BACKGROUND:Knowledge of one's defect is fundamental in optimising outcomes in adults with CHD. We aimed to assess factors associated with defect knowledge and how that knowledge may impact outcomes. METHODS:We performed a cross-sectional cohort study of adults with CHDs aged 18-82 years with intervention between 1982 and 2003 at one of 11 US centres in the Pediatric Cardiac Care Consortium. Participants completed a survey in 2021-2023 regarding disease knowledge and various health and quality of life outcomes. After comparing participants' self-identification of their defect to that recorded in the medical record, we identified demographic factors associated with correct identification and assessed the association of correct identification with outcomes. RESULTS:Among 2747 respondents with one of the 18 types of CHDs on the survey, 2271 (83%) correctly identified their defect. Those who correctly identified their defect were more likely to be non-Hispanic White (84% correct), were <25 years of age (89%), have a higher level of education (86% for a bachelor's degree and 87% for a graduate degree), and have higher household income (85% for income ≥$60,000). Those with severe two-ventricle disease were most likely to correctly identify their defect (88% correct); those with moderate disease were least likely (80% correct). Correct identification was associated with seeing a cardiologist in the last 5 years (74% vs. 63%, p < 0.001) but was mixed for other outcomes. CONCLUSIONS:Improving a person's knowledge of their CHD may be an effective strategy for maintaining continuity of care.

Background: Understanding the long-term implications of CHD has become a priority as survival rates have improved. Little is understood about the economic implications of living with CHD into adulthood.Objectives: We aimed to determine the prevalence and risk factors of financial fragility (i.e., ability to pay $2,000 for an emergency expense) among adults with CHD in the United States and compare outcomes to their healthy siblings.Methods: We conducted a cross-sectional study using data from the CHD Project to Understand Lifelong Survivor Experience survey (2021-2023). The survey assessed demographics, medical history, and economic outcomes, including financial fragility. Analyses included chi-square and Wilcoxon rank-sum tests with multivariable logistic regression models adjusted for demographic and socioeconomic factors.Results: There were 3074 adults with CHD and 324 siblings who answered the financial fragility question. The prevalence of financial fragility was 20.6% for individuals with CHD versus 12.7% in siblings (p = 0.02); individuals with CHD had higher odds (OR 1.50, 95% CI 1.04-2.17, p = 0.029) of financial fragility compared to siblings. Single ventricle anatomy was the only CHD group significantly associated with financial fragility (aOR 1.59, 95% CI 1.12-2.26). Financially fragile individuals were more likely to report blindness, be female or a race other than non-Hispanic white, and have single ventricle CHD.Conclusion: Adults with CHD experience greater financial fragility than their siblings and disease severity is associated with increased risk. Addressing financial fragility is essential to mitigate the long-term economic burden of CHD on patients and families.

Background: The coronavirus disease 2019 (COVID-19) pandemic affected exposure to infectious agents. We aimed to analyze changes in Group A streptococcal (GAS), acute rheumatic fever (ARF) and rheumatic heart disease (RHD) prevalence during the COVID-19 pandemic and assess associated social vulnerability indices (SVI). Methods: Using the Epic Cosmos database, we performed a descriptive analysis of patients aged 2–21 in the United States from January 2016 through December 2023. We compared monthly GAS positivity rates and ARF/RHD diagnosis frequencies across pre-pandemic (January 2016–February 2020), early-pandemic (March 2020–August 2022), and late-pandemic (September 2022–December 2023) periods, incorporating Centers for Disease Control–derived SVI themes. Results: GAS positivity significantly decreased from pre-pandemic (15.5%; 838 tests/100,000) to early pandemic (12.3%; 363; P < 0.001), then rebounded in the late pandemic (20.8%; 1340; P < 0.001). Among 412 ARF and 780 RHD patients, early pandemic diagnoses dropped significantly for ARF (0.87–0.56 per 100,000; P < 0.001) and RHD (0.54–0.43; P = 0.003). Neither showed a significant late-pandemic resurgence (ARF 0.60, P = 0.915; RHD 0.40, P = 0.182). Compared with >29 million controls, RHD patients showed higher vulnerability across all SVI themes, while ARF patients were more vulnerable in Racial and Ethnic Minority Status, Housing Type and Transportation. Conclusions: This study identifies a significant reduction in GAS, ARF and RHD during the pandemic. Despite a late-pandemic surge in GAS infections, ARF and RHD diagnoses remained stable. These findings highlight a potential decline in overall ARF and RHD diagnoses and identify specific social vulnerability targets for public health intervention in the United States.

