Moderate Congenital Heart Disease Research Articles

Abstract 19033: Increased Prevalence of Gaps in Care for Patients With Congenital Heart Disease Are Associated With Social Determinants of Health Including Childhood Opportunity Index

Background: Lifelong continuity of care is imperative for patients with congenital heart disease (CHD). Unfortunately, gaps in care (GIC) are common. Hypothesis: Social determinants of health are associated with GIC in patients with CHD. Methods and Results: A total of 2511 patients followed at Nemours Cardiac Center (NCC) with CHD were seen in the clinic between January 2005 and December 2022. Patients were divided into 3 cohorts based on anatomic complexity described in 2018 AHA/ACC adult congenital guidelines as simple, moderate, and complex CHD. Patient characteristics were analyzed, including race, ethnicity, gender, language, insurance status, and Childhood Opportunity Index (COI) based on home address zip code. GIC of > 3 years were identified in 56% (n=1168) of patients. Linear regression model showed that the proportion of GIC stayed consistent for complex CHD with an increase of 0.1% per year and had a significant increase for those with simple and moderate CHD (Figure 1) with a yearly increase of 1.1% and 0.6% respectively. On the multivariable model GIC were associated with a non-Delaware residence (P<0.001), simple to moderate CHD (P<0.001), having less than two caregivers listed (P<0.001), not having public insurance (P<0.001), being none-white (P<0.007), age (P<0.001) Overall COI (P<0.007) with a c-Statistic of 0.692. Conclusions: Over half of the patients with CHD experienced a substantial GIC. GIC were consistent for those with complex CHD but increased significantly for those with simple to moderate CHD over almost two decades and were associated with various SDOH factors including COI. Providing consistency in practice, minimizing practice variation especially for those with mild and moderate CHD and understanding the role of SDOH factors can help decrease GIC and improve patient care for this vulnerable population.

Activities of Daily Living, Physical Activity, Physical Fitness and Quality of Life in Children with Congenital Heart Disease: A Case-Control Study.

Despite reports of reduced physical fitness in children with congenital heart disease (CHD), no specific performance evaluations for activities of daily living have been conducted. The aim was to compare the activities of daily living, quality of life, posture, physical fitness and physical activity levels of children with CHD with healthy controls (HC). The study included 30 children aged 6-14 diagnosed with moderate or severe CHD and 30 age-sex-matched HC. The sociodemographic and clinical data of the participants were recorded. All participants went through several tests, namely the TGlittre-P test for activities of daily living, the 6-minute walk test (6MWT) for functional capacity, the Fitnessgram test battery for physical fitness, the hand dynamometer for measuring grip strength, the pedometer for measuring physical activity, and both the child and parents reported the Pediatric Quality of Life Inventory (PedsQL) for evaluating the quality of life, in addition to posture analyses. Values of p < 0.05 were considered statistically significant. Individuals with CHD had a longer TGlittre-P test completion time and a shorter 6MWT distance than HC (TGlittre-P: CHD 3.45 [3.24-4.02]min vs. HC 3.10 [2.57-3.23]min, 6MWT: CHD 514.00 [412.50-566.00]m vs. HC 591.50 [533.00-631.00]m). For the CHD group, sit-ups, push-ups, trunk lift, and sit-and-reach test scores within the Fitnessgram battery, grip strength, posture, and quality of life scores were lower than those for the HC group. Physical activity levels were similar in the groups. The performance of activities of daily living, functional capacity, physical fitness, posture, and quality of life of children with moderate and severe CHD were affected compared to healthy peers.

Mental Health Conditions Among Children and Adolescents With Congenital Heart Disease: A Danish Population-Based Cohort Study.

