Continuous Electroencephalogram (cEEG) Findings and Neurodevelopmental Outcomes in Neonates with Congenital Heart Disease (CHD) at 12-24 Months of Age.

OBJECTIVE: This study aims to assess the role of continuous EEG (cEEG) background patterns and duration of cross-clamp time and cardiopulmonary bypass (CPB) in children with congenital heart disease (CHD) undergoing cardiac surgery and its correlation with abnormal neurodevelopmental outcomes at 12-24 months on Bayley Scales of Infant and Toddler Development (BSID-III). METHODS: This retrospective cohort study included infants with CHD and cEEG monitoring, who underwent surgery by 44 weeks gestational age. RESULTS: 34 patients were included, who were operated at median age - 7 days. Longer duration of cross- camp time was associated with poor language composite scores (LCS) (p value = 0.036). A significant association existed between severity of encephalopathy in 24-hour post-operative period and poor LCS (p value = 0.026). CONCLUSION: Majority of neonates with CHD have below average cognitive, language and motor composite scores on BSID-III. Longer duration of cross-clamp time and severity of encephalopathy during 24-hour post-operative EEG monitoring are associated with poor LCS.

Follow-up of telehealth parent training for sleep disturbances in young children with autism spectrum disorder.

OBJECTIVE/BACKGROUND: Bedtime and sleep disturbances are ubiquitous in children with autism. The telehealth delivery of a behavior analytic parent training program was earlier reported to be efficacious in improving child sleep and parent sense of competency. Our aim in this brief report was to determine the durability of the telehealth delivered sleep parent training program (SPT) compared to the control condition in this randomized controlled trial. Telehealth delivery could be a means to expand access to such early treatment if efficacious. PARTICIPANTS/METHODS: Parents of young children, ages 2-7 years, with autism and co-occurring sleep disturbances were enrolled in this study. Participants were randomized to either SPT or a comparison arm that included non-sleep related parent education except for one session Sleep Parent Education; SPE). Each participant was individually administered a 5 session program delivered over 10 weeks. Outcome measures were collected at weeks 5 and 10 and at post-treatment follow-up at 16 weeks. RESULTS: Follow-up week 16 data were available for 30 participants randomized to SPT and 24 participants randomized to SPE. Demographics for this follow-up cohort were similar to the full sample. At week 16 follow-up, there was a significant group difference between SPT and SPE the Modified Simonds & Parraga Sleep Questionnaire-Composite Sleep Index (MSPSQ - CSI) (p = 0.011) with an effect size of 0.68. Further, 8 of the 10 children in SPT who were negative responders at the week 10 endpoint were rated as positive responders at week 16 by the treatment masked IE compared to 2 out of 12 in the SPE group (p = 0.008). CONCLUSIONS: Post-treatment follow-up data suggests SPT is durable over time compared to SPE, the active control group. Further, for some participants in SPT, a positive responsive emerged after the treatment endpoint. This brief report adds to the efficacy of SPT as a time-limited intervention for insomnia in young autistic children.

Association of Fluid Overload with Escalation of Respiratory Support and Endotracheal Intubation in Acute Bronchiolitis Patients.

