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There is a growing awareness that diversity, health equity, and inclusion play a significant role in improving patient outcomes and advancing knowledge. The Pediatric Heart Network launched an initiative to incorporate diversity, health equity, and inclusion into its 2021 Scholar Award Funding Opportunity Announcement. This manuscript describes the process of incorporating diversity, health equity, and inclusion into the Pediatric Heart Network Scholar Award and the lessons learned. Recommendations for future Pediatric Heart Network grant application cycles are made which could be replicated by other funding agencies.

Objectives: Obesity is a known risk factor for developing cardiovascular disease (CVD). American Indian (AI) children have the highest obesity rates of all racial/ethnic groups in the United States (US). However, cardiometabolic health among AI adolescents is understudied. The purpose of this study was to describe the cardiometabolic characteristics of AI adolescents enrolled in the Strong Heart Family Study (SHFS), a longitudinal study of CVD from 12 AI communities, and associations with the development of abnormal left ventricular mass index (LVMI) and ejection fraction (EF)., Study Design: This study included AI youth ages 14-22 years. Primary outcome variables were depressed EF (< 55%) and abnormal LVMI at 6-year follow-up. The primary predictor variable was BMI. Covariates included sex, cholesterol levels, blood pressure, and hemoglobin A1c. Wilcoxon signed-rank test for matched pairs evaluated cardiometabolic trends over time. Stratified univariate logistic regression by obese/nonobese categories evaluated associations with depressed EF and abnormal LVMI., Results: There were 384 subjects, 58% of whom were overweight (n = 87) or obese (n = 122) at baseline. Among overweight/obese subjects, cholesterol and triglyceride levels were elevated. At 6-year follow-up, BMI increased while EF decreased among overweight/obese males, with 3.6 times the odds of having depressed EF (95% CI 1.22-10.98, p = 0.021) compared to normal-weight males. There were no significant cardiometabolic associations with LVMI., Conclusions: This is one of the largest longitudinal evaluations of CVD in AI adolescents. Concerning trends in BMI and depressed EF among AI males suggest the importance of culturally competent interventions to promote healthy weight and reduce cardiometabolic risk., (© 2024. W. Montague Cobb-NMA Health Institute.)

Background: Given increased survival for adults with CHD, we aim to determine outcome differences of infective endocarditis compared to patients with structurally normal hearts in the general population., Methods: We conducted a retrospective cross-sectional study identifying infective endocarditis hospitalisations in patients 18 years and older from the National Inpatient Sample database between 2001 and 2016 using International Classification of Disease diagnosis and procedure codes. Weighting was used to create national annual estimates indexed to the United States population, and multivariable logistic regression analysis determined variable associations. Outcome variables were mortality and surgery. The primary predictor variable was the presence or absence of CHD., Results: We identified 1,096,858 estimated infective endocarditis hospitalisations, of which 17,729 (1.6%) were adults with CHD. A 125% increase in infective endocarditis hospitalisations occurred for adult CHD patients during the studied time period ( p < 0.001). Adults with CHD were significantly less likely to experience mortality (5.4% vs. 9.5%, OR 0.54, CI 0.47-0.63, p < 0.001) and more likely to undergo in-hospital surgery (31.6% vs. 6.7%, OR 6.49, CI 6.03-6.98, p < 0.001) compared to the general population. CHD severity was not associated with increased mortality ( p = 0.53). Microbiologic aetiology of infective endocarditis varied between groups ( p < 0.001) with Streptococcus identified more commonly in adults with CHD compared to patients with structurally normal hearts (36.2% vs. 14.4%)., Conclusions: Adults with CHD hospitalised for infective endocarditis are less likely to experience mortality and more likely to undergo surgery than the general population.

Introduction: Traditional strategies for grouping congenital heart defects (CHDs) using birth defect registry data do not adequately address differences in expected clinical consequences between different combinations of CHDs. We report a lesion-specific classification system for birth defect registry-based outcome studies., Methods: For Core Cardiac Lesion Outcome Classifications (C-CLOC) groups, common CHDs expected to have reasonable clinical homogeneity were defined. Criteria based on combinations of Centers for Disease and Control-modified British Pediatric Association (BPA) codes were defined for each C-CLOC group. To demonstrate proof of concept and retention of reasonable case counts within C-CLOC groups, Texas Birth Defect Registry data (1999-2017 deliveries) were used to compare case counts and neonatal mortality between traditional vs. C-CLOC classification approaches., Results: C-CLOC defined 59 CHD groups among 62,262 infants with CHDs. Classifying cases into the single, mutually exclusive C-CLOC group reflecting the highest complexity CHD present reduced case counts among lower complexity lesions (e.g., 86.5% of cases with a common atrium BPA code were reclassified to a higher complexity group for a co-occurring CHD). As expected, C-CLOC groups had retained larger sample sizes (i.e., representing presumably better-powered analytic groups) compared to cases with only one CHD code and no occurring CHDs., Discussion: This new CHD classification system for investigators using birth defect registry data, C-CLOC, is expected to balance clinical outcome homogeneity in analytic groups while maintaining sufficiently large case counts within categories, thus improving power for CHD-specific outcome association comparisons. Future outcome studies utilizing C-CLOC-based classifications are planned., (© 2024 Wiley Periodicals LLC.)

Social determinants of health (SDoH) are the economic, social, environmental, and psychosocial factors that influence health. Adolescents and young adults with congenital heart disease (CHD) require lifelong cardiology follow-up and therefore coordinated transition from pediatric to adult healthcare systems. However, gaps in care are common during transition, and they are driven in part by pervasive disparities in SDoH, including race, ethnicity, socioeconomic status, access to insurance, and remote location of residence. These disparities often coexist and compound the challenges faced by patients and families. For example, Black and Indigenous individuals are more likely to be subject to systemic racism and implicit bias within healthcare and other settings, to be unemployed and poor, to have limited access to insurance, and to have a lower likelihood of transfer of care to adult CHD specialists. SDoH also are associated with acquired cardiovascular disease, a comorbidity that adults with CHD face. This review summarizes existing evidence regarding the impact of SDoH on the transition to adult care and proposes strategies at the individual, institutional, and population and/or system levels. to reduce inequities faced by transition-age youth. These strategies include routinely screening for SDoH in clinical settings with referral to appropriate services, providing formal transition education for all transition-age youth, including training on navigating complex medical systems, creating satellite cardiology clinics to facilitate access to care for those who live remote from tertiary centres, advocating for lifelong insurance coverage where applicable, mandating cultural-sensitivity training for providers, and increasing the diversity of healthcare providers in pediatric and adult CHD care., (Copyright © 2024 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Congenital heart disease (CHD) comprises a range of structural anomalies, each with a unique natural history, evolving treatment strategies, and distinct long-term consequences. Current prediction models are challenged by generalizability, limited validation, and questionable application to extended follow-up periods. In this JACC Scientific Statement, we tackle the difficulty of risk measurement across the lifespan. We appraise current and future risk measurement frameworks and describe domains of risk specific to CHD. Risk of adverse outcomes varies with age, sex, genetics, era, socioeconomic status, behavior, and comorbidities as they evolve through the lifespan and across care settings. Emerging technologies and approaches promise to improve risk assessment, but there is also need for large, longitudinal, representative, prospective CHD cohorts with multidimensional data and consensus-driven methodologies to provide insight into time-varying risk. Communication of risk, particularly with patients and their families, poses a separate and equally important challenge, and best practices are reviewed., Competing Interests: Funding Support and Author Disclosures Dr Opotowsky is supported by the National Heart, Lung, and Blood Institute of the National Institutes of Health under award number R01HL151604. Dr Marelli is supported by a Canadian Institutes of Health Research Foundation grant (no. 148462). The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Evidence from medicine and other fields has shown that gender diversity results in better decision making and outcomes. The incoming workforce of congenital heart specialists (especially in pediatric cardiology) appears to be more gender balanced, but past studies have shown many inequities. Gender-associated differences in leadership positions, opportunities presented for academic advancement, and recognition for academic contributions to the field persist. In addition, compensation packages remain disparate if evaluated based on gender with equivalent experience and expertise. This review explores these inequities and has suggested individual and institutional changes that could be made to recruit and retain women, monitor the climate of the institution, and identify and eliminate bias in areas like salary and promotions.