Despite the known mental health burden among children with congenital heart disease (CHD), the literature is constrained by a lack of comparison cohorts and population-based follow-up data. We examined the incidence of mental health conditions among children with CHD, relative to 3 comparison cohorts. This population-based cohort study identified all children with CHD (<18 years of age; n=16 473) in Denmark from 1996 to 2017, through linkage of individual-level data across national registries. This allowed for complete follow-up of the population. Comparison cohorts included children from the general population (n=162 204), siblings of children with CHD (n=20 079), and children with non-CHD major congenital anomalies (n=47 799). Mental health conditions were identified using inpatient and outpatient hospital discharge codes, prescription data, and data on use of community-based psychology, psychiatry, and psychotherapy services. We computed cumulative incidence by 18 years of age, incidence rates, and adjusted hazard ratios (aHRs) using Cox regression. aHRs accounted for sex, year of CHD diagnosis, parental mental health, and socioeconomic status. All estimates were stratified by age, sex, and CHD complexity. The cumulative incidence of mental health conditions by 18 years of age in the CHD cohort was 35.1% (95% CI, 34.0%-36.1%), corresponding to aHRs of 1.64 (95% CI, 1.58-1.71), 1.41 (95% CI, 1.30-1.52), and 1.02 (95% CI, 0.98-1.07) compared with the general population, sibling, and major congenital anomaly cohorts, respectively. Mental health incidence rates showed prominent peaks in early childhood and adolescence. Males and children with severe or single-ventricle CHD demonstrated higher incidence rates of mental health conditions relative to females and children with mild or moderate CHD, respectively. Compared with the general population and sibling cohorts, incidence rates and aHRs in the CHD cohort were highest for severe stress reactions, attention deficit/hyperactivity disorder, intellectual disability, and autism spectrum disorder. Compared with children in the major congenital anomaly cohort, the aHRs were close to 1. More than one-third of children with CHD were diagnosed or treated for a mental health condition by 18 years of age. Mental health conditions began early in life and were most prominent among males and children with severe or single-ventricle heart disease.

Cardiometabolic Risk Factors in Mexican Adults With Congenital Heart Disease

BackgroundIn recent decades, adults living with congenital heart disease (ACHD) have improved their survival, thus increasing their predisposition to the onset of cardiometabolic risk factors and chronic health conditions. ObjectivesThe purpose of this study was to describe cardiometabolic risk profiles in the ACHD population and their relationship to congenital heart disease (CHD) lesion complexity. MethodsWe performed a cross-sectional study from ACHD in a third-tier referral center in Mexico City. The association between cardiometabolic risk factors and CHD complexity was estimated using logistic regression models. ResultsOur study cohort included 1,171 ACHD patients (median age: 31 [IQR: 23.2-42.7] years, male 63.6%). Cardiac diagnosis was classified as mild (44.9%), moderate (37.8%), and severe (17.2%) CHD complexity. Low high-density lipoprotein cholesterol (55%) was the most common cardiometabolic risk factor; followed by insulin resistance (54.5%) and prediabetes (52.4%). Patients with mild and moderate CHD had a higher prevalence of obesity and metabolic syndrome, while patients with severe CHD had a higher prevalence of hyperuricemia and subclinical hypothyroidism. In the logistic regression analysis, the severity of CHD was associated with higher odds of hyperuricemia (moderate CHD, OR: 1.87; 95% CI: 1.20-2.93; P = 0.010; severe CHD, OR: 2.75; 95% CI: 1.64-4.62; P < 0.001) and lower risks of metabolic syndrome (OR: 0.61; 95% CI: 0.41-0.91; P = 0.010), prediabetes (OR: 0.58; 95% CI: 0.42-0.81; P < 0.001), and arterial hypertension (OR: 0.49; 95% CI: 0.33-0.74; P < 0.001) compared with mild CHD complexity. ConclusionsWe observed high rates of cardiometabolic risk factors in Mexican ACHD patients and these risk profiles varied by CHD lesion complexity. These results highlight the need for ongoing metabolic health surveillance in the ACHD population.

Mobile Cardiac Telemetry Use to Predict Adverse Pregnancy Outcomes in Patients With Congenital Heart Disease

BackgroundPatients with congenital heart disease (CHD) have a higher incidence of arrhythmias during pregnancy, yet the utility of mobile cardiac telemetry (MCT) to predict adverse outcomes is unknown. ObjectivesThe purpose of this study is to determine whether arrhythmias on screening MCT correlate with adverse pregnancy outcomes. MethodsPatients with CHD prospectively enrolled in the Standardized Outcomes in Reproductive Cardiovascular Care initiative underwent 24-hour MCT (within 18 months prior to pregnancy). Positive findings on MCT were defined as episodes of bradyarrhythmia, symptomatic atrioventricular block, ectopic atrial or ventricular activity, and supraventricular or ventricular tachycardia. Clinically significant arrhythmia events (CSAEs) were those requiring medical or device intervention or an emergency room visit. Clinical events during the antepartum, intrapartum, and postpartum periods were compared using Fisher’s exact test. Analyses were performed using Stata version 16. ResultsIn 141 pregnancies in 118 patients with CHD, MCT detected positive findings in 17%. Adverse cardiac outcomes occurred in 11% of pregnancies, of which CSAE occurred in 3.5%. Positive MCT was significantly associated with subsequent CSAE (21% vs 0%, P < 0.001) and cumulative adverse maternal cardiac outcomes (33% vs 7%, P = 0.001) but did not correlate with obstetric (46% vs 41%, P = 0.660) or neonatal outcomes (33% vs 31%, P = 0.810). Of the patients with CSAE, 75% had ≥moderate CHD complexity. ConclusionsPatients with CHD had a high rate of positive MCT findings. This was associated with CSAE and adverse maternal cardiac outcomes. Patients with ≥moderate CHD complexity may benefit from screening MCT to improve preconceptual counseling and planning.