Fluid overload has been associated with increased oxygen requirement, prolonged duration of mechanical ventilation, and longer length of hospital stay in children hospitalized with pulmonary diseases. Critically ill infants with bronchiolitis admitted to the pediatric intensive care unit (PICU) also tend to develop fluid overload and there is limited information of its role on noninvasive respiratory support. Thus, our primary objective was to study the association of fluid overload in patients with bronchiolitis admitted to the PICU with respiratory support escalation (RSE) and need for endotracheal intubation (ETI). Infants ≤24 months of age with bronchiolitis and admitted to the PICU between 9/2009 and 6/2015 were retrospectively studied. Demographic variables, clinical characteristics including type of respiratory support and need for ETI were evaluated. Fluid overload as assessed by net fluid intake and output (net fluid balance), cumulative fluid balance (CFB) (mL/kg), and percentage fluid overload (FO%), was compared between patients requiring and not requiring RSE and among patients requiring ETI and not requiring ETI at 0 (PICU admission), 12, 24, 36, 48, 72, 96, and 120 hours. One-hundred sixty four of 283 patients with bronchiolitis admitted to the PICU qualified for our study. Thirty-four of 164 (21%) patients required escalation of respiratory support within 5 days of PICU admission and of these 34 patients, 11 patients required ETI. Univariate analysis by Kruskal-Wallis test of fluid overload as assessed by net fluid balance, CFB, and FO% between 34 patients requiring and 130 patients not requiring RSE and among 11 patients requiring ETI and 153 patients not requiring ETI, at 0, 12, 24, 36, 48, 72, 96 and 120 hours did not reveal any significant difference ( p >0.05) at any time interval. Multivariable logistic regression analysis revealed higher PRISM score (odds ratio [OR]: 4.95, 95% confidence interval [95% CI]: 1.79-13.66; p = 0.002), longer hours on high flow nasal cannula (OR: 4.86, 95% CI: 1.68-14.03; p = 0.003) and longer hours on noninvasive ventilation (OR: 11.16, 95% CI: 3.36-36.98; p < 0.001) were associated with RSE. Fluid overload as assessed by net fluid balance, CFB, and FO% was not associated with RSE or need for ETI in critically ill bronchiolitis patients admitted to the PICU. Further prospective studies involving larger number of patients with bronchiolitis are needed to corroborate our findings.

Telehealth parent training for sleep disturbances in young children with autism spectrum disorder: A randomized controlled trial.

BACKGROUND AND PURPOSE: Young children with autism spectrum disorder (autism) have bedtime and sleep disturbances at much higher frequency and persistency than their neurotypical counterparts. Hence, access to early, effective treatment is critical in view of the importance of sleep in early childhood. Telehealth delivery could be a means to expand access to such early treatment if efficacious. The aim of this randomized control trial (RCT) was to compare a manualized, telehealth delivered, behaviorally based sleep parent training (SPT) intervention for parents of young children with autism and sleep disturbances to a control condition, a telehealth delivered parent education program with one sleep focused session (SPE). We hypothesized that the SPT group would show more improvements on child measures of sleep outcome measures, and daytime behaviors and parent measures of stress and sense of competence. We further aimed to explore the overall feasibility of telehealth delivery of SPT and SPE. PARTICIPANTS AND METHODS: Parents of 77 young children, ages 2-7 years, with autism and co-occurring sleep disturbances were enrolled in this study. Participants were randomized to either SPT or a comparison arm that included non-sleep related parent education except for one session. Each participant was individually administered a 5 session program delivered over 10 weeks. Outcome measures, including child sleep measures, child daytime behavior and parent stress and sense of competency were collected at weeks 5 and 10 after the baseline time point. Feasibility indicators (treatment fidelity, parent adherence, and parent attendance), and safety measures were also collected. RESULTS: Of 77 randomized participants, data were available for 36 participants randomized to SPT and 38 participants randomized to SPE. The mean age was 3 years, 8 months. Results support the efficacy of this manualized SPT intervention for bedtime and sleep disturbances. Sleep outcome measures were significantly improved in the SPT group compared to SPE on the Modified Simonds & Parraga Sleep Questionnaire-Composite Sleep Index (MSPSQ - CSI) (p < 0.001) with a large effect size of 0.83 at week 10. Positive response to treatment, as determined from the Clinical Global Impression-Improvement scale (CGI-I) at week 10 was observed in 56% of SPT participants compared to 32% in SPE (p = 0.037). There were no significant group differences in either the ABC-I as measure of daytime behaviors or in parental stress. There were group differences in favor of SPT over SPE on the PSOC, a measure of parent sense of competency. Feasibility and safety were further demonstrated with telehealth delivery. CONCLUSIONS: This RCT demonstrated the efficacy of a telehealth delivered parent training intervention for bedtime and sleep disturbances in young autistic children compared to an active control condition. Further, parents in SPT reported more confidence in their parenting role than those in the SPE group, but SPT did not result in overall decreases in parental stress. Telehealth delivery allowed for a much broader reach with enrolled participants from 24 states. This study supports a telehealth approach to a manualized behavioral parent mediated intervention for sleep disturbance in young autistic children and offers an alternative to in-person delivered approaches. This telehealth delivery has the potential to improve access for families who have a young autistic child with sleep disturbances. Given the small sample size, determining predictors and moderators of treatment response was not possible and should be examined in a larger trial.

