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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.

Background and ObjectivesStudies 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.MethodsRetrospective single-center cohort study of cardiology clinic patients ages 15-21 years with CHD between 2012-2019 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 was analyzed using descriptive statistics, univariable, and multivariable logistic regression.ResultsOf 1053 CHD patients, 33% (n=349) were actively missing. Of those missing, 58% were male and median age was 17 yrs (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.ConclusionsMany 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.Article SummaryThere is a growing population of youth with congenital heart disease leaving cardiology care; we investigate patients actively missing from care and target their re-engagement.What’s Known on This Subject?Maintaining cardiac care is a problem for many patients with congenital heart disease, especially teens and young adults, and characteristics of both patients and their healthcare environment contribute to the maintenance and ability to continue appropriate cardiac care.What This Study AddsThis study uniquely investigates patients with congenital heart disease that are actively missing from care, and investigates if a simple intervention of contacting them while they are missing to re-engage them in care impacts their re-entry into cardiac care.Contributors’ Statement PageJudson Moore conceptualized and designed the study, designed data collection instruments, collected data, drafted the initial manuscript, and completed necessary revisions to final manuscript.Keila Lopez conceptualized and designed the study, designed data collection instruments, critically reviewed data, revised manuscript drafts, and provided content expertise.Shreya Sheth conceptualized and designed the study, critically reviewed data, and revised the manuscript.Wilson Lam contributed to study design, critically reviewed data for intellectual content, and revised the manuscript.Alexander Alexander, Andre Espaillat, and John Shabosky designed data collection instruments, collected data, and reviewed/revised the manuscript.Karla Dyer performed data extraction, data cleaning, and reviewed/revised the manuscript.Nicole Broussard and Donna Lovick provided expert knowledge for interpretation of data and reviewed/revised the manuscript.All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

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.

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.

Objective 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. Methods 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

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.

The 2nd annual Health Equity Summer Research Summit organized by the Center of Excellence (COE) in Health Equity, Training and Research, Baylor College of Medicine (BCM), was a forum to catalyze the fertilization and exchange of cutting-edge ideas in the area of disparity research in medicine. The summit fosters understanding of current health equity research, training, clinical care, and outreach initiatives, in addition to offering an opportunity for connecting with allies and partners working in the field of health equity and health disparities. The summit provides opportunities for our COE Scholars (undergraduate/post-baccalaureate students and Baylor medical students, clinical fellows and junior faculty) to showcase their research findings which are reflective of their past year's endeavor as scholars at the center. Abstracts in this Book of Abstracts represent a summary of these research efforts. With this summit, BCM continues to build on its long history of educational outreach initiatives to promote diversity in medicine by focusing on programs aimed at increasing the number of diverse and highly qualified medical professionals ready to introduce effective and innovative approaches to reduce or eliminate health disparities. These programs will improve information resources, clinical education, curricula, research and cultural competence as they relate to minority health issues and social determinants of health.

As the cardiac community strives to improve outcomes, accurate methods of risk stratification are imperative. Since adoption of International Classification of Disease-10th Revision (ICD-10) in 2015, there is no published method for congenital heart surgery risk stratification for administrative data.This study sought to develop an empirically derived, publicly available Risk Stratification for Congenital Heart Surgery (RACHS-2) tool for ICD-10 administrative data.The RACHS-2 stratification system was iteratively and empirically refined in a training dataset of Pediatric Health Information Systems claims to optimize sensitivity and specificity compared with corresponding locally held Society of Thoracic Surgeons-Congenital Heart Surgery (STS-CHS) clinical registry data. The tool was validated in a second administrative data source: New York State Medicaid claims. Logistic regression was used to compare the ability of RACHS-2 in administrative data to predict operative mortality vs STAT Mortality Categories in registry data.The RACHS-2 system captured 99.6% of total congenital heart surgery registry cases, with 1.0% false positives. RACHS-2 predicted operative mortality in both training and validation administrative datasets similarly to STAT Mortality Categories in registry data. C-statistics for models for operative mortality in training and validation administrative datasets-adjusted for RACHS-2-were 0.76 and 0.84 (95% CI: 0.72-0.80 and 0.80-0.89); C-statistics for models for operative mortality-adjusted for STAT Mortality Categories-in corresponding clinical registry data were 0.75 and 0.84 (95% CI: 0.71-0.79 and 0.79-0.89).RACHS-2 is a risk stratification system for pediatric cardiac surgery for ICD-10 administrative data, validated in 2 administrative-registry-linked datasets. Statistical code is publicly available upon request.