Introduction: Disparities in CHD outcomes exist across the lifespan. However, less is known about disparities for patients with CHD admitted to neonatal ICU. We sought to identify sociodemographic disparities in neonatal ICU admissions among neonates born with cyanotic CHD., Materials & Methods: Annual natality files from the US National Center for Health Statistics for years 2009-2018 were obtained. For each neonate, we identified sex, birthweight, pre-term birth, presence of cyanotic CHD, and neonatal ICU admission at time of birth, as well as maternal age, race, ethnicity, comorbidities/risk factors, trimester at start of prenatal care, educational attainment, and two measures of socio-economic status (Special Supplemental Nutrition Program for Women, Infants, and Children [WIC] status and insurance type). Multivariable logistic regression models were fit to determine the association of maternal socio-economic status with neonatal ICU admission. A covariate for race/ethnicity was then added to each model to determine if race/ethnicity attenuate the relationship between socio-economic status and neonatal ICU admission., Results: Of 22,373 neonates born with cyanotic CHD, 77.2% had a neonatal ICU admission. Receipt of WIC benefits was associated with higher odds of neonatal ICU admission (adjusted odds ratio [aOR] 1.20, 95% CI 1.1-1.29, p < 0.01). Neonates born to non-Hispanic Black mothers had increased odds of neonatal ICU admission (aOR 1.20, 95% CI 1.07-1.35, p < 0.01), whereas neonates born to Hispanic mothers were at lower odds of neonatal ICU admission (aOR 0.84, 95% CI 0.76-0.93, p < 0.01)., Conclusion: Maternal Black race and low socio-economic status are associated with increased risk of neonatal ICU admission for neonates born with cyanotic CHD. Further work is needed to identify the underlying causes of these disparities.

Over the past decade, new research has advanced scientific knowledge of neurodevelopmental trajectories, factors that increase neurodevelopmental risk, and neuroprotective strategies for individuals with congenital heart disease. In addition, best practices for evaluation and management of developmental delays and disorders in this high-risk patient population have been formulated based on literature review and expert consensus. This American Heart Association scientific statement serves as an update to the 2012 statement on the evaluation and management of neurodevelopmental outcomes in children with congenital heart disease. It includes revised risk categories for developmental delay or disorder and an updated list of factors that increase neurodevelopmental risk in individuals with congenital heart disease according to current evidence, including genetic predisposition, fetal and perinatal factors, surgical and perioperative factors, socioeconomic disadvantage, and parental psychological distress. It also includes an updated algorithm for referral, evaluation, and management of individuals at high risk. Risk stratification of individuals with congenital heart disease with the updated categories and risk factors will identify a large and growing population of survivors at high risk for developmental delay or disorder and associated impacts across the life span. Critical next steps must include efforts to prevent and mitigate developmental delays and disorders. The goal of this scientific statement is to inform health care professionals caring for patients with congenital heart disease and other key stakeholders about the current state of knowledge of neurodevelopmental outcomes for individuals with congenital heart disease and best practices for neuroprotection, risk stratification, evaluation, and management.

Achieving health equity in populations with congenital heart disease (CHD) requires recognizing existing disparities throughout the lifespan that negatively and disproportionately impact specific groups of individuals. These disparities occur at individual, institutional, or system levels and often result in increased morbidity and mortality for marginalized or racially minoritized populations (population subgroups (e.g., ethnic, racial, social, religious) with differential power compared to those deemed to hold the majority power in the population). Creating actionable strategies and solutions to address these health disparities in patients with CHD requires critically examining multilevel factors and health policies that continue to drive health inequities, including varying social determinants of health (SDOH), systemic inequities, and structural racism. In this comprehensive review article, we focus on health equity solutions and health policy considerations for minoritized and marginalized populations with CHD throughout their lifespan in the United States. We review unique challenges that these populations may face and strategies for mitigating disparities in lifelong CHD care. We assess ways to deliver culturally competent CHD care and to help lower-health-literacy populations navigate CHD care. Finally, we review system-level health policies that impact reimbursement and research funding, as well as institutional policies that impact leadership diversity and representation in the workforce.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains., (Copyright © 2023 [Author/Employing Institution]. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate the association between race/ethnicity, poverty, and mental health in youth with chronic conditions., Study Design: A cross-sectional comparative study was performed using the records of a tertiary care center from 2011 to 2015., Inclusion Criteria: children aged 4-17 years with ≥1 hospitalization or emergency department visit. Exclusion criteria were those with arrhythmias or treatment with clonidine/benzodiazepines. The primary outcome variable was diagnosis or medication for anxiety, depression, or attention deficit hyperactivity disorder. The primary predictor variable was diagnosis of cystic fibrosis (CF), sickle cell disease (SCD), or congenital heart disease (CHD)., Results: We identified 112 313 patients, 0.2% with CF, 0.4% with SCD, and 1.0% with CHD. Patients with CF had the highest prevalence (23%) and odds (OR, 4.21; 95% CI, 3.07-5.77) of anxiety or depression, whereas patients with SCD had the lowest prevalence (7%) and odds (OR, 1.54; 95% CI, 1.11-2.14). Those with CHD had a prevalence of up to 17%, with 3-4 times higher odds of anxiety or depression (OR, 3.70; 95% CI, 2.98-4.61). All non-White participants were less likely to be diagnosed or treated for anxiety or depression and attention deficit hyperactivity disorder. Although poverty increased the probability of anxiety or depression in patients with CHD, this finding was not seen in patients with CF or SCD., Conclusions: Children with CF, SCD, and CHD are at increased risk of anxiety or depression; however non-White patients are likely being underdiagnosed and undertreated. Increased screening and recognition in minority children are needed to decrease disparities in mental health outcomes., (Copyright © 2023 Elsevier Inc. All rights reserved.)