Universal suicide screening in emergency departments across a large healthcare system.

BACKGROUND: Suicidal ideation is a common complaint in Emergency Departments (EDs) across the United States (US) and is an important preventable cause of death. Consequently, current Joint Commission guidelines require screening high-risk patients and those with behavioral health needs for suicide. Accordingly, we implemented universal suicide screening for all patients presenting to EDs in our healthcare system and sought to describe the characteristics of the identified "high-risk" patients. We also sought to determine whether universal suicide screening was feasible and what its impact was on ED length of stay (LOS). METHODS: All ED encounters in the healthcare system were assessed. Data were collected from February 1, 2020, through June 30, 2022. All patients aged 18 and over were screened using the Columbia Suicide Severity Rating Scale (C-SSRS) and categorized as no risk, low risk, moderate risk, and high risk. Encounters were then grouped into 'high risk" and "not high risk," defined as no, low, and moderate risk patients. Data collected included gender, discharge disposition, LOS, and insurance status. RESULTS: A total of 1,058,735 patient encounter records were analyzed. The "high risk" group (n = 11,359; 10.7%) was found to have a higher proportion of male patients (50.9 vs 43.7%) and government payors (71.6 vs. 67.1%) and a higher ED LOS [medians 380 min vs. 198 min] than the not high-risk group (p ≤0.001). Those with suicidal ideation comprised 0.73-1.58% of ED encounters in a given month. A secondary analysis of 2,255,616 ED encounter records from January 2019 - June 30, 2022, revealed that 40,854 (1.81%) encounters required 1:1 observation. The proportion of 1:1 observations in 2019, the year before implementation, was 1.91%. Using a non-inferiority margin of 25%, we found that the proportion of 1:1 patients in 2020, the year following implementation, was non-inferior to (no worse than) the previous year at 2.09% and decreased from 2021 to 2022 (1.69% and 1.57% respectively). CONCLUSION: Implementing universal suicide screening in all EDs within a healthcare system is feasible. The percentage of patients who screened high risk was under 5% of the overall ED population. While the median LOS was longer for "high-risk" patients than for the general ED population, it was not excessively so. Adequate staffing to properly maintain the safety of these patients is paramount.

Bivalirudin or heparin for systemic anticoagulation during pediatric extracorporeal membrane oxygenation: Multicenter retrospective study.