We examined the role of neighborhood-level socioeconomic position (nSEP) in 1- and 5-year survival among children with critical congenital heart defects (CCHDs). Children with CCHDs in the National Birth Defects Prevention Study (1999-2011) were grouped into univentricular and biventricular defects and linked to vital records for 1- and 5-year mortality. The Neighborhood Deprivation Index (NDI) was used to classify census-tract nSEP (low [referent], moderate, high deprivation) using maternal periconceptional address. Kaplan-Meier survival curves and log-rank tests were used to evaluate survival differences. Cox proportional hazards regression models were used to estimate crude and adjusted hazard ratios and 95% confidence intervals, adjusting for birth years, maternal sociodemographic factors, and residential mobility. Among 2459 children with CCHDs, 1 year survival curves differed by neighborhood deprivation. Survival was lowest among children of mothers living in high- vs low-deprivation neighborhoods. In crude analyses, high deprivation was associated with higher 1-year mortality (all CCHDs: 1.58 [1.17, 2.13]; univentricular CCHDs: 1.50 [0.99, 2.26]; biventricular CCHDs: 1.64 [1.02, 2.64]). After adjustment, estimates were generally attenuated and less precise. Five-year survival showed similar trends. Further research could inform strategies to address the structural, environmental, and/or physiological factors associated with a potential reduced survival among children with CCHDs born to mothers in socioeconomically deprived areas.

Given the heart-brain relationship, children with congenital heart conditions (CHC) may experience poor academic outcomes, even if not diagnosed with developmental conditions. Among children without diagnosed developmental conditions, this study evaluates the “healthy and ready to learn” (HRL) metric by CHC status and identifies associated factors. Using 2022–2023 National Survey of Children’s Health (NSCH) data, we compared HRL among 3-5-year-olds with no known genetic, intellectual, or developmental disabilities by CHC status using adjusted prevalence ratios (aPRs) and 95

Background Many US children have congenital heart defects (CHD), yet data on their long-term outcomes have limitations. The Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG of KIDS (CHSTRONG KIDS) addresses these gaps by surveying caregivers of children with CHD identified through birth defect surveillance systems [BDSS]. Objectives To describe the CHSTRONG KIDS project design, characteristics of the eligible population, and the percentage not up-to-date on recommended cardiology care. Methods Children born 2006–2021 with CHD were identified using active, population-based BDSS in Atlanta, Georgia, Massachusetts, and Minnesota and linked to vital records. Caregivers of eligible (living) children with CHD were invited to complete surveys in 2024–2025. Characteristics were compared by site and response status using χ2 tests. We also estimated percentages of children in CHSTRONG KIDS who had not seen a cardiologist within the guideline-recommended timeframe for their specific defects. Results Among 7239 identified children with CHD, 6240 were eligible for survey recruitment. Of those,1841 (30%) had caregiver-reported survey data. Several characteristics, including CHD severity, birth year, and Trisomy 21 diagnosis, varied by site (p < 0.05). Survey response rates differed by site, CHD severity, maternal race, maternal education, and rurality (p < 0.05), prompting development of post-stratification weights. A weighted 21.6% were not up-to-date on their cardiology care. Conclusion With data on >7200 CHD cases and >1800 caregiver-reported surveys, CHSTRONG KIDS provides a population-based view of long-term outcomes among children with CHD. Notably, one in five were not up-to-date on their cardiology care and therefore may not be represented in clinical cohorts.