Outcomes of out-of-hospital cardiac arrest in adult congenital heart disease: a Danish nationwide study.

The risk, characteristics, and outcome of out-of-hospital cardiac arrest (OHCA) in patients with congenital heart disease (CHD) remain scarcely investigated. An epidemiological registry-based study was conducted. Using time-dependent Cox regression models fitted with a nested case-control design, hazard ratios (HRs) with 95% confidence intervals of OHCA of presumed cardiac cause (2001-19) associated with simple, moderate, and severe CHD were calculated. Moreover, using multiple logistic regression, we investigated the association between pre-hospital OHCA characteristics and 30-day survival and compared 30-day survival in OHCA patients with and without CHD. Overall, 43 967 cases (105 with simple, 144 with moderate, and 53 with severe CHD) and 219 772 controls (median age 72 years, 68.2% male) were identified. Any type of CHD was found to be associated with higher rates of OHCA compared with the background population [simple CHD: HR 1.37 (1.08-1.70); moderate CHD: HR 1.64 (1.36-1.99); and severe CHD: HR 4.36 (3.01-6.30)]. Pre-hospital cardiopulmonary resuscitation and defibrillation were both associated with improved 30-day survival in patients with CHD, regardless of CHD severity. Among patients with OHCA, simple, moderate, and severe CHD had a similar likelihood of 30-day survival compared with no CHD [odds ratio 0.95 (0.53-1.69), 0.70 (0.43-1.14), and 0.68 (0.33-1.57), respectively]. A higher risk of OHCA was found throughout the spectrum of CHD. Patients with and without CHD showed the same 30-day survival, which relies on the pre-hospital chain of survival, namely cardiopulmonary resuscitation and defibrillation.

Digital health nudging to increase physical activity and quality of life in pediatric patients with congenital heart disease: a randomized controlled trial

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): This research was funded by an unrestricted grant from the “Stiftung KinderHerz Deutschland gGmbH.” Background Digital health nudging has been shown to be a compelling, age-appropriate, and modern e-health intervention to increase physical activity (PA) in healthy adolescents. However, such studies are lacking in adolescents with congenital heart disease (CHD), although activity promotion is particularly important in this vulnerable population. Purpose This randomized-controlled trail examines if digital health nudging via daily smartphone messages increases PA and health-related quality of life (HrQoL) in adolescents with CHD. Methods From May 2021 to April 2022, 97 patients (15.1 ± 2.0 years, 50% girls) with moderate or severe CHD were randomly allocated 1:1 to an intervention group (IG) or control group (CG). Daily PA was objectively assessed in minutes of moderate-to-vigorous PA (MVPA) by the wrist worn wearable "Garmin Vivofit jr." over the entire study period. HrQoL was assessed with the KINDL-questionnaire at baseline and after the intervention. The IG received daily, short and informative smartphone messages based on Bandura’s social cognitive theory on the subject of MVPA, thus nudging them to be more active in their everyday lives over a period of three month. Intention-to-treat analysis was performed based on multiple imputed data. To analyze the change of MVPA, the first two weeks of the intervention were compared to the last two weeks of the intervention with Mann-Whitney-U test. Results are expressed as median [25 quartile; 75 quartile]. Results MVPA decreased from 78.1 [62.5; 86.5] to 71.4 [56.6; 75.2] min/day in the IC and from 80.2 [64.7; 102.9] to 76.0 [68.0; 94.0] min/day in the CG over the study period of three month. There was no significant change in MVPA when comparing the IG with the CG group (IG: Δ -6.4 [-18.6; 3.0] min/day vs. CG: -2.5 [-12.8; 8.5] min/day, p=0.199). Physical well-being significantly increased over the study period (IG: Δ1.60 [-0.24; 6.25] vs. CG: Δ0.00 [-12.5; 6.35], p=0.043), but not the total HrQoL score (p=0.518). Conclusion Three month of digital health nudging did not increase PA, but improved feelings of physical well-being in adolescents with CHD. Our results might indicate that although wearables and digital health nudging initially have a positive influence on physical activity, activity declines over time in both groups and digital health nudging apparently does not have the potential to stop or slow down this phenomenon.