The prevalence of obesity in children with congenital heart disease (CHD) is greater than 25%, putting these patients at-risk for increased surgical morbidity and mortality. Our goal was to determine the association between CHD complexity, sociodemographic factors, and obesity. Our hypothesis was that among CHD patients, the odds of obesity would be highest in older children with simple CHD, and in all children with a lower socioeconomic status. We conducted a retrospective cohort study, reviewing electronic medical records of children aged 2-17 years from over 50 outpatient pediatric clinics in Houston, TX. Children were classified as simple or moderate/complex CHD, and obesity was defined by BMI ≥ 95th percentile for age and sex. Logistic regression was used to determine the association between sociodemographic factors and CHD complexity with obesity. We identified 648 CHD and 369,776 non-CHD patients. Children with simple CHD had a similar odds of obesity as non-CHD children. Children with CHD had a higher prevalence of obesity if they were older, male, Black, Hispanic, and publicly insured. Children with moderate/complex CHD had lower odds of obesity [OR 0.24 (95% CI 0.07-0.73)], however their predicted probability of obesity approached that of the general population as they aged. Additionally, there was an incremental relationship with poverty and obesity [1.01 (1.01-1.01)]. Awareness of which patients with CHD are at highest risk of obesity may help in targeting interventions to assist at-risk patients maintain a healthy lifestyle., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Competing Interests: Disclosures None.

The COVID-19 pandemic restricted in-person appointments and prompted an increase in remote healthcare delivery. Our goal was to assess access to remote care for complex pediatric cardiology patients. We performed a retrospective chart review of Texas Children's Hospital (TCH) pediatric cardiology outpatient appointments from March 2020 to December 2020 for established congenital heart disease (CHD) patients 1 to 17 yo. Primary outcome variables were remote care use of telemedicine and patient portal activation. Primary predictor variables were age, sex, insurance, race/ethnicity, language, and location. Descriptive statistics were used to analyze patient demographics. Multivariate logistic regression determined associations with remote care use (p < 0.05). We identified 5,410 established patients with clinic appointments during the identified timeframe. Adopters of telemedicine included 13% of patients (n = 691). Of the prior non patient portal users, 4.5% activated their accounts. On multivariate analysis, older age (10-17 yo) was associated with increased telemedicine (OR 2.04, 95%CI 1.71, 2.43) and patient portal use (OR 1.70, 95%CI 1.33, 2.17). Public insurance (OR 1.66, 95%CI 1.25, 2.20) and Spanish speaking were associated with increased patient portal adoption. Race/ethnicity was not significantly associated with telemedicine use or patient portal adoption. Telehealth adoption among older children may be indicative of their ability to aid in the use of these technologies. Higher participation in patient portal activation among publicly insured and Spanish speaking patients is encouraging and demonstrates ability to navigate some degree of remote patient care. Adoption of remote patient care may assist in reducing access to care disparities., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Research has led to major achievements in public policy and child health. Despite the gains, the need for research to inform policy remains paramount against a backdrop of inadequate public health investments, health inequities, and public skepticism toward science. However, the translation of research into child health policy has often been slow due to misalignments in incentives between researchers and policy makers and a paucity of conceptual models to inform translation. This article outlines barriers to translation, provides examples of discordance between evidence and policy, summarizes models to inform translation, and offers strategies to improve translation of research to policy., Competing Interests: Disclosure The authors have no commercial or financial relationships relevant to this article to disclose. Potential conflict of interest The authors have no conflicts of interest to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

The National Heart, Lung, and Blood Institute convened a workshop in August 2021 to identify opportunities in pediatric and congenital cardiovascular research that would improve outcomes for individuals with congenital heart disease across the lifespan. A subsidiary goal was to provide feedback on and visions for the Pediatric Heart Network. This paper summarizes several key research opportunities identified in the areas of: data quality, access, and sharing; aligning cardiovascular research with patient priorities (eg, neurodevelopmental and psychological impacts); integrating research within clinical care and supporting implementation into practice; leveraging creative study designs; and proactively enriching diversity of investigators, participants, and perspectives throughout the research process., Competing Interests: Funding Support and Author Disclosures Dr Opotowsky is supported by the Heart Institute Research Core at Cincinnati Children's Hospital, and the Samuel and Molly Kaplan Fund for Adult Congenital Heart Disease. Dr Miyamoto is supported by the Children's Hospital Colorado Jack Cooper Millisor Chair in Pediatric Heart Disease. The views expressed in this manuscript are those of the authors and do not necessarily represent the views of the National Heart, Lung, and Blood Institute; the National Institutes of Health; the U.S. Department of Health and Human Services; or of other institutions. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. All rights reserved.)

Objective: Congenital heart disease (CHD) is a leading cause of premature death in infants and children. Currently limited data are available regarding lesion specific mortality over time. Our study aimed to describe pediatric mortality trends by CHD lesion in the United States., Methods: We conducted a 19 year analysis (1999 to 2017) of publicly available, deidentified multiple cause of death data compiled and produced by the National Center for Health Statistics. Analysis was stratified by CHD diagnosis and age using 3 age categories (infants, 1 to 4 years, and 5 to 17 years). Temporal trends of CHD mortality and the effect of contributing risk factors were analyzed by using joinpoint regression., Results: Mortality was highest for in infants for all CHD lesions, in particular for total anomalous pulmonary venous return. Significant declines in infant CHD mortality occurred for most other lesions. Contributing risk factors, including prematurity, extracardiac birth defects, and genetic conditions, occurred in 19% of infant CHD deaths and demonstrated worse mortality trends in the majority of lesions. Mortality rates remained highest for single ventricle lesions in all ages, with an infant mortality rate plateau in the later half of the study and progressive increasing mortality rates for children 5 to 17 years., Conclusions: CHD mortality is decreasing for most lesions. Because of the heterogenicity of CHD lesions, there is expected variability in mortality trends by lesion and age group. Single ventricle lesions continue to contribute most heavily to premature death because of CHD demonstrated by significant increases in mortality rate for children aged 5 to 17 years., (Copyright © 2022 by the American Academy of Pediatrics.)

Background: Adults with congenital heart disease (ACHD) comprise an increasing proportion of individuals living with CHD in the United States (US) although little is known regarding lesion or age-specific mortality in the modern era. Our study aimed to describe current era ACHD mortality rates by age and CHD lesion and provide temporal mortality rate trends for the last two decades., Methods: We conducted a 19-year analysis (1999-2017) of publicly available, de-identified Multiple Cause of Death data compiled and produced by the National Center for Health Statistics. Age and lesion-specific mortality rates were calculated using joinpoint regression., Results: ACHD mortality rates decreased by an average of 2%-4% per year for all adults. CHD lesions resulting in the highest ACHD-related mortality varied by age. Unlike the other lesions, mortality attributed to single ventricle physiology failed to improve in early adulthood (average of 1.6% increase per year). In decedents age 65 years or older, simpler forms of CHD like shunt lesions became more prominent contributors, accounting for 46% of deaths., Conclusion: Rates of mortality due to ACHD have declined significantly for adults with CHD, however, continued mortality due to single ventricle physiology remains an area requiring improved strategies to increase survival., (© 2022 Wiley Periodicals LLC.)