Introduction: Studies have demonstrated that preterm delivery (PTD) in infants with critical congenital heart disease (CCHD) is associated with worse outcomes compared to those delivered at term. PTD in singletons with CCHD is decreasing. However, little is known about PTD trends over time in multiple gestation deliveries (MGD) with CCHD. Hypothesis: We hypothesized that in a state population-based cohort over the last 20 years, PTD in MGD with CCHD initially increased compared to the general population, but more recently decreased coincident with advancements in prenatal detection of CCHD. Methods: The Texas Public Use Data File 1999-2018 was used to evaluate percent PTD ( Results: Of 7,428,329 livebirths from 1999-2018, 211,931 (2.8%) were MGD and of those 129,253 (61%) were PTD. Overall, the rate of PTD increased from 1999 to 2003 (mean increase of 1%/y) then remained constant 2003-2018 (Figure). Of 308 MGD with CCHD, 229 (74%) were PTD. The rate of PTD in CCHD decreased from 1999 to 2003 (mean decrease of 2.2%/y) then remained relatively constant 2003-2018. When examining by race/ethnicity, there was an overall decrease in PTD with CCHD in Non-Hispanic White and Hispanic MGD, but an increase in Non-Hispanic Black MGD (Figure). There was a decrease in PTD with CCHD for those with private insurance however an increase for those with public insurance (Figure). Conclusions: For MGD with CCHD, while PTD rates initially decreased, they have remained somewhat constant over the last 15 years. The rate of PTD in non-Hispanic Black MGD and in those publicly insured, however, remains the highest. Mechanisms for persisting disparities in PTD for these populations requires further investigation, including determining associations with other social determinants of health and implicit bias.

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. TheParent Mental Health and Family Functioning Working Groupof 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: Heterotaxy syndrome is a condition in which the thoracoabdominal organs demonstrate abnormal lateral arrangement and is often associated with congenital heart disease. Little is known about the adult heterotaxy syndrome population with congenital heart disease (CHD). OBJECTIVE: Describe the outcomes and sociodemographics of the adult CHD population with heterotaxy syndrome. METHODS: Records of patients 18 years of age or older with diagnoses of both congenital heart disease and heterotaxy syndrome at Texas Children’s Hospital from 1964–2018 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. 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. 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. CONCLUSIONS: At a tertiary referral center, adult patients with CHD and heterotaxy syndrome 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.

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.

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.

The literature on neighborhoods and child obesity links contextual conditions to risk, assuming that if place matters, it matters in a similar way for everyone in those places. We explore the extent to which distinctive neighborhood types give rise to social patterning that produces variation in the odds of child obesity. We leverage geocoded electronic medical records for a diverse sample of over 135,000 children aged 2 to 12 and latent profile modeling to characterize places into distinctive neighborhood contexts. Multilevel models with cross-level interactions between neighborhood type and family socioeconomic standing (SES) reveal that children with different SES, but living in the same neighborhoods, have different odds of obesity. Specifically, we find lower-SES children benefit, but to a lesser degree, from neighborhood advantages and higher-SES children are negatively influenced, to a larger degree, by neighborhood disadvantages. The resulting narrowing of the gap in obesity by neighborhood disadvantage helps clarify how place matters for children’s odds of obesity and suggests that efforts to improve access to community advantages as well as efforts to address community disadvantages are important to curbing obesity and improving the health of all children.

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 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 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.

