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.

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.

Contemporary outcomes for functional single ventricle with total anomalous pulmonary venous connection.

OBJECTIVE: In 2004, we reported improved early survival for patients with functional single ventricle anatomy and total anomalous pulmonary venous connection. This study sought to discover if outcomes have been ameliorated in the contemporary era. METHODS: This was a single-center review of patients with single ventricle anatomy and total anomalous pulmonary venous connection who were admitted from 1984 to 2021. The cohort was divided into similarly sized groups by date of admission: Era 1: 1984 to 1992, Era 2: 1993 to 2007, and Era 3: 2008 to 2021. Survival was compared, and Cox proportional hazards models were used to evaluate the likelihood of mortality. RESULTS: We included 190 patients with single ventricle anatomy and total anomalous pulmonary venous connection. Unbalanced atrioventricular canal defect (70%) was the most common primary diagnosis. The most common type of total anomalous pulmonary venous connection was supracardiac (49%). Approximately one-third (32%) of patients had pulmonary venous obstruction. There were no significant differences in patient characteristics across eras. Early survival after initial palliative operation improved between Eras 1 and 2, and then remained stable in Era 3. Overall survival improved from Era 1 to Eras 2 and 3 (P < .001), but not between Era 2 and 3. Survival to 10 years by Eras 1 to 3 was 15%, 51%, and 54%, respectively. The anatomic features associated with worse survival were hypoplastic left heart syndrome diagnosis (hazard ratio, 1.60; 1.04-2.57) and pulmonary venous obstruction (hazard ratio, 1.80; 1.24-2.69). CONCLUSIONS: Overall survival for patients with single ventricle anatomy and total anomalous pulmonary venous connection has plateaued since the early 2000s. Even in the most recent era, survival to age 10 years remains less than 60%. Risk factors for mortality include the diagnosis of hypoplastic left heart syndrome and pulmonary venous obstruction. Further studies should focus on identification of the pathophysiological factors underlying the increased mortality.

uConsent: Addressing the gap in measuring understanding of informed consent in clinical research.

The purpose of this study was to establish the technical merit, feasibility, and generalizability of a new measure of understanding of informed consent for use with clinical research participants. A total of 109 teens/young adults at a large, pediatric medical center completed the consenting process of a hypothetical biobanking study. Data were analyzed using a combination of classical and modern theory analytic methods to produce a final set of 19 items referred to as the uConsent scale. A requirement of the scale was that each item mapped directly onto one or more of the Basic Elements of Informed Consent from the 2018 Final Rule. Descriptive statistics were computed for each item as well as the scale as a whole. Partial credit (Rasch) logistic modeling was then used to generate difficulty/endorsability estimates for each item. The final, 19-item uConsent scale was derived using inferential methods to yield a set of items that ranged across difficulty levels (-3.02 to 3.10 logits) with a range of point-measure correlations (0.12 to 0.50), within-range item- and model-fit statistics, varying item types mapped to both Bloom's Taxonomy of Learning and required regulatory components of the 2018 Final Rule. Median coverage rate for the uConsent scale was 95% for the 25 randomly selected studies from ClinicalTrials.gov. The uConsent scale may be used as an effective measure of informed consent when measuring and documenting participant understanding in clinical research studies today.

From clinical data management to clinical data science: Time for a new educational model.

The purpose of this article is to propose and provide a blueprint for a graduate-level curriculum in clinical data science, devoted to the measurement, acquisition, care, treatment, and inferencing of clinical research data. The curriculum presented here contains a series of five required core courses, five required research courses, and a list of potential electives. The coursework draws from but does not duplicate content from the foundational areas of biostatistics, clinical medicine, biomedical informatics, and regulatory affairs, and may be reproduced by any institution interested in and capable of offering such a program. This new curriculum in "clinical" data science will prepare students for work in academic, industry, and government research settings as well as offer a unifying knowledge base for the profession.

Impact of brachial plexus birth injury on health-related quality of life in adulthood: a mixed methods survey study.