Studies describing gaps in care for youth with congenital heart disease (CHD), focus on those who have returned to care, but rarely those actively missing from care. Our objective was to determine barriers for young adults with CHD actively missing from cardiac care and to re-engage them in care. Retrospective single-center cohort study of cardiology clinic patients ages 15-21 years with CHD between 2012 and 2019 for patients actively missing from care (≥ 12 months beyond requested clinic follow-up). We conducted prospective interviews, offered clinic scheduling information, and recorded cardiac follow-up. Data analyzed using descriptive statistics, univariable, and multivariable logistic regression. Of 1053 CHD patients, 33% (n = 349) were actively missing. Of those missing, 58% were male and median age was 17 years (IQR 16-19). Forty-six percent were Non-Hispanic White, 33% Hispanic, and 9% Black. Moderately complex CHD was in 71%, and 62% had private insurance. Patients with simple CHD, older age at last encounter (18-21), and scheduled follow-up > 12 months from last encounter were more likely to be actively missing. Interviews were completed by 125 patients/parents (36%). Lack of cardiac care was reported in 52%, and common barriers included: insurance (33%), appointment scheduling (26%), and unknown ACHD center care (15%). Roughly half (55%) accepted appointment information, yet only 3% successfully returned. Many patients require assistance beyond CHD knowledge to maintain and re-engage in care. Future interventions should include scheduling assistance, focused insurance maintenance, understanding where to obtain ACHD care, and educating on need for lifelong care., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: To summarize existing literature on neonatal disparities in congenital heart disease surgical outcomes and identify potential policies to address these disparities., Finding: Disparities in outcomes for neonatal congenital heart surgery were largely published under four domains: race/ethnicity, insurance type, neighborhood/socioeconomic status, and cardiac center characteristics. While existing research identifies associations between these domains and mortality, more nuanced analyses are emerging to understand the mediators between these domains and other non-mortality outcomes, as well as potential interventions and policies to reduce disparities. A broader look into social determinants of health (SDOH), prenatal diagnosis, proximity of birth to a cardiac surgical center, and post-surgical outpatient and neurodevelopmental follow-up may accelerate interventions to mitigate disparities in outcomes., Conclusion: Understanding the mechanisms of how SDOH relate to neonatal surgical outcomes is paramount, as disparities research in neonatal congenital heart surgery continues to shift from identification and description, to intervention and policy., Competing Interests: Declaratrion of Competing Interest The authors report no proprietary or commercial interest in any product mentioned or concept discussed in this article., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Despite the overall improvement in life expectancy of patients living with congenital heart disease (congenital HD), disparities in morbidity and mortality remain throughout the lifespan. Longstanding systemic inequities, disparities in the social determinants of health, and the inability to obtain quality lifelong care contribute to poorer outcomes. To work toward health equity in populations with congenital HD, we must recognize the existence and strategize the elimination of inequities in overall congenital HD morbidity and mortality, disparate health care access, and overall quality of health services in the context of varying social determinants of health, systemic inequities, and structural racism. This requires critically examining multilevel contributions that continue to facilitate health inequities in the natural history and consequences of congenital HD. In this scientific statement, we focus on population, systemic, institutional, and individual-level contributions to health inequities from prenatal to adult congenital HD care. We review opportunities and strategies for improvement in lifelong congenital HD care based on current public health and scientific evidence, surgical data, experiences from other patient populations, and recognition of implicit bias and microaggressions. Furthermore, we review directions and goals for both quantitative and qualitative research approaches to understanding and mitigating health inequities in congenital HD care. Finally, we assess ways to improve the diversity of the congenital HD workforce as well as ethical guidance on addressing social determinants of health in the context of clinical care and research.

It is now expected that most individuals with congenital heart disease will survive to adulthood, including those with complex heart conditions. Maintaining lifelong medical care requires those with congenital heart disease to eventually transfer from pediatric to adult-oriented health care systems. Developing health care transition skills and gaining independence in managing one's own health care is imperative to this process and to ongoing medical and psychosocial success. This scientific statement reviews the recent evidence regarding transition and provides resources, components, and suggestions for development of congenital heart disease transition programs with the goals of improving patient knowledge, self-management, and self-efficacy skills to the level they are capable to eventually integrate smoothly into adult-oriented health care. Specifically, the scientific statement updates 3 sections relevant to transition programming. First, there is a review of specific factors to consider, including social determinants of health, psychosocial well-being, and neurocognitive status. The second section reviews costs of inadequate transition including the public health burden and the impairment in individual quality of life. Finally, the last section discusses considerations and suggestions for transition program design including communication platforms, a family-centered approach, and individual models. Although this scientific statement reviews recent literature surrounding transitions of care for individuals with congenital heart disease there remain significant knowledge gaps. As a field, we have yet to determine ideal timing and methods of transition, and barriers to transition and transfer remain, particularly for the underserved populations. The consequences of poor health care transition are great and garnering outcomes and information through organized, multifaceted, collaborative approaches to transition is critical to improving the lifelong care of individuals with congenital heart disease.

The congenital heart care community faces a myriad of public health issues that act as barriers toward optimum patient outcomes. In this article, we attempt to define advocacy and policy initiatives meant to spotlight and potentially address these challenges. Issues are organized into the following 3 key facets of our community: patient population, health care delivery, and workforce. We discuss the social determinants of health and health care disparities that affect patients in the community that require the attention of policy makers. Furthermore, we highlight the many needs of the growing adults with congenital heart disease and those with comorbidities, highlighting concerns regarding the inequities in access to cardiac care and the need for multidisciplinary care. We also recognize the problems of transparency in outcomes reporting and the promising application of telehealth. Finally, we highlight the training of providers, measures of productivity, diversity in the workforce, and the importance of patient-family centered organizations in advocating for patients. Although all of these issues remain relevant to many subspecialties in medicine, this article attempts to illustrate the unique needs of this population and highlight ways in which to work together to address important opportunities for change in the cardiac care community and beyond. This article provides a framework for policy and advocacy efforts for the next decade.

Diagnosis of CHD substantially affects parent mental health and family functioning, thereby influencing child neurodevelopmental and psychosocial outcomes. Recognition of the need to proactively support parent mental health and family functioning following cardiac diagnosis to promote psychosocial adaptation has increased substantially over recent years. However, significant gaps in knowledge remain and families continue to report critical unmet psychosocial needs. The Parent Mental Health and Family Functioning Working Group of the Cardiac Neurodevelopmental Outcome Collaborative was formed in 2018 through support from an R13 grant from the National Heart, Lung, and Blood Institute to identify significant knowledge gaps related to parent mental health and family functioning, as well as critical questions that must be answered to further knowledge, policy, care, and outcomes. Conceptually driven investigations are needed to identify parent mental health and family functioning factors with the strongest influence on child outcomes, to obtain a deeper understanding of the biomarkers associated with these factors, and to better understand how parent mental health and family functioning influence child outcomes over time. Investigations are also needed to develop, test, and implement sustainable models of mental health screening and assessment, as well as effective interventions to optimise parent mental health and family functioning to promote psychosocial adaptation. The critical questions and investigations outlined in this paper provide a roadmap for future research to close gaps in knowledge, improve care, and promote positive outcomes for families of children with CHD.