BACKGROUND: The objective of this study is to evaluate the outcomes of unfractionated heparin (UFH) compared to bivalirudin anticoagulation in pediatric ExtraCorporeal Membrane Oxygenation (ECMO). METHODS: A multicenter retrospective study, that included pediatric patients <18 years of age, who were supported on ECMO between June 2017 and May 2020. Patients treated with UFH were matched 2:1 by age and type of ECMO support to the bivalirudin group. RESULTS: The bivalirudin group (75 patients) were matched to 150 patients treated with UFH. Baseline characteristics and comorbidities of the two groups were similar. Veno-Arterial ECMO was the most common mode (141/225 [63 %]) followed by extracorporeal cardiopulmonary resuscitation (48/225 [21 %]). Bivalirudin treatment was associated with lower odds of bleeding events (aOR 0.23, 95%CI 0.12-0.45, p < 0.001) and lower odds of thrombotic events (aOR 0.48, 95%CI 0.23-0.98, p = 0.045). Patients who received bivalirudin had lesser odds for transfusion with fresh frozen plasma, and platelets (aOR 0.26, CI 0.12-0.57, p ≤0.001 and aOR 0.28, CI 0.15-0.53, p < 0.001, respectively). After adjusting for the type of ECMO support and adjusting for age, bivalirudin was associated with a decrease in hospital mortality by 50 % compared to the UFH group (aOR 0.50, 95%CI 0.27-0.93, p = 0.028). Similarly, for neurological disability at time of discharge, bivalirudin was associated with higher odds of intact neurological outcomes compared to UFH (OR 1.99 [95%CI 1.13-3.51], p = 0.017). CONCLUSIONS: This study demonstrated that effective anticoagulation can be achieved with bivalirudin, which was associated with lesser odds of bleeding events and utilization of blood products. Bivalirudin, in comparison with UFH, was associated with greater odds of hospital survival and intact neurological function at the time of discharge. A prospective randomized trial is required to validate the results of this study.

Intra-host mutation rate of acute SARS-CoV-2 infection during the initial pandemic wave.

SARS-CoV-2 mutation is minimized through a proofreading function encoded by NSP-14. Most estimates of the SARS-CoV-2 mutation rate are derived from population based sequence data. Our understanding of SARS-CoV-2 evolution might be enhanced through analysis of intra-host viral mutation rates in specific populations. Viral genome analysis was performed between paired samples and mutations quantified at allele frequencies (AF) ≥ 0.25, ≥ 0.5 and ≥ 0.75. Mutation rate was determined employing F81 and JC69 evolution models and compared between isolates with (ΔNSP-14) and without (wtNSP-14) non-synonymous mutations in NSP-14 and by patient comorbidity. Forty paired samples with median interval of 13 days [IQR 8.5-20] were analyzed. The estimated mutation rate by F81 modeling was 93.6 (95%CI 90.8-96.4], 40.7 (95%CI 38.9-42.6) and 34.7 (95%CI 33.0-36.4) substitutions/genome/year at AF ≥ 0.25, ≥ 0.5, ≥ 0.75 respectively. Mutation rate in ΔNSP-14 were significantly elevated at AF ≥ 0.25 vs wtNSP-14. Patients with immune comorbidities had higher mutation rate at all allele frequencies. Intra-host SARS-CoV-2 mutation rates are substantially higher than those reported through population analysis. Virus strains with altered NSP-14 have accelerated mutation rate at low AF. Immunosuppressed patients have elevated mutation rate at all AF. Understanding intra-host virus evolution will aid in current and future pandemic modeling.

Continuous electroencephalography (cEEG) in infants with congenital heart disease (CHD).

BACKGROUND: Neonates with congenital heart disease (CHD) undergoing cardiopulmonary bypass (CPB) surgery have increased risk of impaired neurodevelopmental outcomes secondary to brain injury. This study aims to characterize pre- and post-operative continuous EEG (cEEG) patterns to detect abnormal cerebral activity in infants with CHD and investigate whether an association exists between the degree of encephalopathy in pre- and post-operative cEEG. METHODS: This retrospective cohort study conducted between 2010 and 2018 at a tertiary hospital in Cleveland, OH included infants with CHD with cEEG monitoring, who underwent CPB surgery within first 6 months of life. RESULTS: Study included 77 patients, of which 61% were males who were operated at median age 6 days. Pre-operatively, 69% and 87% had normal cEEG and sleep-wake cycles, respectively. Post-operatively, 80% had abnormal cEEG. Longer circulatory arrest time and CPB were associated with lack of continuity (p 0.011), excessive discontinuity (p 0.007) and prolonged inter-burst interval (IBI) duration (p value < 0.001). A significant association existed between severity of encephalopathy in immediate and 24-h post-operative period (p value < 0.001). CONCLUSIONS: More than 80% of neonates with CHD have abnormal post-operative EEG. Longer circulatory arrest time and CPB were associated with lack of continuity, excessive discontinuity, and prolonged IBI duration on post-operative EEG. IMPACT: This study shows that majority of neonates with congenital heart disease (CHD) have normal pre-operative EEG with a continuous background and normal sleep-wake cycles. Also, 80% of neonates had abnormal post-operative EEG. Longer duration of arrest time and bypass time was associated with lack of continuity, excessive discontinuity, and prolonged IBI duration during post-operative EEG monitoring. These findings will help clinicians when counseling parents in the intensive care unit, risk stratification, and long-term neurodevelopmental monitoring in these high-risk patients.