PURPOSE: To describe the scope and nature of health concerns, functional impairments, and quality of life issues among adults with brachial plexus birth injury (BPBI). METHODS: A mixed methods study was conducted by surveying two social media networks of adults with BPBI using a combination of closed-ended and open-ended questions regarding the role of BPBI on ones' health, function, and quality of life. Closed-ended responses were compared across ages and genders. Open-ended responses were qualitatively analyzed to expand upon the close-ended responses. RESULTS: Surveys were completed by 183 respondents (83% female, age range 20-87 years). BPBI was reported to impact hand and arm use in 80% of participants (including affected and unaffected limbs and bimanual tasks), overall health in 60% (predominantly pain), activity participation in 79% (predominantly activities of daily living and leisure), life roles in 76% (predominantly occupation and parenting), and overall quality of life in 73% (predominantly self-esteem, relationships, and appearance). Significantly more females than males reported other medical conditions and an impact on hand and arm use and life roles. No other responses varied by age or gender. CONCLUSIONS: BPBI affects many facets of health related quality of life in adulthood with variability among affected individuals.

Brachial plexus birth injury's scope of impact in adulthood is broad, covering every aspect of HRQoL.The focus of rehabilitation for brachial plexus birth injury (BPBI) through the lifespan should extend beyond improving physical musculoskeletal function and include comprehensive support for physical, emotional, social, and life role concerns.Brachial plexus birth injury's nature of impact in adulthood varies among individuals within each aspect of health-related quality of life.The variability of BPBI's impact in adulthood underscores the need for individualized, patient-centered assessment and rehabilitative care.

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.

Combined ventricular dysfunction and atrioventricular valve regurgitation after the Norwood procedure are associated with attrition prior to superior cavopulmonary connection.

Background: Infants with hypoplastic left heart syndrome (HLHS) or a variant are at risk of ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) prior to superior cavopulmonary connection (SCPC). Although the impact of these complications in isolation has been described, their effect in combination on attrition is poorly defined. Methods: A retrospective observational study of patients with HLHS or variants undergoing a Norwood procedure between 2008 and 2020 at a single center was performed. VD and AVVR were defined as moderate or severe when seen on 2 sequential echocardiograms outside the perioperative period. Attrition was defined as death, listing for heart transplant, or unsuitability for SCPC or transplant. Descriptive statistics and regression models were used for analysis. Results: A total of 397 patients were included, of whom 75% had HLHS and 57% had received a Blalock-Thomas-Taussig shunt. Isolated VD occurred in 9% of patients, AVVR occurred in 13%, and both occurred in 6%. Attrition prior to SCPC occurred in 19% of the overall cohort, in 52% of patients with combined VD and AVVR (odds ratio [OR], 5.2; 95% confidence interval [CI], 2.3-12.0; P < .01), 26% of those with VD (OR, 1.5; 95% CI, 0.7-3.3; P = .32), 25% of those with AVVR (OR, 1.5; 95% CI, 0.7-2.9; P = .27), and 15% in those with neither (OR, 0.3; 95% CI, 0.2-0.6; P < .01). Other factors associated with attrition included prematurity, total bypass time at Norwood, and extracorporeal membrane oxygenation after Norwood, whereas later year of Norwood was protective (P < .01 for all). Conclusions: The presence of combined VD and AVVR markedly increases the likelihood of attrition prior to SCPC, identifying a high-risk group.

FIT Teens RCT for juvenile fibromyalgia: Protocol adaptations in response to the COVID 19 pandemic.

Objective: To describe protocol adaptations to the Fibromyalgia Integrative Training for Teens (FIT Teens) randomized controlled trial in response to the COVID-19 pandemic. The overarching aims of the FIT Teens multi-site 3-arm comparative effectiveness trial are to assess whether a specialized neuromuscular exercise training intervention combined with cognitive-behavioral therapy (CBT) is superior to CBT alone or graded aerobic exercise alone. Design/methods: The trial was originally designed as an in-person, group-based treatment with assessments at baseline, mid- and post-treatment, and four follow-up time points. The original study design and methodology was maintained with specific modifications to screening, consenting, assessments, and group-based treatments to be delivered in remote (telehealth) format in response to COVID-19 restrictions. Results: Study enrollment was paused in March 2020 for five months to revise operations manuals, pilot remote treatment sessions for accuracy and fidelity, complete programming of REDCap assent/consent and assessment materials, train study staff for new procedures and obtain regulatory approvals. The trial was relaunched and has been successfully implemented in remote format since July 2020. Trial metrics thus far demonstrate a consistent rate of enrollment, strong attendance at remote treatment sessions, high retention rates and high treatment fidelity after protocol adaptations were implemented. Conclusions: Preliminary findings indicate that FIT Teens protocol adaptations from in-person to remote are feasible and allowed for sustained enrollment, retention, and treatment fidelity comparable to the in-person format. Methodologic and statistical considerations resulting from the adaptations are discussed as well as implications for interpretation of results upon completion of the trial.