Background: Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada., Methods: In this retrospective cohort study, fetuses and infants <2 months of age with HLHS or TGA admitted between 2012 and 2016 to participating Fetal Heart Society Research Collaborative institutions in the United States and Canada were included. SEQ, rural residence, and distance of residence were derived using maternal census tract from the maternal address at first visit. Subjects were assigned a SEQ z score using the neighborhood summary score or Canadian Chan index and separated into quartiles. Insurance type and self-reported race and ethnicity were obtained from medical charts. We evaluated associations among SEQ, insurance type, race and ethnicity, rural residence, and distance of residence with PND of HLHS and TGA (aggregate and individually) using bivariate analysis with adjusted associations for confounding variables and cluster analysis for centers., Results: Data on 1862 subjects (HLHS: n=1171, 92% PND; TGA: n=691, 58% PND) were submitted by 21 centers (19 in the United States). In the United States, lower SEQ was associated with lower PND in HLHS and TGA, with the strongest association in the lower SEQ of pregnancies with fetal TGA (quartile 1, 0.78 [95% CI, 0.64-0.85], quartile 2, 0.77 [95% CI, 0.64-0.93], quartile 3, 0.83 [95% CI, 0.69-1.00], quartile 4, reference). Hispanic ethnicity (relative risk, 0.85 [95% CI, 0.72-0.99]) and rural residence (relative risk, 0.78 [95% CI, 0.64-0.95]) were also associated with lower PND in TGA. Lower SEQ was associated with later PND overall; in the United States, rural residence and public insurance were also associated with later PND., Conclusions: We demonstrate that lower SEQ, Hispanic ethnicity, and rural residence are associated with decreased PND for TGA, with lower SEQ also being associated with decreased PND for HLHS. Future work to increase PND should be considered in these specific populations.

Background: Data are lacking regarding the insurance status of adults with congenital heart disease (ACHD). We investigated whether the Affordable Care Act (ACA) impacted insurance status among hospitalized ACHD, identified associated sociodemographic factors, and compared coverage to adults with other chronic childhood conditions., Methods: Serial cross-sectional analysis of National Inpatient Sample hospitalizations from 2007 to 2016 was performed for patients 18-64 years old. ACHD were identified using ICD-9/10-CM codes and compared to patients with sickle cell disease (SCD), cystic fibrosis (CF), and the general population. Age was dichotomized as 18-25 years (transition aged) or 26-64 years. Groups were compared by era (pre-ACA [January 2007-June 2010]; early-ACA [July 2010-December 2013], which eliminated pre-existing condition exclusions; and full-ACA [January 2014-December 2016]) using interrupted time series and multivariable Poisson regression analyses., Results: Overall, uninsured hospitalizations decreased from pre-ACA (12.0%) to full-ACA (8.5%). After full ACA implementation, ACHD had lower uninsured rates than the general hospitalized population (6.0 vs. 8.6%, p < .01), but higher rates than those with other chronic childhood diseases (SCD [4.5%]; CF [1.6%]). Across ACA eras, transition aged ACHD had higher uninsured rates than older patients (8.9 vs. 7.6%, p < .01), and Hispanic patients remained less insured than other groups., Conclusions: Hospitalized ACHD were better insured than the general population but less insured than those with SCD or CF. Full ACA implementation was associated with improved insurance coverage for all groups, but disparities persisted for transition aged and Hispanic patients. Ongoing evaluation of the effects of insurance and health policy on ACHD remains critical to diminish health disparities., (© 2021 Wiley Periodicals LLC.)

Although evidence suggests that neighborhood context, particularly socioeconomic context, influences child obesity, little is known about how these neighborhood factors may be heterogeneous rather than monolithic. Using a novel dataset comprised of the electronic medical records for over 250,000 children aged 2-17 nested within 992 neighborhoods in the greater Houston area, we assessed whether neighborhoods influenced the obesity of children differently based on sex. Results indicated that neighborhood disadvantage, assessed using a comprehensive, multidimensional, latent profile analysis-generated measure, had a strong, positive association with the odds of obesity for both boys and girls. Interactions revealed that the relationship between disadvantage and obesity was stronger for girls, relative to boys. Our findings demonstrated the complex dynamics underlying the influence of residential neighborhood context on obesity for specific subgroups of children., (Copyright © 2021. Published by Elsevier Ltd.)

Background: Data on anxiety, depression, and attention-deficit/hyperactivity disorder (ADHD) are lacking for youth with congenital heart disease (CHD), particularly those with simple CHD. This study aims to characterize these disorders in youth with CHD compared to those without CHD., Methods: A comparative cross-sectional study was conducted by using the electronic medical records of a large tertiary care hospital between 2011 and 2016. Inclusion criteria were youth aged 4 to 17 years with >1 hospitalization or emergency department visits. Exclusion criteria were patients with arrhythmias or treatment with clonidine and/or benzodiazepines. The primary predictor variable was CHD type: simple, complex nonsingle ventricle, and complex single ventricle. The primary outcome variable was a diagnosis and/or medication for anxiety and/or depression or ADHD. Data were analyzed by using logistic regression (Stata v15; Stata Corp, College Station, TX)., Results: We identified 118 785 patients, 1164 with CHD. Overall, 18.2% ( n = 212) of patients with CHD had a diagnosis or medication for anxiety or depression, compared with 5.2% ( n = 6088) of those without CHD. All youth with CHD had significantly higher odds of anxiety and/or depression or ADHD. Children aged 4 to 9 years with simple CHD had ∼5 times higher odds (odds ratio: 5.23; 95% confidence interval: 3.87-7.07) and those with complex single ventricle CHD had ∼7 times higher odds (odds ratio: 7.46; 95% confidence interval: 3.70-15.07) of diagnosis or treatment for anxiety and/or depression. Minority and uninsured youth were significantly less likely to be diagnosed or treated for anxiety and/or depression or ADHD, regardless of disease severity., Conclusions: Youth with CHD of all severities have significantly higher odds of anxiety and/or depression and ADHD compared to those without CHD. Screening for these conditions should be considered in all patients with CHD., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