BACKGROUND:Improved survival of individuals with congenital heart disease (CHD) into adulthood has made reproductive health a crucial aspect of CHD care. Despite existing guidelines on heritability, contraception, pregnancy, and postpartum care, little is known about CHD survivors' experiences and attitudes toward reproductive health. This study aimed to examine reproductive health outcomes and experiences among adults with CHD, with a focus on female survivors. METHODS:Data were drawn from the CHD PULSE (Project to Understand Lifelong Survivor Experience) study, a cross-sectional survey conducted from September 2021 to April 2023. Participants were adults (≥18 years) with at least 1 CHD intervention at 1 of 11 U.S. Pediatric Cardiac Care Consortium centers. CHD severity was categorized based on initial diagnosis and intervention. Categorical and continuous variables were analyzed using Chi-square and Kruskal-Wallis tests, respectively. RESULTS:Among 3,073 respondents (1,704 females, 1,369 males), 48% of female participants reported receiving no reproductive counseling. Factors significantly associated with receiving counseling included older age, later age at last surgery, marital history, severe CHD, higher income, government insurance, and more comorbidities (P < .001). Women were significantly more likely to have biological children than men (40.4% vs 37.5%, P < .0001). CONCLUSION:Reproductive counseling is infrequent among women with CHD, especially among younger, unmarried, low-income individuals with less severe CHD and fewer comorbidities. Significant gender disparities in reproductive outcomes emphasize the need for tailored, gender-specific reproductive health counseling for CHD survivors.

BACKGROUND:Long-term outcomes after Fontan vary widely. Although pre-Fontan hemodynamics predict early failure, their association with long-term outcomes remains unclear. We hypothesized that pre-Fontan hemodynamics predict long-term risk of death or transplantation. METHODS:We analyzed data from the Pediatric Cardiac Care Consortium, a US-based multicenter registry including patients undergoing first-time Fontan with pre-Fontan catheterization and long-term follow-up. Patients undergoing a Fontan procedure before 18 years of age at any time between 1982 and 2011 were included in the study. Outcomes of interest were in-hospital Fontan failure (death or takedown) and postdischarge death or transplantation, identified through matching with the National Death Index and the Organ Procurement and Transplantation Network through 2022. Associations between pre-Fontan hemodynamics and long-term risk for death or transplantation were assessed with Kaplan-Meier survival curves and extended Cox regression. RESULTS:Among 1175 patients (736 [62.6%] male, 626 [53.3%] with systemic left ventricle), 1111 were discharged with Fontan physiology. Over a median postdischarge follow-up of 20.6 years (interquartile range, 18.2-24.4 years), 85 deaths and 49 transplantations occurred. Pre-Fontan mean pulmonary arterial pressure was the strongest hemodynamic predictor of postdischarge outcomes with a continuous association and no clear inflection point; 25-year transplantation-free survival declined from 83.7% (95% CI, 77.6-88.3) in the low mean pulmonary arterial pressure tertile to 73.7% (95% CI, 65.5-80.3) in the highest tertile (log-rank P=0.02). Each 1-SD increase in mean pulmonary arterial pressure was associated with 1.33-fold higher odds of in-hospital failure (adjusted odds ratio, 1.33 [95% CI, 1.00-1.77]; P=0.05) and a 2.2-fold higher hazard of death or transplantation (adjusted hazard ratio, 2.20 [95% CI, 1.62-3.00]; P<0.01) estimated at discharge. This hazard declined 3% per year after discharge (adjusted hazard ratio per year, 0.97 [95% CI, 0.95-0.99]; P<0.01) and resolved by 17 years in patients with systemic right ventricle and by 23 years in those with systemic left ventricle. Additional independent risk factors included systemic right ventricle versus systemic left ventricle (adjusted hazard ratio, 2.39 [95% CI, 1.65-3.46]; P<0.01) and delayed Fontan completion (>4 years of age versus 2 to 4 years of age; adjusted hazard ratio, 1.80 [95% CI, 1.25-2.60]; P=0.02). CONCLUSIONS:Elevated pre-Fontan mean pulmonary arterial pressure is a strong predictor of in-hospital and long-term post-Fontan risk of death or transplantation. Systemic right ventricle and delayed Fontan completion (>4 years of age) further increased risk. These findings support early Fontan consideration and ongoing hemodynamic surveillance to optimize long-term outcomes.