Leveraging community engagement to increase the health literacy of youth in rural Pennsylvania: The ACHIEVE program.

PURPOSE: Limited health literacy is prevalent within rural populations and associated with poor health outcomes. This study examined a school-based, community-engaged program called ACHIEVE (Advancing Community Health Innovation through Education, Vision, and Empowerment) for preliminary efficacy in improving knowledge and self-efficacy related to health literacy among youth in rural Huntingdon County, Pennsylvania. METHODS: ACHIEVE was designed using an iterative process that utilized validated sources, educational standards, and community engagement. Five novel health literacy modules were piloted by the program in Huntingdon Area High School and delivered to ∼269 students during the 2019-2020 and 2020-2021 school years. To determine the impact of the program, we assessed participants' change in health knowledge and self-efficacy using pre- and post-tests for each module. Responses were collected via anonymous surveys and analyzed using unequal variance t-tests and chi-square tests. FINDINGS: The overall mean difference between pre- and post-tests ranged from 0.07 to 0.67, with a significant increase in participants' assessment scores following 4 out of the 5 program modules (P < .05). Across the 5 modules, both knowledge and self-efficacy domains displayed a significant improvement from pre- to post-test (P < .001). CONCLUSION: Our findings suggest that community partnerships in rural communities can be used to create effective community health interventions, such as our health literacy program, which significantly increased high school students' knowledge and self-efficacy.

Central-line-associated bloodstream infections and central-line-associated non-CLABSI complications among pediatric oncology patients.

OBJECTIVE: To assess central venous catheter (CVC) harm in pediatric oncology patients, we explored risks for central-line-associated bloodstream infections (CLABSIs) and central-line-associated non-CLABSI complications (CLANCs). DESIGN: Retrospective cohort study. SETTING: Midwestern US pediatric oncology program. PATIENTS: The study cohort comprised 592 pediatric oncology patients seen between 2006 and 2016. METHODS: CLABSIs were defined according to Centers for Disease Control and Prevention (CDC)/National Health Safety Network (NHSN) definitions. CLANCs were classified using a novel definition requiring CVC removal. Patient-level and central-line-level risks were calculated using a negative binomial model to adjust for correlations between total events and line numbers. RESULTS: CVCs were inserted in 62% of patients, with 175,937 total catheter days. The inpatient CLABSI and CLANC rates were 5.8 and 8.5 times higher than outpatient rates. At the patient level, shared risks included acute myeloid leukemia (AML) and age <1 year at diagnosis. At the line level, shared risks included age <1 year at diagnosis, non-mediports, and >1 lumen. AML was a CLABSI-specific risk. CLANC-specific risks included non-brain-tumor diagnosis, younger age at diagnosis or central-line placement, and age <1 year at diagnosis or line placement. Multivariable risks were for CLABSI >1 lumen and for CLANC age <1 year at placement. CONCLUSIONS: Among patients with CVCs, CLABSI and CLANC rates were similar, higher among inpatients than outpatients. For both CLABSIs and CLANCs, infants and patients with AML were at higher risk. In both univariate and multivariate models, lines with >1 lumen were associated with CLABSIs and placement during infancy with CLANCs.

Outcomes After Positive Syphilis Screening.