Introduction: Many studies describe gaps in care for youth with congenital heart disease (CHD), but few reach out to patients actively missing from care. Our study goal was to identify young adults with CHD actively missing from cardiac care to: 1) administer a needs assessment and 2) re-engage patients in cardiac care. Methods: We performed a retrospective review of outpatient medical records (2012-2019) for patients 15-21 yrs with CHD at Texas Children’s Hospital. ICD 9/10 codes identified CHD patients; those ≥1 year beyond requested clinic follow-up were defined as ‘actively missing’. Sociodemographic data was collected, and a telephone needs assessment conducted to determine CHD knowledge, barriers to care, and health status. Either a patient or parent completed the survey and was offered to re-engage in cardiac care. Descriptive statistics were performed on available data. Results: Of 1057 CHD patients, 33% (n=353) were actively missing. Of those missing, 58% were male and median age was 17 yrs (IQR 16-19). Patients were 46% Non-Hispanic White, 9% Non-Hispanic Black, and 33% Hispanic. Moderately complex CHD was noted in 71%. Insurance status was 63% private and 31% public. Ninety-eight patients/parents (28%) participated in our survey. Fifty-two percent reported having no cardiac care, often citing lack of or incompatible hospital insurance (33%; n = 32). Active cardiac symptoms were reported in 25%. Half of patients felt they could explain their cardiac condition well, and 82% could name their medications. Scheduling barriers (26%; n = 25) and being unaware adults could be cared for at our heart center (15%; n = 15) were the most common obstacles to re-engaging in care. Participants felt text messaging and scheduling visits during cardiology clinic would improve care access. More than half of patients/parents (55%) accepted our help to re-engage in cardiac care (scheduling assistance). Conclusions: This data suggests patients require assistance beyond CHD knowledge to stay in care. Problems with insurance, scheduling, and perceptions of adult CHD care were key barriers to maintaining cardiac care despite moderate CHD complexity and active symptoms. Future quality initiatives should include scheduling, insurance coverage, and adult CHD services.

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 declined. STUDY DESIGN: Data from the Texas Public Use Data File 1999–2016 was used to evaluate annual percent preterm delivery (

BackgroundCongenital heart disease (CHD) accounts for approximately 40% percent of deaths in United States (US) children with birth defects. Previous US data from 1999-2006 demonstrated an overall decrease in CHD mortality. The objective of our study was to assess current trends in US mortality related to CHD from infancy to adulthood over the last 19 years and determine differences by sex and race/ethnicity.MethodsWe conducted an analysis of death certificates from 1999-2017 to calculate annual CHD mortality by age at death, race/ethnicity, and sex. Population estimates used as denominators in mortality rate calculation for infants were based on National Center for Health Statistics live birth data. Mortality rates in individuals>1 year of age utilized US Census Bureau bridged-race estimates as denominators for population estimates. We characterized temporal trends in all-cause mortality, mortality resulting directly due to and related to CHD by age, race/ethnicity, and sex using joinpoint regression.ResultsThere were 47.7 million deaths with 1 in 814 deaths due to CHD (n=58,599). While 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 age >65years. The age-adjusted mortality rate decreased from 1.37 to 0.83 per 100,000. Males had higher mortality due to CHD than females throughout the study, although both sexes declined at a similar rate (∼40% overall), with a 3-4% 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 race/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).ConclusionsWhile overall US mortality due to CHD has decreased over the last 19 years, disparities in mortality persist for males compared to females and for non-Hispanic Blacks compared to 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.

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.

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.

New advances in machine learning could facilitate and reduce disparities in the prenatal diagnosis of congenital health disease, the most common and lethal birth defect.

Background: Adults with congenital heart disease (CHD) comprise an increasing proportion of individuals living with CHD in the United States (US). A greater than 20% decrease in adult congenital heart disease (ACHD) related mortality in the US was noted from 1999 to 2006. No recent national data are available evaluating mortality in the ACHD population. Our study aims to estimate US ACHD mortality rates by age and specific CHD lesion over the last two decades. Methods: We conducted a 19-year analysis (1999-2017) of publicly available, de-identified Multiple Cause of Death …

ObjectiveTo examine the risk for cyanotic congenital heart diseases (CCHDs) among live births in the USA, resulting from various forms of infertility treatments.MethodsThis 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).ResultsAmong 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.ConclusionsOur findings suggest an increased risk for CCHD in infants conceived after all types of infertility treatment.