Design of a multi-institutional neurocognitive discovery study in adult congenital heart disease (MINDS-ACHD)

Neurocognitive dysfunction (NCD) is a common comorbidity among children with congenital heart disease (CHD). However, it is unclear how underlying CHD and its sequelae combine with genetics and acquired cardiovascular and neurological disease to impact NCD and outcomes across the lifespan in adults with CHD. The Multi-Institutional Neurocognitive Discovery Study in Adults with Congenital Heart Disease (MINDS-ACHD) is a partnership between the Pediatric Heart Network (PHN) and the Adult Alliance for Research in Congenital Cardiology (AARCC) that examines objective and subjective neurocognitive function and genetics in young ACHD. This multicenter cross-sectional pilot study is enrolling 500 young adults between 18 and 30 years with moderate or severe complexity CHD at 14 centers in North America. Enrollment includes 4 groups (125 participants each): 1) d-looped Transposition of the Great Arteries (d-TGA); 2) Tetralogy of Fallot (TOF); 3) single ventricle (SV) physiology; and 4) "other moderately or severely complex CHD." Participants complete the standardized tests from the NIH Toolbox Cognitive Battery, the NeuroQoL, the Hospital Anxiety and Depression Scale, and the PROMIS Global QoL measure. Clinical and demographic variables are collected by interview and medical record review, and an optional biospecimen is collected for genetic analysis. Due to the COVID-19 pandemic, participation may be done remotely. Tests are reviewed by a Neurocognitive Core Laboratory. MINDS-ACHD is the largest study to date characterizing NCD in young adults with moderate or severely complex CHD in North America. Its results will provide valuable data to inform screening and management strategies for NCD in ACHD and improve lifelong care.

Digital Health Nudging to increase physical activity in pediatric patients with congenital heart disease: A randomized controlled trial

Digital nudging is a modern e-health approach to increase physical activity (PA) in younger age groups. As activity promotion is particularly important in adolescents with congenital heart disease (CHD) this randomized-controlled trail examines if Digital Health Nudging via daily smartphone messages increases PA, activity-related self-efficacy (ArSE) and health-related quality of life (HrQoL) in adolescents with CHD. From May 2021 to April 2022, 97 patients (15.1 ± 2.0 years, 50% girls) with moderate or severe CHD were randomly allocated 1:1 to intervention (IG) or control group (CG). Daily PA was objectively assessed in minutes of moderate-to-vigorous PA (MVPA) by the wearable "Garmin Vivofit jr. 2®" over the entire study period. The IG received daily smartphone messages based on Bandura's social cognitive theory on the subject of PA over a period of 12-weeks. According to the linear mixed model, the change of MVPA over the study period did not significantly differ between IG and CG when taking baseline MVPA into account (b=0.136, 95%-CI [-0.355; 0.627], p=0.587). Activity level was comparably high and showed only minor variability in both groups with 73.7 [62.3; 78.8] min/day in IG and 78.4 [66.6; 93.9] min/day in CG throughout the whole 12-weeks. Emotional well-being significantly increased over the study period in the IG (IG: Δ1.60 [-0.2; 6.3] vs. CG: Δ0.0 [-12.5; 6.3], p=0.043), but not total HrQoL (p=0.518) and ArSE (p=0.305). 12-weeks of Digital Health Nudging did not increase PA, but improved feelings of emotional well-being in adolescents with CHD. Clinical Trials Identifier NCT04933786.

Evaluation of a new digital pediatric malnutrition risk screening tool for hospitalized children with congenital heart disease

BackgroundIn a cohort of hospitalized children with congenital heart disease (CHD), a new digital pediatric malnutrition screening tool as a mobile application was validated, and its effectiveness and clinical value were determined as a prospective study.Methods and resultsChildren with CHD (n = 1125) were screened for malnutrition risk. The incidence of risk and the differences among various age groups and types of CHD were characterized. The optimal threshold for the tool to determine if there is a risk of malnutrition is score 2, while the Youden index was 79.1%, and the sensitivity and specificity were 91.2% and 87.9%, respectively. Based on such criterion, 351 children were at risk of malnutrition accounting for 31.20% of the total. Compared with the non-malnutritional risk group, the median age for the group at risk for malnutrition was younger (8.641 months [4.8, 23.1] vs. 31.589 months [12.4, 54.3], P < 0.01), and the length of stay was longer (12.000 [8.0, 17.0] vs. (8.420 [5.0, 12.0], P < 0.01]. There were significant differences in malnutrition risk among different age groups (χ2 = 144.933, P < 0.01), and children under one year of age exhibited the highest risk for malnutrition and more extended hospital stay (H = 78.085, P < 0.01). The risk of malnutrition among children with cyanotic CHD was higher than in those with non-cyanotic CHD (χ2 = 104.384, P < 0.01).ConclusionsThe new digital pediatric malnutrition screening tool showed high sensitivity and specificity in children with CHD. The tool indicated that the malnutrition risk for young children and children with cyanotic or Bethesda moderate and complex CHD was higher, and the hospitalization time was longer than in the non-risk group. The tool provides a rational approach to targeted nutrition intervention and support and may improve clinical outcomes.