BACKGROUND: Syphilis screening during pregnancy helps prevent congenital syphilis. The harms associated with false positive (FP) screens and whether screening leads to correct treatments has not been well determined. METHODS: The population included mothers and infants from 75 056 pregnancies. Using laboratory-based criteria we classified initial positive syphilis screens as FP or true positive (TP) and calculated false discovery rates. For mothers and infants we determined treatments, clinical characteristics, and syphilis classifications. RESULTS: There were 221 positive screens: 183 FP and 38 TP. The false discovery rate was 0.83 (95% confidence interval [CI], 0.78-0.88). False discovery rates were similar for traditional 0.83 [95% CI, 0.72-0.94] and reverse algorithms 0.83 (95% CI, 0.77-0.88), and for syphilis Immunoglobin (Ig) G 0.79 (95% CI, 0.71-0.86) and total 0.90 (95% CI, 0.82-0.97) assays. FP screens led to treatment in 2 women and 1 infant. Two high-risk women were not rescreened at delivery and were diagnosed after hospital discharge; 1 infant developed congenital syphilis. Among 15 TP women with new syphilis, the diagnosis was before the late third trimester in 14 (93%). In one-half of these women, there was concern for reinfection, treatment failure, inadequate treatment or follow-up care, or late treatment, and their infants did not achieve an optimal syphilis classification. CONCLUSIONS: Syphilis screening identifies maternal syphilis, but limitations include FP screens, which occasionally lead to unnecessary treatment, inconsistent risk-based rescreening, and among TP mothers failure to optimize care to prevent birth of infants at higher risk for congenital syphilis.

Genome-Wide Association Study of Pregnancy in Childhood Cancer Survivors: A Report from the Childhood Cancer Survivor Study.

BACKGROUND: Gonadotoxic treatment-related infertility has a significant impact on quality of life in childhood cancer survivors. Genome-wide association analyses to delineate the risk of infertility in childhood cancer survivors have not been previously reported. METHODS: Leveraging genotype data from a large survivor cohort, the Childhood Cancer Survivor Study (CCSS), we investigated the role of SNPs on future pregnancy or siring a pregnancy in survivors without pelvic, testicular, or brain radiation who had ever been married. We calculated sex-stratified hazard ratios, using Cox proportional hazards modeling, adjusting for birth cohort (before 1965 vs. 1965 or later) and doses of relevant chemotherapies; replication was attempted in the independent St. Jude Lifetime Cohort study (SJLIFE). RESULTS: In the CCSS cohort, nine SNPs were found to be suggestive (P < 10-7) or statistically significantly (P < 5 × 10-8) associated with pregnancy, however, none of the SNPs were replicated in SJLIFE. Cohorts differed based on the overall pregnancy rate, frequency of sterilizing procedures, and birth cohort. CONCLUSIONS: We were not able to replicate our findings of SNPs associated with pregnancy in childhood cancer survivors. IMPACT: Future attempts at replication should be considered in cohorts treated in a comparable era. In addition, understanding the role of genetics in fertility in childhood cancer survivors may be better approached using more advanced sequencing techniques.

In the grey zone-survival and morbidities of periviable births.

OBJECTIVE: To compare the survival and morbidities of infants born between 22 0/7-25 6/7 weeks of gestation. STUDY DESIGN: This observational cohort study consisted of 187 eligible infants liveborn at a single, Level III Neonatal Intensive Care Unit (NICU) between June 1, 2009, and December 31, 2016, in Cleveland, Ohio. Infants with recognized syndromes or major congenital malformations were excluded from the review. RESULT: The rate of survival to discharge for NICU-admitted infants born at 22- and 23- week was 56% and 54% respectively at our institution. There was no trend observed between gestational ages and incidence of necrotizing enterocolitis (NEC), patent ductus arteriousus (PDA), sepsis, or severe intraventricular hemorrhage (IVH- Grade 3 or 4). The infants born at 22 weeks had a higher incidence of retinopathy of prematurity (ROP) as compared to 25 weeks gestation (p < 0.001). The need for home oxygen was significantly higher in the smallest infants 70% at 22 weeks, 62% and 60% at 23 and 24 weeks versus 33% at 25 weeks gestation (p < 0.007). Those born at 22 weeks had the same rate of survival to discharge with severe IVH as those born at 23 weeks but required fewer VP shunts (p > 0.52). CONCLUSIONS: The course of extremely preterm infants shows no difference between those born at 22 and 23 weeks of gestation in our NICU with regards to both mortality and short-term morbidities, although they differed marginally from 24 week gestation infants and significantly from those born at 25 weeks gestation.

