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.

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.

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.

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.

Decreasing Interstage Mortality After the Norwood Procedure: A 30-Year Experience.

Background The superior cavo-pulmonary connection was introduced at our institution in 1988 for infants undergoing surgery for hypoplastic left heart syndrome. Patients with hypoplastic left heart syndrome remain at high risk for mortality in the time period between the Norwood procedure and the superior cavo-pulmonary connection. The primary objectives of this study were to compare interstage mortality across 4 eras and analyze factors that may impact interstage mortality. Methods and Results Patients with hypoplastic left heart syndrome who underwent the Norwood procedure, were discharged from the hospital, and were eligible for superior cavo-pulmonary connection between January 1, 1988, and December 31, 2017, were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated with 95% CIs. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality. There were 1111 patients who met the inclusion criteria. Overall, interstage mortality was 120/1111 (10.8%). Interstage mortality was significantly lower in era 4 relative to era 1 (4.6% versus 13.4%; P=0.02) during the time that age at the superior cavo-pulmonary connection was the lowest (135 days; P<0.01) and the interstage monitoring program was introduced. In addition, use of the right ventricle to pulmonary artery shunt was associated with decreased interstage mortality (P=0.02) and was more routinely practiced in era 4. Conclusions During this 30-year experience, the risk of interstage mortality decreased significantly in the most recent era. Factors that coincide with this finding include younger age at superior cavo-pulmonary connection, introduction of an interstage monitoring program, and increased use of the right ventricle to pulmonary artery shunt.

Outcomes for the superior cavopulmonary connection in children with hypoplastic left heart syndrome: a 30-year experience.

OBJECTIVES: The objective of this study was to estimate hospital mortality and length of stay (LOS) for children with hypoplastic left heart syndrome undergoing superior cavopulmonary connection (SCPC). METHODS: All hypoplastic left heart syndrome interstage survivors who underwent SCPC between 1 January 1988 and 31 December 2017 were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated using standard binomial proportions. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality and LOS. RESULTS: The most common procedures for the cohort (n = 958) were Hemi-Fontan (57.3%) or Bidrectional Glenn shunt (35.7%). The mortality was 4.1% overall and decreased in all 3 later eras compared to era 1. Factors associated with mortality in a multiple covariate model included longer total support time, earlier gestational age, longer LOS at the Norwood Procedure and need for additional procedures. Overall, the median LOS was 7.0 days with a decrease from eras 1 to 2 and plateaued in eras 3 and 4. Predictors of longer LOS included genetic anomaly, longer Norwood LOS, additional procedures, lower weight at surgery and longer total support time. The type of SCPC was not associated with mortality or LOS. CONCLUSIONS: In this large cohort of patients with hypoplastic left heart syndrome undergoing SCPC, hospital mortality has decreased significantly. LOS initially declined but plateaued in recent eras. The risk factors for mortality and longer LOS are related to patient and procedural complexity, especially the need for additional procedures at the time of SCPC.

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.

Thirty years and 1663 consecutive Norwood procedures: Has survival plateaued?

OBJECTIVE: Hypoplastic left heart syndrome is one of the most common and challenging lesions requiring surgical intervention in the neonatal period. The Norwood procedure for hypoplastic left heart syndrome was first reported in 1983. The objective of this study was to describe early outcomes after the Norwood procedure at a single institution over 30 years. METHODS: This retrospective cohort study included all patients with hypoplastic left heart syndrome (and variants) who underwent the Norwood procedure between January 1984 and May 2014 at a single institution. The study period was divided into 6 eras: era 1, 1984 to 1988; era 2, 1989 to 1993; era 3, 1994 to 1998; era 4, 1999 to 2003; era 5, 2004 to 2008; and era 6, 2009 to 2014. The primary outcome was in-hospital mortality after the Norwood procedure. Binomial point estimates complete with 95% confidence intervals (CL0.95) were computed for the entire cohort and by era. RESULTS: During the study period, 1663 infants underwent the Norwood procedure. Overall in-hospital mortality was 25.9% (CL0.95, 23.8-28.0). Mortality by chronologic era was 40.4% (CL0.95, 34.9-45.9), 33.6% (CL0.95, 29.2-37.9), 28.7% (CL0.95, 22.8-34.6), 14.9% (CL0.95, 10.4-19.3), 11.2% (CL0.95, 7.4-15.0), and 15.7% (CL0.95, 10.3-21.1). Survival was improved in eras 4 to 6 compared with eras 1 to 3 (P all < .03). Anomalous pulmonary drainage, moderate to severe atrioventricular valve regurgitation, lower birth weight, earlier era, younger gestational age, genetic anomaly, preterm birth, race other than white or African-American, and lower weight at the Norwood procedure were associated with increased mortality. Mortality was greatest in patients with 3 or more risk factors. In the best-fitting multiple covariate model, anomalous pulmonary venous drainage, gestational age in weeks, genetic anomaly, and race other than white and African American were statistically significant contributors, after adjusting for era. CONCLUSIONS: Survival after the Norwood procedure has plateaued despite improvements in diagnosis, perioperative care, and surgical techniques. Nonmodifiable patient characteristics are important determinants of the risk of mortality.