Background:Digoxin use after the Norwood procedure has been associated with improved interstage survival in hypoplastic left heart syndrome and related conditions. Whether this benefit translates into improved longer-term outcomes through staged palliation remains unknown. We aimed to determine the association of digoxin use at Norwood discharge with transplant-free survival and Fontan completion. Methods:We conducted a retrospective cohort study using the Pediatric Heart Network (PHN) Single Ventricle Reconstruction trial public dataset, including 549 infants enrolled at 15 North American centers between 2005 and 2008. Competing risk analysis was used to evaluate Fontan completion and Cox regression to assess death or transplantation within 6 years after the Norwood procedure. Mixed-effects models compared pre-Fontan hemodynamic and echocardiographic right ventricular indices between patients treated with and without digoxin after accounting for center clustering and adjustment for sex, shunt type, heart failure medications at Norwood discharge, and census block poverty level. Results:The 6-year cumulative incidence of Fontan completion was higher among patients discharged on digoxin than among those not receiving digoxin (82% vs 71%; p = 0.013). Competing-risk analysis accounting for death and transplant demonstrated a greater likelihood of Fontan completion among digoxin users (aHR 1.31; 95%CI 1.09-1.58; p = 0.005), without significant difference in the hazard of death or transplant (aHR 0.78; 95%CI 0.53-1.15; p = 0.208). No significant differences in pre-Fontan hemodynamic or echocardiographic indices were observed between groups. Initiation of digoxin post Stage II procedure was not associated with improved survival or likelihood to complete Fontan. Conclusion:Digoxin use at the time of Norwood discharge was associated with a 30% greater likelihood of Fontan completion by 6 years, without accompanying improvement in transplant-free survival. These findings extend prior observations of improved interstage outcomes associated with digoxin use and suggest that treatment may facilitate progression through staged palliation.

OBJECTIVE:To examine the accuracy of pulse oximetry in neonates by race and ethnicity. STUDY DESIGN:We performed a single-centre retrospective cross-sectional study in neonates aged ≤ 7 days at Children's Healthcare of Atlanta in the neonatal and cardiac ICU between 2010 and 2022. Eligible patients had pulse oximetry with arterial oxygen saturation measurements taken within 10 minutes. We evaluated the mean saturation bias, accuracy root mean square, and the frequency of occult hypoxaemia by race and ethnicity. RESULTS:Of 1645 infants, there were 639 non-Hispanic Black infants (38.8%), 651 non-Hispanic White infants (39.6%), and 215 Hispanic infants (13.1%). The majority had no CHD (71.4%), required respiratory support (81.1%), and had oxygen saturation levels = > 90% (87.6%). The mean pulse oximetry and arterial oxygen measurement time difference was 0.8 minutes. The mean bias and accuracy root mean square were 8.7% and 13.7%, respectively, with no significant difference between the groups (p = 0.91). Occult hypoxaemia was found in 20.5% of infants. Compared to White infants, there were no differences in likelihood of occult hypoxaemia for either Black (adjusted prevalence ratio 1.13 (0.92-1.4), p = 0.24) or Hispanic (1.06 (0.77-1.45, p = 0.72) infants. CONCLUSION:There was not a systemic discrepancy in pulse oximetry between racial and ethnic groups as previously described in older children and adults. However, pulse oximetry significantly overestimated arterial oxygen saturation. Future prospective studies that objectively measure skin pigmentation may be able to overcome some of the limitations of our study.