Measurement of Quality-of-Life Outcomes in Pediatric and Young Adult Patients Treated for Eosinophilic Esophagitis.

Eosinophilic esophagitis is a chronic, immune-mediated esophageal condition that may lead to impairment of quality of life in pediatric and young adult patients. We performed a prospective, cross-sectional study on 40 patients between the ages of 2-21 years with an established diagnosis of eosinophilic esophagitis. The study evaluated physical, emotional, social, and school functioning in patients undergoing treatment with proton pump inhibitors, dietary elimination, or swallowed corticosteroids. There were no statistically significant differences in total or domain-specific quality of life scores between proton pump inhibitors, dietary elimination, and swallowed corticosteroid therapy. Overall, total and domain-specific quality of life were well-preserved in patients with eosinophilic esophagitis, with the highest scores reported in social functioning. There were also no statistically significant associations between clinical, endoscopic, and histologic features and quality-of-life measures.

Waitlist Outcomes for Children With Congenital Heart Disease: Lessons Learned From Over 5000 Heart Transplant Listings in the United States.

BACKGROUND: We evaluated the impact of pediatric heart-allocation policy changes over time and the approval of the Berlin ventricular assist device (VAD) on waitlist (WL) outcomes for children with congenital heart disease (CHD). METHODS: The Scientific Registry of Transplant Recipients database was evaluated to include all children (age < 18) with CHD and cardiomyopathy (CMP) on the WL between 1999 and 2019, divided into 4 eras: Era 1 (1999-2008); Era 2 (2009-2011); Era 3 (2012-2016); and Era 4 (2016-2019). WL characteristics and survival outcomes were evaluated for patients with CHD over time and were compared to those with CMP listed currently (Era 4). RESULTS: We included 5185 children with CHD on the WL during the study period; 1999 (39%) were listed in Era 1; 693 (13%) in Era 2; 1196 (23%) in Era 3; and 1297 (25%) in Era 4. Compared to the CHD WL in eras 1 and 2, those in Era 4 were less likely to be infants (48% vs 49% vs 43%), on mechanical ventilation (30% vs 26% vs 19%), on extracorporeal membrane oxygenation (15% vs 9.7% vs 6.2%), and were more likely to be on a VAD (2.4% vs 2.2% vs 6.0%) (P < .05 for all). WL survival improved in children with CHD from Era 1 to Era 4 (P < .001). However, in Era 4, children with CHD had lower WL survival than those with CMP (P < .001). CONCLUSION: Children with CHD are increasingly being listed with less advanced heart failure, and they have had improved WL survival over time; however, WL outcomes remain inferior to those with CMP. Advances in pediatric medical and VAD therapy may improve future WL outcomes.

Contemporary Provider Management Practices and Attitudes Toward Referral for Advanced Heart Failure Therapies in Fontan Patients Across North America.