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.

Perioperative Exposure to Suspect Neurotoxicants From Medical Devices in Newborns With Congenital Heart Defects.

BACKGROUND: Industrial chemicals are increasingly recognized as potential developmental neurotoxicants. Di(2-ethylhexyl) phthalate (DEHP), used to impart flexibility and temperature tolerance to polyvinylchloride, and bisphenol A (BPA), used to manufacture polycarbonate, are commonly present in medical devices. The magnitude of exposure in neonates during hospitalization for cardiac operations is unknown. METHODS: We quantified urinary concentrations of DEHP metabolites and BPA preoperatively and postoperatively in neonates undergoing cardiac operations and their mothers. Urinary concentrations of these biomarkers reflect recent exposures (half-lives are approximately 6 to 24 hours). Biomarker concentrations in mothers' and infants' preoperative and postoperative samples were compared. RESULTS: Operations were performed in 18 infants (mean age, 5 ± 4 [SD] days). The maternal sample was obtained on postpartum day 4 ± 4. The preoperative urine sample was obtained on day-of-life 4 ± 2 and the postoperative sample on day-of-life 6 ± 4. Mean maternal concentrations for DEHP metabolites and BPA were at the 50th percentile for females in the United States general population. Infant preoperative concentrations of 1 DEHP metabolite and BPA were significantly higher than maternal concentrations. Postoperative concentrations for all DEHP metabolites were significantly greater than preoperative concentrations. CONCLUSIONS: There is considerable perioperative exposure to DEHP and BPA for neonates undergoing cardiac operations. Infant concentrations for both BPA and DEHP metabolites were significantly higher than maternal concentrations, consistent with the infant's exposure to medical devices. Further study is needed to determine the potential role of these suspect neurotoxicants in the etiology of neurodevelopmental disability after cardiac operations.

The impact of the maternal-foetal environment on outcomes of surgery for congenital heart disease in neonates.

OBJECTIVES: Pregnancies with congenital heart disease in the foetus have an increased prevalence of pre-eclampsia, small for gestational age and preterm birth, which are evidence of an impaired maternal-foetal environment (MFE). METHODS: The impact of an impaired MFE, defined as pre-eclampsia, small for gestational age or preterm birth, on outcomes after cardiac surgery was evaluated in neonates (n = 135) enrolled in a study evaluating exposure to environmental toxicants and neuro-developmental outcomes. RESULTS: The most common diagnoses were transposition of the great arteries (n = 47) and hypoplastic left heart syndrome (n = 43). Impaired MFE was present in 28 of 135 (21%) subjects, with small for gestational age present in 17 (61%) patients. The presence of an impaired MFE was similar for all diagnoses, except transposition of the great arteries (P < 0.006). Postoperative length of stay was shorter for subjects without an impaired MFE (14 vs 38 days, P < 0.001). Hospital mortality was not significantly different with or without impaired MFE (11.7% vs 2.8%, P = 0.104). However, for the entire cohort, survival at 36 months was greater for those without an impaired MFE (96% vs 68%, P = 0.001). For patients with hypoplastic left heart syndrome, survival was also greater for those without an impaired MFE (90% vs 43%, P = 0.007). CONCLUSIONS: An impaired MFE is common in pregnancies in which the foetus has congenital heart disease. After cardiac surgery in neonates, the presence of an impaired MFE was associated with lower survival at 36 months of age for the entire cohort and for the subgroup with hypoplastic left heart syndrome.

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.

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.

Effect of congenital heart disease on 4-year neurodevelopment within multiple-gestation births.

OBJECTIVES: We sought to assess the effect of congenital heart disease requiring infant surgery with cardiopulmonary bypass on neurodevelopmental outcomes and growth at 4 years of age, while matching for gestational age, socioeconomic status, maternal gestational conditions, home environment, and parental intelligence by studying multiple-gestation births. METHODS: We performed within-family comparison of 14 multiple-gestation births in which 1 child had congenital heart disease requiring surgery with cardiopulmonary bypass at ≤6 months of age. Between 4 and 5 years of age, a comprehensive neurodevelopmental assessment was performed. Paired comparisons were conducted between siblings with and without heart defects using a series of nonparametric tests. RESULTS: On average, the children qualified as late preterm (mean gestational age 35.4 ± 2.6 weeks). At an average age of 4.8 ± 0.1 years, children with congenital heart disease weighed less than their siblings (median weight for age z score -0.4 vs 0.1, P = .02) and had worse performance for cognition (median full-scale IQ 99 vs 109, P = .02) and fine motor skills (median Wide Range Assessment of Visual Motor Ability, Fine Motor score 94.5 vs 107.5, P < .01). CONCLUSIONS: After controlling for socioeconomic status, home environment, parental intelligence, and gestational factors by using multiple-gestation births, congenital heart disease requiring surgery with cardiopulmonary bypass at ≤6 months of age is associated with lower weight, cognitive abilities and fine motor skills at 4 years of age.