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.

Aims Adolescents and young adults (AYA) with congenital heart disease (CHD) are a high-risk group particularly during the transition period from pediatric to adult care. We aimed to create a clinic-based tailored CHD transition program to improve adolescent knowledge and activation, increase participation in shared-decision making, and facilitate patient empowerment through Instructional Conversations (IC). Methods We conducted a needs assessment with key stakeholders (pediatric and adult cardiologists, parents, and adolescents with CHD) to identify potential barriers to transition. A three-pronged approach was taken to spearhead transition education: (1) Streamlining dedicated transition visits; (2) Creating individualized learning plans (ILP) to encourage shared decision making and patient engagement: and (3) Utilizing motivational interviewing (MI) and instructional conversation (IC) to administer a novel patient activation tool: CHHATT- The Congenital Heart Health Activation Transition Tool. Results Six Plan-Do-Study-Act (PDSA) cycles were conducted and included streamlining educational/assessment materials, modifying teaching style, implementing MI and IC, and creating the CHHATT tool. During PDSA cycles, three major themes emerged regarding patient activation and engagement. First, families better received transition services if given advanced notice. Thus, we built separate clinic visits for program introduction (14 year old) and serial education/training visits (15 year old). Second, having a streamlined script for education of various CHD diagnoses and creating ILPs resulted in increased patient engagement and shared decision-making. Third, patient self-assessments poorly revealed true knowledge deficits. MI was used to reveal these deficits through creation of a CHHATT tool, which reveals knowledge/skills gaps and reinforces education through guided IC. Patients demonstrated increased confidence when using the CHHATT to teach-back to parents and ask physician questions. Parents’ verbalized satisfaction in the increased empowerment teens demonstrated after serial visits using CHHATT. Conclusion We created a CHD transition program for AYA with streamlined serial education, individualized learning plans, and motivational interviewing and instructional conversation. Patients demonstrated increased activation and engagement using our novel tool CHHATT. We strive to lead transitional clinical care and further patient success by (1) introducing additional assessment tools for health literacy and resilience and (2) guiding clinical practice by sharing our sustainable/billable program model for CHD transition programs.

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 U.S. population. OBJECTIVE: To identify and characterize potential disparities in access to ACHD care in the U.S. 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 2011–2015 American Community Survey (U.S. Census) and the Environmental Systems Research Institute was analyzed based on drive time to nearest ACHD center. Previously established CHD prevalence estimates were used to estimate the similarly located U.S. ACHD population. RESULTS: Nearly half of the continental U.S. 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 >6 hour drive to a center (p6 hours away have higher proportions of uninsured adults (29% vs. 18%; p1 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.

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.

OBJECTIVE Acute maternal hyperoxygenation (AMH) results in increased fetal left heart blood flow. Our aim was to perform a pilot study to determine the safety, feasibility and direction and magnitude of effect of chronic maternal hyperoxygenation (CMH) on mitral and aortic valve annular dimensions in fetuses with left heart hypoplasia (LHH) after CMH. METHODS Gravidae with fetal LHH were eligible for inclusion in a prospective evaluation of CMH. LHH was defined as: sum of aortic and mitral valve annuli Z-scores

Background: Data regarding racial and ethnic differences in incidence and survival in pediatric cardiac arrest are unknown. In large cities, universal tracking of cardiac arrest is limited by multiple response centers fielding calls. This study was performed in Houston, one of the largest and most ethnically diverse cities in the US. Every 911 call in the city is tracked by a single operations center, providing a unique opportunity to conduct a population based study. Objective: We aimed to examine race and ethnic differences in incidence and survival rates among pediatric cardiac arrests utilizing a non-sampled population in a large metropolitan area. Methods: We performed a retrospective review of all 911 emergency response records involving non-traumatic pediatric cardiac arrests Results: There were 598 (57% males) pediatric cardiac arrests at median age of 10 mo (IQR 2 mo - 6 yrs). Infants Conclusions: There are racial differences in cardiac arrest in Houston based on age. Identifying reasons for these differences may provide insights into environmental or genetic risk factors associated with pediatric cardiac arrests.

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.

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