Childhood exposures to environmental chemicals and neurodevelopmental outcomes in congenital heart disease.

BACKGROUND: Children with congenital heart defects have an increased risk of neurodevelopmental disability. The impact of environmental chemical exposures during daily life on neurodevelopmental outcomes in toddlers with congenital heart defects is unknown. METHODS: This prospective study investigated the impacts of early childhood exposure to mixtures of environmental chemicals on neurodevelopmental outcomes after cardiac surgery. Outcomes were assessed at 18 months of age using The Bayley Scales of Infant and Toddler Development-III. Urinary concentrations of exposure biomarkers of pesticides, phenols, parabens, and phthalates, and blood levels of lead, mercury, and nicotine were measured at the same time point. Bayesian profile regression and weighted quantile sum regression were utilized to assess associations between mixtures of biomarkers and neurodevelopmental scores. RESULTS: One-hundred and forty infants were enrolled, and 110 (79%) returned at 18 months of age. Six biomarker exposure clusters were identified from the Bayesian profile regression analysis; and the pattern was driven by 15 of the 30 biomarkers, most notably 13 phthalate biomarkers. Children in the highest exposure cluster had significantly lower adjusted language scores by -9.41 points (95%CI: -17.2, -1.7) and adjusted motor scores by -4.9 points (-9.5, -0.4) compared to the lowest exposure. Weighted quantile sum regression modeling for the overall exposure-response relationship showed a significantly lower adjusted motor score (ß = -2.8 points [2.5th and 97.5th percentile: -6.0, -0.6]). The weighted quantile sum regression index weights for several phthalates, one paraben, and one phenol suggest their relevance for poorer neurodevelopmental outcomes. CONCLUSIONS: Like other children, infants with congenital heart defects are exposed to complex mixtures of environmental chemicals in daily life. Higher exposure biomarker concentrations were associated with significantly worse performance for language and motor skills in this population.

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.

Reference Ranges for Bone Mineral Content and Density by Dual Energy X-Ray Absorptiometry for Young Children.

BACKGROUND: Assessment of bone health in young children has been hampered by limited reference values for bone mineral content (BMC) and areal bone mineral density (aBMD) by dual energy X-ray absorptiometry (DXA). OBJECTIVES: To identify age, sex, and population ancestry effects on BMC and aBMD and develop smoothed reference ranges for BMC and aBMD in young children. To quantify precision of bone measurements and influence of height-for-age Z-scores on bone Z-scores. METHODS: We recruited 484 healthy children ages 1 to 2 years or 4.5 to 5 years at 2 clinical centers, who were seen once or up to 7 times over a 3-year period. Lumbar spine, distal forearm, and whole-body subtotal (ages ≥ 3 years) BMC and aBMD were measured by DXA. These data were combined with data from the Bone Mineral Density in Childhood Study from children ages 5 to 8.9 years to create the smoothed reference curves. RESULTS: For 1- to 5-year-olds, BMC and aBMD at all skeletal sites increased with age. Age trends differed by sex for BMC and aBMD of the spine, distal one-third radius, ultradistal radius, and by ancestry (Black vs non-Black) for all measures. BMC and aBMD precision (% coefficient of variation) ranged from 1.0% to 4.4%. Height Z-scores were positively associated with bone Z-scores and accounted for 4% to 45% of the variance. CONCLUSIONS: We demonstrate the feasibility of bone density measurements in young children and provide robust reference ranges and stature adjustments for calculation of bone Z-scores at multiple skeletal sites to enable bone health assessments.

Decision making about anti-TNF therapy: A pilot trial of a shared decision-making intervention.