Background: Congenital heart disease (CHD) accounts for ≈40% of deaths in US children with birth defects. Previous US data from 1999 to 2006 demonstrated an overall decrease in CHD mortality. Our study aimed to assess current trends in US mortality related to CHD from infancy to adulthood over the past 19 years and determine differences by sex and race/ethnicity., Methods: We conducted an analysis of death certificates from 1999 to 2017 to calculate annual CHD mortality by age at death, race/ethnicity, and sex. Population estimates used as denominators in mortality rate calculations for infants were based on National Center for Health Statistics live birth data. Mortality rates in individuals ≥1 year of age used US Census Bureau bridged-race population estimates as denominators. We used joinpoint regression to characterize temporal trends in all-cause mortality, mortality resulting directly attributable to and related to CHD by age, race/ethnicity, and sex., Results: There were 47.7 million deaths with 1 in 814 deaths attributable to CHD (n=58 599). Although all-cause mortality decreased 16.4% across all ages, mortality resulting from CHD declined 39.4% overall. The mean annual decrease in CHD mortality was 2.6%, with the largest decrease for those >65 years of age. The age-adjusted mortality rate decreased from 1.37 to 0.83 per 100 000. Males had higher mortality attributable to CHD than females throughout the study, although both sexes declined at a similar rate (≈40% overall), with a 3% to 4% annual decrease between 1999 and 2009, followed by a slower annual decrease of 1.4% through 2017. Mortality resulting from CHD significantly declined among all races/ethnicities studied, although disparities in mortality persisted for non-Hispanic Blacks versus non-Hispanic Whites (mean annual decrease 2.3% versus 2.6%, respectively; age-adjusted mortality rate 1.67 to 1.05 versus 1.35 to 0.80 per 100 000, respectively)., Conclusions: Although overall US mortality attributable to CHD has decreased over the past 19 years, disparities in mortality persist for males in comparison with females and for non-Hispanic Blacks in comparison with non-Hispanic Whites. Determining factors that contribute to these disparities such as access to quality care, timely diagnosis, and maintenance of insurance will be important moving into the next decade.

Objective: To examine state-wide population trends in preterm delivery of children with critical congenital heart disease (CHD) over an 18-year period. We hypothesized that, coincident with early advancements in prenatal diagnosis, preterm delivery initially increased compared with the general population, and more recently has decreased., Study Design: Data from the Texas Public Use Data File 1999-2016 was used to evaluate annual percent preterm delivery (<37 weeks) in critical CHD (diagnoses requiring intervention at <1 year of age). We first evaluated for pattern change over time using joinpoint segmented regression. Trends in preterm delivery were then compared with all Texas livebirths. We then compared trends examining sociodemographic covariates including race/ethnicity, sex, and neighborhood poverty levels., Results: Of 7146 births with critical CHD, 1339 (18.7%) were delivered preterm. The rate of preterm birth increased from 1999 to 2004 (a mean increase of 1.69% per year) then decreased between 2005 and 2016 (a mean decrease of -0.41% per year). This represented a faster increase and then a similar decrease to that noted in the general population. Although the greatest proportion of preterm births occurred in newborns of Hispanic ethnicity and non-Hispanic black race, newborns with higher neighborhood poverty level had the most rapidly increasing rate of preterm delivery in the first era, and only a plateau rather than decrease in the latter era., Conclusions: Rates of preterm birth for newborns with critical CHD in Texas first were increasing rapidly, then have been decreasing since 2005., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with congenital heart disease (CHD). Little is known about the adult HS population with CHD., Objective: To describe the outcomes and sociodemographics of the adult CHD population with HS., Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed., Results: Sixty-two patients met inclusion criteria. Median age was 22.7 [IQR 19.6-30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap in care of >3 years. Median follow-up time in adulthood was 2.9 [IQR 1.3-8.2] years. Forty-three (69%) of patients had single ventricle heart disease, 31 (71%) of whom completed Fontan circulation. A total of 36 interventions occurred in 24 patients which included 16 cardiac catherization interventions, 13 electrophysiology-related procedures, and 18 surgical procedures including 2 orthotopic heart transplants. The median age for death or heart transplant was 45.3 (95%CI 34.3-56.1) years. Heart failure-free survival was 80.8 ± 5.2%, 58.7 ± 11.0%, and 31.1 ± 15.7% at 20, 30, and 40 years old, respectively. Cerebrovascular accident-free survival was 84.3 ± 5.1%, 54.2 ± 11.3%, and 40.6 ± 14.5% at 20, 30, and 40 years old, respectively. Tachyarrhythmia-free survival was 54.0 ± 7.1%, 29.2 ± 8.3%, and 19.5 ± 9.7% at 20, 30, and 40 years old and bradyarrhythmia-free survival was 66.0 ± 6.3%, 41.7 ± 9.4%, and 33.4 ± 10.6% at ages 20, 30, and 40 years, respectively., Conclusions: At a tertiary referral center, adult patients with CHD and HS have high rates of comorbidities and early death or heart transplant. Longitudinal surveillance and further exploration into factors associated with improved survival in this population are warranted., (© 2019 Wiley Periodicals, Inc.)

Background: Follow-up at a regional adult congenital heart disease (ACHD) center is recommended for all ACHD patients at least once per the 2018 ACC/AHA guidelines. Other specialties have demonstrated poorer follow-up and outcomes correlating with increased distance from health care providers, but driving time to regional ACHD centers has not been examined in the US population., Objective: To identify and characterize potential disparities in access to ACHD care in the US based on drive time to ACHD centers and compounding sociodemographic factors., Methods: Mid- to high-volume ACHD centers with ≥500 outpatient ACHD visits and ≥20 ACHD surgeries annually were included based on self-reported, public data. Geographic Information System mapping was used to delineate drive times to ACHD centers. Sociodemographic data from the 2012-2016 American Community Survey (US Census) and the Environmental Systems Research Institute were analyzed based on drive time to nearest ACHD center. Previously established CHD prevalence estimates were used to estimate the similarly located US ACHD population., Results: Nearly half of the continental US population (45.1%) lives >1 hour drive to an ACHD center. Overall, 39.7% live 1-4 hours away, 3.4% live 4-6 hours away, and 2.0% live >6 hours away. Hispanics were disproportionately likely to live a >6 hour drive to a center (p < .001). Compared to people with <1 hour drive, those living >6 hours away have higher proportions of uninsured adults (29% vs. 18%; p < .001), households below the federal poverty level (19% vs. 13%; p < .001), and adults with less than college education (18% vs. 12%; p < .001)., Conclusions: We estimate that ~45% of the continental US population lives >1 hour to an ACHD center, with 5.4% living >4 hours away. Compounding barriers exist for Hispanic, uninsured, lower socioeconomic status, and less-educated patients. These results may help drive future policy changes to improve access to ACHD care., (© 2019 Wiley Periodicals, Inc.)