BACKGROUND: To date, no reports have described clinicians' management practices for patients with Fontan circulatory failure or their understanding of risk factors for mortality and transplant outcomes in these patients. METHODS AND RESULTS: A cross-sectional survey of caregivers across North America was conducted from February to September 2020. Responses were compared by primary specialty (heart failure/transplant vs non-heart failure/transplant), years of experience (early, mid, and late career), and Fontan center volume (low, medium, and high). Of 400 responses, the majority were from general cardiologists (111, 28%) followed by heart failure/transplant specialists (93, 23%). Although most agreed that patients with Fontan physiology will have signs/symptoms of heart failure (369 [93%]) and eventuate in heart transplant (286 [72%]), many disagreed (180 [45%]) that routine evaluation by a transplant cardiologist is needed without symptoms. Transplant providers were more likely than non-transplant providers to suggest referral for manifestations of Fontan circulatory failure such as protein-losing enteropathy, plastic bronchitis, liver fibrosis/cirrhosis, and worsening valve regurgitation. Non-transplant providers were more likely to suggest that protein-losing enteropathy, plastic bronchitis, and Fontan-associated liver disease lead to inferior outcomes after transplantation. Early career and transplant providers more favorably viewed ventricular assist device use for Fontan patients failing traditional heart failure therapy (P < .05 for all). CONCLUSIONS: There is significant variation in the management of Fontan patients, including heterogeneous timing of referral of such patients to the heart failure/transplant team, which may have implications for future outcomes.

Sex disparities in the current era of pediatric heart transplantation in the United States.

BACKGROUND: While sex-related differences in transplant outcomes have been well characterized amongst adults, there are no sex-specific pediatric heart transplant studies over the last decade and none evaluating waitlist outcomes. In a contemporary cohort of children undergoing heart transplantation in the United States, this analysis was performed to determine if there were sex disparities in waitlist and/or post-transplant outcomes. METHODS: Retrospective review of Scientific Registry of Transplant Recipients database from December 16, 2011 to February 28, 2019 to compare male and female children after listing and after transplant. Demographic, clinical characteristics and outcomes were compared unadjusted and after 1:1 propensity matching for selected covariates. RESULTS: Of 4089 patients, 2299 (56%) were males. At listing, males were more likely to be older, have congenital heart disease (58% vs 48%), renal dysfunction (49% vs 44%) and implantable cardioverter defibrillator (9% vs 7%). At transplant, males were more likely to have renal (42 % vs 35%) and liver dysfunction (13% vs 10%), PRA >10% (29% vs 22%) and ischemic time >3.5 hours (p < 0.05 for all). There were no significant sex differences found in unadjusted rates of transplant or mortality. After propensity matching, females had increased waitlist mortality (HR 1.3, 95%CI 1.04-1.5; p =0.019) compared to males. There were no significant differences in post-transplant morbidity or mortality (HR 1.2, 95% CI 0.93-1.5; p = 0.18) between groups. CONCLUSION: In a contemporary pediatric cohort, females have inferior heart transplant waitlist survival compared to propensity-matched males despite lower acuity of illness at listing and similar rates of transplantation. There were no sex-disparities noted in post-transplant outcomes.

Three Healthy Eating Patterns and Cardiovascular Disease Risk Markers in 9 to 18 Year Olds With Body Mass Index >95%: A Randomized Trial.

Approximately 20% of American 9 to 18 year olds are obese, and most carry their excess adiposity, with its associated increased risk for cardiovascular disease, into adulthood. We studied cardiovascular disease risk markers changes associated with 3 healthy eating patterns (HEPs) in 96 9 to 18 year olds with a body mass index >95% in a Midwestern health system 1-year randomized trial. All HEPs were associated with similar statistically significant (P < .05 to <.001) cardiovascular disease risk marker improvements in weight, systolic and diastolic blood pressure, total cholesterol, low-density lipoprotein, and myeloperoxidase. Time required was the only identified significant (P < .001) deterrent from enrolling in study. These HEPs had characteristics common to most HEPs: encouraging whole foods, fruits and vegetables, whole grains, beans other legumes, and limiting added salt, saturated fatty acids, added sugars, red meat, processed meats, and other processed foods. Further research on initiatives to ease the time burden, and increase implementation of established healthy eating principles is needed.