OBJECTIVE: We conducted a pre-post pilot trial to determine the feasibility and acceptability of a multi-component intervention (pre-clinic letter, shared decision making cards and follow-up phone call) designed to facilitate SDM in pediatric inflammatory bowel disease (IBD). METHODS: We recruited physicians (n = 11) caring for IBD patients and families (n = 36) expected to discuss anti-tumor necrosis treatment. We measured feasibility and acceptability of the intervention, observed SDM, perceived SDM, decision conflict, and regret. Medical records were used to assess clinical outcomes, time to decision and adherence. We compared all outcomes between the usual care and intervention study arms. RESULTS: Two out of three intervention components were feasible. Visit length increased significantly in the intervention arm. Parents and patients rated the intervention as acceptable, as did most physicians. The intervention was associated with a higher-level of observed SDM. There was no difference perceived SDM, decision conflict, regret or quality of life outcomes between arms. Physician global assessment improved over time in the intervention arm. CONCLUSIONS: This pilot trial provides important guidance for developing a larger scale trial of a modified intervention. PRACTICE IMPLICATIONS: Overall, our intervention shows promise in supporting SDM and engaging both parents and patients in pediatric IBD decisions.

Examination of the Social Emotional Assets and Resilience Scales (SEARS) Youth Report: Factor Structure, Measurement Invariance, and Validity.

The Social and Emotional Assets and Resilience Scale (SEARS) is a promising instrument for prediction of resilience in youth; however, there is limited data to support its use. The purpose of the current study was to examine the factor structure, measurement invariance, internal consistency, and validity of the SEARS-Adolescent Report in youth 8 to 20 years of age. Two hundred and twenty-five childhood cancer survivors (Mage = 15.9, SD = 4.2; 51.4% male; 74.5% White) and 122 student controls without history of significant health problems (Mage = 14.2, SD = 3.5; 54.1% female; 79.5% White) 8 to 20 years of age completed the SEARS-A. The SEARS-A was found to have an adequate factor structure and model fit (χ2 = 1215.5, p < .001; root mean square error of approximation = .057; comparative fit index = .95; standardized root mean square residual = .06) and demonstrated invariance across domains of age, health status, gender, race, and socioeconomic status (Δ comparative fit index < -0.01). It also demonstrated excellent internal reliability, criterion validity, and current validity when compared with another well-established measure of psychological adjustment. As such, the SEARS-A has potential to be a useful, valid, and psychometrically sound tool for predicting social-emotional adjustment outcomes among at-risk youth 8 to 20 years of age.

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.

How many minors are participating in clinical research today? An estimate and important lessons learned.

Little is known about the number of minors enrolled in clinical research today. IRB administrators at leading pediatric medical centers were surveyed regarding studies with minors. Analyses were descriptive in nature with adaptive Bayesian bootstrap imputation used with missing data. Officials from 17/41 (41.5%) pediatric research centers responded: 74,204 active studies were estimated, 29,078 (39%) included minors, and 6574 (23%) were "more than minimal risk." Minors accounted for 0.7-2.87M research subjects. Pediatric medicine desperately needs a more accurate and reliable reporting system for tracking the recruitment, retention, and involvement of minors in clinical research.

Randomized Trial of a Mobile App Introduced During Well-Visits to Enhance Guidance for Reading With Young Children.

OBJECTIVE: To estimate feasibility, usability and efficacy of a mobile parenting app (Rx for Success; RxS) to enhance reading guidance provided to parents of young children during well-visits. METHODS: This trial was conducted at a clinic serving primarily families of Hispanic ethnicity and low-socioeconomic status (SES) where Reach Out and Read (ROR) is standard practice. It involved 252 parent-child dyads in 2 age groups (~6-months old, ~18-months old) randomized during well-visits to receive RxS or a children's book modeling alternatives to screen time (Control) by research coordinators. RxS involves videos, activities and "push" messages. Follow-up assessments were conducted approximately 6 months later, including impression and use, shared reading behaviors, child language and screen time. RESULTS: A total of 217 dyads completed both visits (110 RxS, 107 Control). Time to introduce RxS was under 3 minutes and 32% of parents experienced largely minor performance issues. Parent impression of RxS was favorable for both age groups at baseline and follow-up, though use was infrequent, attributable to a desire for more relevant and updated content. Significant findings favoring RxS included shared reading as a favorite activity, more frequent shared reading reported at 12 months and higher language scores at 24 months. Screen time was equivalent between cohorts, exceeding American Academy of Pediatrics guidelines. CONCLUSIONS: A mobile app introduced to parents of young children from low-SES backgrounds was feasible during well-visits, rated as helpful, and effective to enhance shared reading at younger and language at older ages. While a potentially impactful enhancement to ROR, features needing improvement were identified.