Purpose: The purpose of this study was to determine the accuracy of fetal echocardiogram (ECHO) for detecting cardiac structural anomalies that may impact Extracorporeal Membrane Oxygenation (ECMO) candidacy in infants with Congenital Diaphragmatic Hernia (CDH)., Methods: A retrospective review was performed on fetuses with CDH (January 2007-June 2017). Inclusion criteria were inborn and at least one prenatal and postnatal ECHO. ECHOs were evaluated for structural heart defects. Primary outcomes were accuracy of prenatal fetal ECHO and identify differences between prenatal and postnatal ECHO. Descriptive statistics and Chi-square analysis were performed., Results: 131 inborn patients were identified. Mean gestational age of fetal ECHO was 26.6 ± 5.5 weeks. The median time to postnatal ECHO was DOL 1 [0-30]. Fetal ECHO had 92% accuracy, 83% sensitivity, 93% specificity, PPV of 95%, NPV of 92%, and a 90% accuracy for visualization of at least one pulmonary vein into the left atrium on the contralateral (non-CDH) side. Thirty-five percent of patients received ECMO, and 26% had an associated cardiac anomaly. All ECMO patients had an accurate structural fetal ECHO., Conclusion: Fetal ECHO is sufficient for identifying major structural heart defects at large volume centers with trained pediatric cardiologists and may be used to guide clinical management, particularly in regards to ECMO candidacy., Level of Evidence: Level III., (Copyright © 2019. Published by Elsevier Inc.)

Aberrant left-right patterning in the developing human embryo can lead to a broad spectrum of congenital malformations. The causes of most laterality defects are not known, with variants in established genes accounting for <20% of cases. We sought to characterize the genetic spectrum of these conditions by performing whole-exome sequencing of 323 unrelated laterality cases. We investigated the role of rare, predicted-damaging variation in 1726 putative laterality candidate genes derived from model organisms, pathway analyses, and human phenotypes. We also evaluated the contribution of homo/hemizygous exon deletions and gene-based burden of rare variation. A total of 28 candidate variants (26 rare predicted-damaging variants and 2 hemizygous deletions) were identified, including variants in genes known to cause heterotaxy and primary ciliary dyskinesia (ACVR2B, NODAL, ZIC3, DNAI1, DNAH5, HYDIN, MMP21), and genes without a human phenotype association, but with prior evidence for a role in embryonic laterality or cardiac development. Sanger validation of the latter variants in probands and their parents revealed no de novo variants, but apparent transmitted heterozygous (ROCK2, ISL1, SMAD2), and hemizygous (RAI2, RIPPLY1) variant patterns. Collectively, these variants account for 7.1% of our study subjects. We also observe evidence for an excess burden of rare, predicted loss-of-function variation in PXDNL and BMS1- two genes relevant to the broader laterality phenotype. These findings highlight potential new genes in the development of laterality defects, and suggest extensive locus heterogeneity and complex genetic models in this class of birth defects.

Introduction: In a pilot study of chronic maternal hyperoxygenation (CMH) in left heart hypoplasia (LHH), we sought to determine effect estimates of CMH on head size, vascular resistance indices, and neurodevelopment compared to controls., Material and Methods: Nine gravidae meeting the inclusion criteria (fetal LHH, ≥25.9 weeks' gestation, and ≥10% increase in percent aortic flow after acute hyperoxygenation) were prospectively enrolled. Controls were 9 contemporary gravidae with fetal LHH without CMH. Brain growth and Doppler-derived estimates of fetal cerebrovascular and placental resistance were blindly evaluated and compared using longitudinal regression. Postnatal anthropomorphic and neurodevelopmental assessments were compared., Results: There was no difference in baseline fetal measures between groups. There was significantly slower biparietal diameter (BPD) growth in the CMH group (z-score change -0.03 ± 0.02 vs. +0.09 ± 0.05 units/week, p = 0.02). At 6 months postnatal age, the mean head circumference z-score in the CMH group was smaller than that of controls (-0.20 ± 0.58 vs. +0.85 ± 1.11, p = 0.048). There were no differences in neurodevelopmental testing at 6 and 12 months., Discussion: In this pilot study, relatively diminished fetal BPD growth and smaller infant head circumference z-scores at 6 months were noted with in utero CMH exposure., (© 2018 S. Karger AG, Basel.)

We investigate how distinct residential environments uniquely influence chronic child disease. Aggregating over 200,000 pediatric geocoded medical records to the census tract of residence and linking them to neighborhood-level measures, we use multiple data analysis techniques to assess how heterogeneous exposures of social and environmental neighborhood conditions influence an index of child chronic disease (CCD) prevalence for the neighborhood. We find there is a graded relationship between degree of overall neighborhood disadvantage and children's chronic disease such that the highest neighborhood CCD scores reside in communities with the highest concentrated disadvantage. Finally, results show that higher levels of neighborhood concentrated disadvantage and air pollution exposure associate with higher risks of having at least one chronic condition for children after also considering their individual- and family-level characteristics. Overall, our analysis serves as a comprehensive start for future researchers interested in assessing which neighborhood factors matter most for child chronic health conditions., Competing Interests: Disclosure: The authors report no conflicts of interest.

Aortopulmonary window (APW) is a rare form of congenital heart disease seen in isolation or with complex cardiac lesions. APW has been associated with other cardiac defects such as interrupted aortic arch and Tetralogy of Fallot, but few cases have been reported of APW associated with transposition of the great arteries (TGA). In a newborn with TGA and intact ventricular septum, diagnosis of APW requires a high index of suspicion. This article reviews the literature on TGA with APW and illustrates the importance of additional evaluation in neonates with TGA when oxygen saturation and PaO2 do not match predicted clinical values., (© 2018 Wiley Periodicals, Inc.)

