolites in daily urine samples collected from 10 neonates undergoing cardiac operations (n = 150 samples). Metabolites were quantified using reversed-phase ultra-high performance liquid chromatography and electrospray ionization tandem mass spectrometry. Repeated measures analysis of covariance was performed for each metabolite to examine associations with use of medical devices. RESULTS: At least 3 metabolites were detected in every sample. The median number of metabolites detected in each sample was 14 (range, 3-15). In a model controlling for other factors, the use of extracorporeal membrane oxygenation was associated with significantly (P ≤ .05) greater metabolite levels of acrolein, acrylonitrile, ethylene oxide, propylene oxide, styrene, and ethylbenzene. Patients breathing ambient air had greater levels of metabolites of acrolein, xylene, N,N-dimethylformamide, methyl isocyanate, cyanide, 1,3-butadiene (all P ≤ .05). CONCLUSIONS: Exposure to volatile organic compounds is pervasive in newborns undergoing cardiac surgery. Sources of exposure likely include medical devices and inhalation from the air in the intensive care unit. The contribution of VOC exposure during cardiac surgery in newborns to adverse outcomes warrants further evaluation.

Increased utilization of the hybrid procedure is not associated with improved early survival for newborns with hypoplastic left heart syndrome: a single-centre experience.

OBJECTIVES: The primary objectives were to examine utilization of the Hybrid versus the Norwood procedure for patients with hypoplastic left heart syndrome or variants and the impact on hospital mortality. The Hybrid procedure was 1st used at our institution in 2004. METHODS: Review of all subjects undergoing the Norwood or Hybrid procedure between 1 January 1984 and 31 December 2022. The study period was divided into 8 eras: era 1, 1984-1988; era 2, 1989-1993; era 3, 1994-1998; era 4, 1999-2003; era 5, 2004-2008; era 6, 2009-2014; era 7, 2015-2018 and era 8, 2019-2022. The primary outcome was in-hospital mortality. Mortality rates were computed using standard binomial proportions with 95% confidence intervals. Rates across eras were compared using an ordered logistic regression model with and adjusted using the Tukey-Kramer post-hoc procedure for multiple comparisons. In the risk-modelling phase, logistic regression models were specified and tested. RESULTS: The Norwood procedure was performed in 1899 subjects, and the Hybrid procedure in 82 subjects. Use of the Hybrid procedure increased in each subsequent era, reaching 30% of subjects in era 8. After adjustment for multiple risk factors, use of the Hybrid procedure was significantly and positively associated with hospital mortality. CONCLUSIONS: Despite the increasing use of the Hybrid procedure, overall mortality for the entire cohort has plateaued. After adjustment for risk factors, use of the Hybrid procedure was significantly and positively associated with mortality compared to the Norwood procedure.

Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome.

BACKGROUND: Hypoplastic left heart syndrome (HLHS) is associated with significant mortality and morbidity. Fetal head growth abnormalities have been identified in a subset of HLHS fetuses, but it is unclear whether specific patterns of maladaptive growth affect clinical outcomes. We hypothesized that poor fetal head growth is associated with an increased frequency of adverse clinical outcomes. METHODS: We retrospectively examined a cohort of HLHS patients from midgestation to 1 y of age. Fetal and birth anthropometric measurements were analyzed using the Olsen standard, and clinical outcomes were obtained. RESULTS: A total of 104 HLHS patients were identified over a 12-y period; fetal data were available in 38 cases. HLHS neonates demonstrated a high incidence of microcephaly (12%), small head size (27%), and poor head growth (32%). All-cause mortality was 31% at 30 d and 43% at 1 y. Neurologic outcomes were observed in 12% of patients and were significantly increased with microcephaly (43 vs. 4%; P = 0.02). The average length of hospital stay following stage I palliation was 33.4 ± 33 d, correcting for early death. CONCLUSION: In term nonsyndromic HLHS, fetal and neonatal microcephaly are associated with early adverse neurologic outcomes but not mortality.