BACKGROUND:Total anomalous pulmonary venous return (TAPVR) is a rare heart defect in which the pulmonary veins do not connect to the left atrium, instead draining into the right atrium or directly into other venous systems. We explored factors associated with TAPVR using the National Birth Defects Prevention Study (NBDPS; 1997-2011) and Birth Defects Study To Evaluate Pregnancy exposureS (BD-STEPS; 2014-2021) data. METHODS:We identified potential risk or protective factors from the literature and used logistic regression to calculate crude and adjusted odds ratios (OR) and 95% confidence intervals (CI) for the association between 22 factors encompassing maternal demographics, health history, and medication use and TAPVR. In an exploratory analysis among the subset of NBDPS cases and controls, we examined 163 potential factors using random forest. RESULTS:In the primary analysis including 393 cases with TAPVR and 13,914 non-malformed controls, we observed positive associations between TAPVR and maternal age at delivery < 20 (OR 2.00; 95% CI 1.30-3.06) and 20-24 (1.62; 1.19-2.22) vs. 25-29 years, having one (1.40; 1.07-1.82) and two or more previous live births (1.69; 1.25-2.27) vs. none, and multiple gestation (1.85; 1.11-2.92). Non-Hispanic Black mothers had the lowest risk (0.55; 0.35-0.85) while mothers of other races/ethnicities had the highest risk (1.72; 1.19-2.44) compared to non-Hispanic White mothers. The random forest identified an inverse effect of maternal fish consumption. CONCLUSIONS:Our findings confirmed previously observed associations of younger maternal age and having previous live births with TAPVR. Maternal fish consumption may confer a protective effect and deserves further investigation.

BACKGROUND:Mild congenital heart diseases such as atrial septal defect, ventricular septal defect, patent ductus arteriosus, and pulmonary valve stenosis constitute a significant public health problem. Understanding long-term outcomes after interventions for mild congenital heart disease is essential to inform lifelong care. METHODS:We queried the Pediatric Cardiac Care Consortium, a multicenter US-based registry of patients with congenital heart disease interventions, for patients undergoing surgical or transcatheter procedures for atrial septal defect, ventricular septal defect, patent ductus arteriosus, and pulmonary valve stenosis; 17 407 patients were included. Statistical methods included survival analysis using Kaplan-Meier plots, multivariable Cox proportional hazards models for risk factors for death and standardized mortality ratios. RESULTS:There were 115 in-hospital deaths (0.7%), with the remaining patients included in the postdischarge. The 35-year post-discharge survival ranged from 95.5% for pulmonary valve stenosis to 92.1% for ventricular septal defect. Survival patterns differed by lesion and age at intervention, with intervention at 1≤5 years generally associated with more favorable outcomes. Lower weight-for-age at intervention was associated with an increased hazard of death across all shunt lesions but not for pulmonary valve stenosis. Compared with the general population, mortality risk remained elevated for up to 18 to 20 years after intervention. CONCLUSIONS:Long-term survival after intervention for mild congenital heart disease is excellent; yet lesion-specific risks persist for decades. Age and weight-for-age at intervention are associated with survival, together with excess mortality beyond childhood underscore the need for lifelong, lesion-tailored surveillance in this growing population.