Validation of The Reading House and Association With Cortical Thickness.

BACKGROUND AND OBJECTIVES: The American Academy of Pediatrics recommends literacy and school readiness promotion during well visits. The Reading House (TRH) is a children's book-based screener of emergent literacy skills in preschool-aged children. Vocabulary, rhyming, and rapid naming are core emergent skills, and reading abilities are associated with thicker cortex in the left hemisphere. Our objective was to expand validity of TRH relative to these skills and explore association with cortical thickness. METHODS: Healthy preschool-aged children completed MRI including a T1-weighted anatomic scan. Before MRI, TRH and assessments of rapid naming (Comprehensive Test of Phonological Processing, Second Edition), rhyming (Pre-Reading Inventory of Phonological Awareness), vocabulary (Expressive Vocabulary Test, Second Edition), and emergent literacy (Get Ready to Read!) were administered. Analyses included Spearman-ρ correlations (r ρ) accounting for age, sex, and socioeconomic status (SES). MRI analyses involved whole-brain measures of cortical thickness relative to TRH scores, accounting for covariates. RESULTS: Seventy children completed assessments (36-63 months old; 36 female) and 52 completed MRI (37-63 months; 29 female). TRH scores were positively correlated with Comprehensive Test of Phonological Processing, Second Edition (r ρ = 0.61), Expressive Vocabulary Test, Second Edition (r ρ = 0.54), Get Ready to Read! (r ρ = 0.87), and Pre-Reading Inventory of Phonological Awareness scores (r ρ = 0.64; all P < .001). These correlations remained statistically significant across age, sex, and SES groups. TRH scores were correlated with greater thickness in left-sided language and visual cortex (P-family-wise error <.05), which were similar for higher SES yet more bilateral and frontal for low SES, reflecting a less mature pattern (P-family-wise error <.10). CONCLUSIONS: These findings expand validation evidence for TRH as a screening tool for preschool-aged children, including associations with emergent skills and cortical thickness, and suggest important differences related to SES.

Attrition between the superior cavopulmonary connection and the Fontan procedure in hypoplastic left heart syndrome.

OBJECTIVE: We investigated the incidence and predictors of failure to undergo the Fontan in children with hypoplastic left heart syndrome who survived superior cavopulmonary connection. METHODS: The cohort consists of all patients with hypoplastic left heart syndrome who survived to hospital discharge after superior cavopulmonary connection between 1988 and 2017. The primary outcome was attrition, which was defined as death, nonsuitability for the Fontan, or cardiac transplantation before the Fontan. Subjects were excluded if they were awaiting the Fontan, were lost to follow-up, or underwent biventricular repair. The study period was divided into 4 eras based on changes in operative or medical management. Attrition was estimated with 95% confidence intervals, and predictors were identified using adjusted, logistic regression models. RESULTS: Of the 856 hospital survivors after superior cavopulmonary connection, 52 died, 7 were deemed unsuitable for Fontan, and 12 underwent or were awaiting heart transplant. Overall attrition was 8.3% (71/856). Attrition rate did not change significantly across eras. A best-fitting multiple logistic regression model was used, adjusting for superior cavopulmonary connection year and other influential covariates: right ventricle to pulmonary artery shunt at Norwood (P < .01), total support time at superior cavopulmonary connection (P < .01), atrioventricular valve reconstruction at superior cavopulmonary connection (P = .02), performance of other procedures at superior cavopulmonary connection (P = .01), and length of stay after superior cavopulmonary connection (P < .01). CONCLUSIONS: In this study spanning more than 3 decades, 8.3% of children with hypoplastic left heart syndrome failed to undergo the Fontan after superior cavopulmonary connection. This attrition rate has not decreased over 30 years. Use of a right ventricle to pulmonary artery shunt at the Norwood procedure was associated with increased attrition.

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.