Objectives: To determine the association of furosemide therapy with the incidence of bone fractures in children with congenital heart disease., Study Design: We conducted a retrospective cohort study with data extracted from the 2008-2014 Texas Medicaid databases. Pediatric patients aged <12 years diagnosed with congenital heart disease, cardiomyopathy, or heart failure were included. Patients taking furosemide were categorized into a furosemide-adherent group (medication possession ratio of ≥70%), and a furosemide-nonadherent group (medication possession ratio of <70%). A third group of patients was matched to the furosemide user groups by using propensity score matching. A multivariate logistic regression and Cox proportional hazard model with a Kaplan-Meier plot (time-to-fracture) were used to compare the 3 groups, controlling for baseline demographics and clinical characteristics., Results: After matching, 3912 patients (furosemide adherent, n = 254; furosemide nonadherent, n = 724; no furosemide, n = 2934) were identified. The incidence of fractures was highest for the furosemide-adherent group (9.1%; 23 of 254), followed by the furosemide-nonadherent group (7.2%; 52 of 724), which were both higher than for patients who did not receive furosemide (5.0%; 148 of 2934) (P < .001). Using logistic regression, both furosemide groups were more likely to have fractures than the no furosemide group: furosemide-adherent OR of 1.9 (95% CI, 1.17-2.98; P = .009); furosemide nonadherent OR of 1.5 (95% CI, 1.10-2.14; P = .01). In the Cox proportional hazard model, the risk of fractures for the furosemide-adherent group was significantly higher compared with the no furosemide group (HR, 1.6; 95% CI, 1.00-2.42; P = .04)., Conclusions: Furosemide therapy, even with nonconsistent dosing, was associated with an increased risk of bone fractures in children with congenital heart disease., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Congenital heart diseases (CHDs) are the most common type of birth defects. Improvements in CHD care have led to approximately 1.4 million survivors reaching adulthood. Successful transition and transfer from pediatric to adult care is crucial. Unfortunately, less than 30% of adolescents with CHD successfully transition to adult care; this number is lower for minority and lower socioeconomic status populations. Few CHD programs exist to facilitate successful transition., Objective: The goal of our study was to describe the formative research used to develop a prototype mobile app to facilitate transition to adult care for adolescents with CHD., Methods: A literature search about best practices in transition medicine for CHD was conducted to inform app development. Formative research with a diverse group of CHD adolescents and their parents was conducted to determine gaps and needs for CHD transition to adult care. As part of the interview, surveys assessing transition readiness and CHD knowledge were completed. Two adolescent CHD expert panels were convened to inform educational content and app design., Results: The literature review revealed 113 articles, of which 38 were studies on transition programs and attitudes and 3 identified best practices in transition specific to CHD. A total of 402 adolescents aged 15 to 22 years (median 16 years) participated in semistructured interviews. The group was racially and ethnically diverse (12.6% [51/402] African American and 37.8% [152/402] Latino) and 42.0% (169/402) female; 36.3% (146/402) received public insurance. Most adolescents (313/402, 76.7%) had moderate or severe CHD complexity and reported minimal CHD understanding (79.0% [275/348] of those aged 15 to 17 years and 61.1% [33/54] of those aged 18 to 22 years). Average initial transition readiness score was 50.9/100, meaning that transition readiness training was recommended. When participants with moderate to severe CHD (313/402, 77.9%) were asked about technology use, 94.2% (295/313) reported having access to a mobile phone. Interviews with parents revealed limited interactions with the pediatric cardiologist about transition-related topics: 79.4% (331/417) reported no discussions regarding future family planning, and 55.2% (230/417) reported the adolescent had not been screened for mental health concerns (depression, anxiety). Further, 66.4% (277/417) reported not understanding how health care changes as adolescents become adults. Adolescents in the expert panels (2 groups of 3 adolescents each) expressed interest in a CHD-specific tailored app consisting of quick access to specific educational questions (eg, "Can I exercise?"), a CHD story-blog forum, a mentorship platform, a question and answer space, and a checklist to facilitate transition. They expressed interest in using the app to schedule CHD clinic appointments and receive medication reminders. Based on this data, a prototype mobile app was created to assist in adolescent CHD transition., Conclusions: Formative research revealed that most adolescents with CHD had access to mobile phones, were not prepared for transition to adult care, and were interested in an app to facilitate transition to adult CHD care. Understanding adolescent and parent needs, interests, and concerns helped in the development of a mobile app with a broader, tailored approach for adolescents with CHD., Competing Interests: Conflicts of Interest None declared.

Objective: To examine the risk for cyanotic congenital heart diseases (CCHDs) among live births in the USA, resulting from various forms of infertility treatments., Methods: This study is a cross-sectional analysis of live births in the USA from 2011 to 2014. Infertility treatments are categorised into two of the following groups on birth certificates: assisted reproductive technology (ART) fertility treatment (surgical egg removal; eg, in vitro fertilisation and gamete intrafallopian transfer) and non-ART fertility treatment (eg, medical treatment and intrauterine insemination). We compared the risk for CCHD in ART and non-ART fertility treatment groups with those infants whose mothers received no documented fertility treatment and were naturally conceived (NC)., Results: Among 14 242 267 live births from 2011 to 2014, a total of 101 494 live births were in the ART and 81 242 resulted from non-ART fertility treatments. CCHD prevalence in ART, non-ART and NC groups were 393/100 892 (0.39%), 210/80 884 (0.26%) and 10 749/14 020 749 (0.08%), respectively. As compared with naturally conceiving infants, risk for CCHD was significantly higher among infants born in ART (adjusted relative risk (aRR) 2.4, 95% CI 2.1 to 2.7) and non-ART fertility treatment groups (aRR 1.9, 95% CI 1.6 to 2.2). Absolute risk increase in CCHD due to ART and non-ART treatments were 0.03% and 0.02%, respectively. A similar pattern was observed when the analysis was restricted to twins, newborns with birth weights under 1500 g and gestational age of less than 32 weeks., Conclusions: Our findings suggest an increased risk for CCHD in infants conceived after all types of infertility treatment., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: Previous studies demonstrate that infant and childhood mortality differ among children with birth defects by maternal race/ethnicity, but limited mortality information is published for Hispanic ethnic subgroups., Methods: We performed a retrospective cohort study using data for children with birth defects born to Hispanic mothers during 1999-2007 from 12 population-based state birth defects surveillance programs. Deaths were ascertained through multiple sources. Survival probabilities were estimated by the Kaplan-Meier method. Cox proportional hazards regression was used to examine the effect of clinical and demographic factors on mortality risk., Results: Among 28,497 Hispanic infants and children with major birth defects, 1-year survival was highest for infants born to Cuban mothers at 94.6% (95% confidence intervals [CI] 92.7-96.0) and the lowest for Mexicans at 90.2% (95% CI 89.7-90.6; p < .0001). For children aged up to 8 years, survival remained highest for Cuban Americans at 94.1% (95% CI 91.8-95.7) and lowest for Mexican Americans at 89.2% (95% CI 88.7-89.7; p = .0002). In the multivariable analysis using non-Hispanic White as the reference group, only infants and children born to Mexican mothers were noted to have a higher risk of mortality for cardiovascular defects., Conclusions: This analysis provides a better understanding of survival and mortality for Hispanic infants and children with selected birth defects. The differences found in survival, particularly the highest survival rates for Cuban American children and lowest for Mexican American children with birth defects, underscores the importance of assessing Hispanic ethnic subgroups, as differences among subgroups appear to exist., (© 2017 Wiley Periodicals, Inc.)

Objectives Previous research has established links between child, family, and neighborhood disadvantages and child asthma. We add to this literature by first characterizing neighborhoods in Houston, TX by demographic, economic, and air quality characteristics to establish differences in pediatric asthma diagnoses across neighborhoods. Second, we identify the relative risk of social, economic, and environmental risk factors for child asthma diagnoses. Methods We geocoded and linked electronic pediatric medical records to neighborhood-level social and economic indicators. Using latent profile modeling techniques, we identified Advantaged, Middle-class, and Disadvantaged neighborhoods. We then used a modified version of the Blinder-Oaxaca regression decomposition method to examine differences in asthma diagnoses across children in these different neighborhoods. Results Both compositional (the characteristics of the children and the ambient air quality in the neighborhood) and associational (the relationship between child and air quality characteristics and asthma) differences within the distinctive neighborhood contexts influence asthma outcomes. For example, unequal exposure to PM 2.5 and O 3 among children in Disadvantaged and Middle-class neighborhoods contribute to asthma diagnosis disparities within these contexts. For children in Disadvantaged and Advantaged neighborhoods, associational differences between racial/ethnic and socioeconomic characteristics and asthma diagnoses explain a significant proportion of the gap. Conclusions for Practice Our results provide evidence that differential exposure to pollution and protective factors associated with non-Hispanic White children and children from affluent families contribute to asthma disparities between neighborhoods. Future researchers should consider social and racial inequalities as more proximate drivers, not merely as associated, with asthma disparities in children.