BACKGROUND:Among individuals with single ventricle (SV) congenital heart disease (CHD), systemic right ventricle morphology is associated with decreased survival compared to systemic left ventricle morphology. We aimed to determine the association of ventricular morphology with quality of life and medical, neurocognitive, socioeconomic outcomes and compare these between SV CHD patients and siblings. METHODS:We performed a cross-sectional cohort study using data from congenital heart disease project to understand lifelong survivor experience, a survey conducted from 2021 to 2023 among adults with CHD interventions and siblings across 11 US Pediatric Cardiac Care Consortium centers. Quality of life was assessed by Patient-Reported Outcomes Measurement Information System (PROMIS) surveys. RESULTS:We included 226 SV patients (146 left and 80 right ventricular) and 40 siblings. No outcome differences were found between left and right ventricular groups. Compared to siblings, SV patients reported more medical comorbidities (P < .001), future health concerns (0.008), disability benefits (P < .001), and work affected by health (P < .001). Siblings had higher rates of college/graduate degrees (P = .002) and employment (P = .044). However, 95.6% of SV patients completed high school, 45.6% had college/graduate degrees, and 73.9% were employed. SV patients had lower PROMIS scores for global mental health and physical function but similar scores for global physical health, cognitive function, anxiety, depression, and ability to participate in social roles. CONCLUSIONS:Ventricular morphology was not associated with differences in long-term outcomes. Adults with SV CHD have medical, neurocognitive, and socioeconomic differences compared to siblings, but similar PROMIS scores compared to the general population.

BACKGROUND:The coronavirus disease 2019 (COVID-19) pandemic affected exposure to infectious agents. We aimed to analyze changes in Group A streptococcal (GAS), acute rheumatic fever (ARF) and rheumatic heart disease (RHD) prevalence during the COVID-19 pandemic and assess associated social vulnerability indices (SVI). METHODS:Using the Epic Cosmos database, we performed a descriptive analysis of patients aged 2-21 in the United States from January 2016 through December 2023. We compared monthly GAS positivity rates and ARF/RHD diagnosis frequencies across pre-pandemic (January 2016-February 2020), early-pandemic (March 2020-August 2022), and late-pandemic (September 2022-December 2023) periods, incorporating Centers for Disease Control-derived SVI themes. RESULTS:GAS positivity significantly decreased from pre-pandemic (15.5%; 838 tests/100,000) to early pandemic (12.3%; 363; P < 0.001), then rebounded in the late pandemic (20.8%; 1340; P < 0.001). Among 412 ARF and 780 RHD patients, early pandemic diagnoses dropped significantly for ARF (0.87-0.56 per 100,000; P < 0.001) and RHD (0.54-0.43; P = 0.003). Neither showed a significant late-pandemic resurgence (ARF 0.60, P = 0.915; RHD 0.40, P = 0.182). Compared with >29 million controls, RHD patients showed higher vulnerability across all SVI themes, while ARF patients were more vulnerable in Racial and Ethnic Minority Status, Housing Type and Transportation. CONCLUSIONS:This study identifies a significant reduction in GAS, ARF and RHD during the pandemic. Despite a late-pandemic surge in GAS infections, ARF and RHD diagnoses remained stable. These findings highlight a potential decline in overall ARF and RHD diagnoses and identify specific social vulnerability targets for public health intervention in the United States.

As survival improves for adults with tetralogy of Fallot (TOF), long-term outcomes have taken on greater importance. We aimed to compare the long-term health, socioeconomic, and quality of life (QOL) outcomes between adult survivors and sibling controls. CHD PULSE is a cross-sectional survey from 2021 to 2023 across 11 Pediatric Cardiac Care Consortium centers; 3,133 adults with a history of CHD and 326 unaffected siblings completed surveys on health, socioeconomic, and QOL outcomes with the Patient-Reported Outcomes Measurement Information System (PROMIS). There were 337 TOF survivors, who were similar in age to controls (33.4 yrs vs 32.0 yrs, p = 0.076) but less likely to be female (56% vs 65%, p = 0.022). TOF survivors more frequently reported difficulty paying for medical care (31% vs 15%, p < 0.001), overnight hospitalizations (13% vs 7%, p = 0.015), and concern for future health (53% vs 38%, p < 0.001). They were less likely to have an associate's degree or higher (53% vs 74%, p < 0.001) and more likely to have health-related work limitations (43% vs 8%, p < 0.001) or be unemployed within the preceding year (20% vs 7% p < 0.001). However, PROMIS scores were favorable for both TOF survivors and the sibling comparison group. In conclusion, adults with TOF face persistent medical, education, and socioeconomic challenges relative to unaffected siblings. However, PROMIS quality of life scores demonstrate resiliency in the TOF survivors, with similar self-perceived QOL to their siblings.

IntroductionAccessing specialty cardiac care presents challenges for U.S. individuals with congenital heart defects (CHD), despite recommendations for regular cardiac follow-up. Additionally, the impact of the COVID-19 pandemic and telehealth expansion on CHD-related health care is not well-understood. Therefore, our objective was to evaluate changes in CHD-related telehealth and other outpatient utilization in the Medicaid population from 2018 to 2022 and a privately-insured population from 2018 to 2023.MethodsUsing claims from national Medicaid (2018-2022) and private insurance (2018-2023) databases, we calculated the number of CHD-related outpatient encounters per 100,000 enrolled children or adults and the number conducted via telehealth. We compared CHD-related outpatient utilization trends before and after the COVID-19 interruption (identified as March-May 2020) as an interrupted times series with Poisson regression.ResultsAmong adults and children in both insured populations, CHD-related outpatient utilization increased before the COVID-19 interruption (January 2018-February 2020); afterward, it decreased in Medicaid but continued increasing at a slower rate among the privately-insured (all P < 0.01). For children, CHD-related outpatient utilization in June 2020 was lower (8%-Medicaid, 5%-private) than would be expected without the interruption (both P < 0.01). Though <1% pre-pandemic, recent (2022/2023) telehealth utilization accounted for 1%-4% of pediatric and adult outpatient CHD-related encounters in both databases.DiscussionCHD-related outpatient care has declined among Medicaid beneficiaries and slowed among private insurance beneficiaries since the COVID-19 interruption, and efforts may be needed to re-engage CHD patients in regular outpatient cardiac care. Telehealth may be an underutilized option to improve access to CHD care.

BACKGROUND:We assessed healthcare access and utilization among adults with congenital heart defects (ACHD) across racial and ethnic groups using a US population-based sample. METHODS:The cross-sectional CH STRONG was conducted from 2016 to 2019 among ACHD in Arkansas, Arizona, and Atlanta. Participants completed a self-administered questionnaire on their healthcare access, utilization, health status, comorbidities, and demographics. Differences in healthcare access and utilization among non-Hispanic White (NH-White), non-Hispanic Black (NH-Black), and Hispanic ACHD were assessed, and adjusted prevalence ratios (aPRs) and 95% confidence intervals (CI) reported. Health-related quality of life (HRQOL) was also compared with standardized norms for the general population from the Patient-Reported Outcomes Measurement Information System (PROMIS). Models were adjusted for age, sex, educational attainment, marital status, study site, and CHD severity. RESULTS:Of 1455 respondents, 76.7% were NH-White, 15.0% were NH-Black, and 8.9% were Hispanic. Compared to NH-White, NH-Black ACHD were less likely to have health insurance (aPR = 0.94; CI: 0.88-0.99) and more likely to report a prior gap in insurance coverage (aPR = 1.88; CI: 1.44-2.46). They were also less likely to have a usual place of care (aPR = 0.82; CI: 0.75-0.89) or any healthcare visits (aPR = 0.88; CI: 0.81-0.96) but more likely to have ≥ 1 emergency room visit (aPR = 1.66; CI: 1.38-2.00) and hospitalization (aPR = 1.96; CI: 1.40-2.74) in the past year. Hispanic ACHD were more likely than NH-White to report a prior gap in insurance coverage (aPR = 1.78; CI: 1.19-2.65). CH STRONG ACHD did not differ from the general population on global mental-health and physical-health scores. CONCLUSIONS:Racial and ethnic differences in healthcare access and utilization exist among a population-based group of ACHD. Strategies for improvement in access to quality care may help reduce differences.

The initial and updated Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STAT and STAT 2020) and Risk Adjusted Classification for Congenital Heart Surgery-1 and Risk Adjusted Classification for Congenital Heart Surgery-2 scoring systems are validated to predict early postoperative mortality following congenital heart surgery in children; however, their ability to predict long-term mortality has not been examined. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium, a US-based registry of cardiac interventions in 47 participating centres between 1982 and 2011. Patients included in this cohort analysis had select congenital heart surgery representing the spectrum of severity as determined by STAT and Risk Adjusted Classification for Congenital Heart Surgery-1 and were less than 21 years of age. We applied STAT, STAT 2020, Risk Adjusted Classification for Congenital Heart Surgery-1, and Risk Adjusted Classification for Congenital Heart Surgery-2 for prediction of early mortality and long-term postoperative survival probability by surgical risk category. Long-term outcomes were obtained by matching Pediatric Cardiac Care Consortium patients with deaths reported in the National Death Index through 2021. Of 20,753 eligible patients, 18,755 survived the postoperative period and 2,058 deaths occurred over a median follow up of 24.4 years (Interquartile Range: 21-28.4). Each scoring system performed well for predicting early postoperative mortality with the following c-statistics: STAT: 0.7872, Risk Adjusted Classification for Congenital Heart Surgery-1: 0.7872, STAT 2020: 0.7724 and Risk Adjusted Classification for Congenital Heart Surgery-2: 0.7668. The predictive ability for long-term risk of death was as follows: STAT: 0.6995, Risk Adjusted Classification for Congenital Heart Surgery-1 c = 0.6741, Risk Adjusted Classification for Congenital Heart Surgery-2: 0.7156 and STAT 2020: c = 0.7156. Risk-adjusted score systems for congenital heart surgery maintain adequate but diminishing discriminative power to predict long-term mortality. Future efforts are warranted to develop a tool with improved long-term survival prediction.

OBJECTIVE:To evaluate prevalence of and characteristics associated with prenatal and late critical congenital heart defect (CCHD) detection among infants. STUDY DESIGN:Infants with CCHD born during 2014-2021 with interviewed mothers were included from the Birth Defects Study To Evaluate Pregnancy exposureS, a multisite, population-based case-control study. Timing of detection was based on date of earliest fetal or postnatal echocardiogram: prenatal, timely postnatal (0-3 days after birth), and late (>3 days after birth). Unadjusted log-linear models evaluated trends in timing of CCHD detection by birth year. Multivariable log-binomial models calculated adjusted prevalence ratios for prenatal and late CCHD detection by demographic and clinical characteristics. RESULTS:There were 996 liveborn infants with CCHD included in this analysis. Prenatal detection increased from 2014 (25.0%) to 2021 (39.1%; P for trend = .01). The prevalence of late detection was 22.7% in 2014 and 16.4% in 2021; P = .06. Almost half (48.6%) of infants had timely postnatal detection. Prenatal detection was 1.2 times (95% CI 1.1-1.5) more likely among infants with extracardiac compared with isolated defects. Late CCHD detection was 2.0 times (95% CI 1.2-3.4) more common among infants whose mothers lacked prenatal insurance compared with those with prenatal insurance. CONCLUSIONS:Disparities in timing of CCHD detection exist by defect characteristics and insurance. Implementation of improved prenatal detection methods to detect more defect types and interventions to increase access to prenatal care may further improve earlier CCHD detection.

Critical congenital heart defects are a leading cause of mortality in childhood. Early identification can help reduce morbidity and mortality. This review provides an overview of the evaluation of newborn infants to identify critical congenital heart defect in a timely manner. It also details ancillary tests and newer technologies that can be utilized for this assessment.