Transition to adult care of young people with congenital heart disease: impact of a service on knowledge and self-care skills and correlates of a successful transition.

Less than one-third of adolescents with congenital heart disease (CHD) successfully transition to adult care, missing out on education of their cardiac condition, and risking loss to follow-up. We assessed the efficacy of our transition clinic on patient education and empowerment and identified correlates of successful transition. Overall, 592 patients were seen at least once in our transition service between 2015 and 2022 (age 15.2±1.8 years, 47.5% female). Most adolescents (53%) had moderate CHD, followed by simple (27.9%) and severe (19.1%) CHD. Learning disability (LD) was present in 18.9% and physical disability (PD) in 4.7%. In patients without LD, knowledge of their cardiac condition improved significantly from the first to the second visit (naming their condition: from 20 to 52.3%, P<0.0001; describing: 14.4-42.7%, P<0.0001; understanding: 26.1-60.7%, P<0.0001), and from the second to the third (naming: 67.4%, P=0.004, describing: 61.4%, P<0.001, understanding: 71.1%, P=0.02;). Patients with LD did not improve their disease knowledge over time (all P>0.05). Treatment adherence and management involvement, self-reported anxiety, and dental care awareness did not change over time. Successful transition (attendance of≥2 clinics) was achieved in 49.3%. Younger age at the first visit, simpler CHD, and absence of PD were associated with successful transition. A transition service positively impacts on patient education and empowerment in most CHD adolescents transitioning to adult care. Strategies to promote a tailored support for patients with LD should be sought, and earlier engagement should be encouraged to minimize follow-up losses.

Moderate and Severe Congenital Heart Diseases Adversely Affect the Growth of Children in Italy: A Retrospective Monocentric Study.

Children with congenital heart disease (CHD) are at increased risk for undernutrition. The aim of our study was to describe the growth parameters of Italian children with CHD compared to healthy children. We performed a cross-sectional study collecting the anthropometric data of pediatric patients with CHD and healthy controls. WHO and Italian z-scores for weight for age (WZ), length/height for age (HZ), weight for height (WHZ) and body mass index (BMIZ) were collected. A total of 657 patients (566 with CHD and 91 healthy controls) were enrolled: 255 had mild CHD, 223 had moderate CHD and 88 had severe CHD. Compared to CHD patients, healthy children were younger (age: 7.5 ± 5.4 vs. 5.6 ± 4.3 years, p = 0.0009), taller/longer (HZ: 0.14 ± 1.41 vs. 0.62 ± 1.20, p < 0.002) and heavier (WZ: -0,07 ± 1.32 vs. 0.31 ± 1.13, p = 0.009) with no significant differences in BMIZ (-0,14 ± 1.24 vs. -0.07 ± 1.13, p = 0.64) and WHZ (0.05 ± 1.47 vs. 0.43 ± 1.07, p = 0.1187). Moderate and severe CHD patients presented lower z-scores at any age, with a more remarkable difference in children younger than 2 years (WZ) and older than 5 years (HZ, WZ and BMIZ). Stunting and underweight were significantly more present in children affected by CHD (p < 0.01). In conclusion, CHD negatively affects the growth of children based on the severity of the disease, even in a high-income country, resulting in a significant percentage of undernutrition in this population.

Congenital Heart Disease and Fertility: A Danish Nationwide Cohort Study Including Both Men and Women.

Background Despite an increasing number of patients with congenital heart disease (CHD) reaching reproductive age, the fertility of these patients remains undescribed. Therefore, the aim of the study was to evaluate the fertility in men and women with CHD by estimating the risk of infertility and comparing the birth rates, proportions of individuals becoming parents or remaining childless, and the number of children per parent with unaffected individuals. Methods and Results The study population consisted of individuals born between 1977 and 2000. Information on CHD, infertility, and live born children were obtained from the Danish health registries. Hazard ratios for infertility were analyzed using a Cox regression model. Differences of proportions and birth rates were calculated and compared between groups. Among 1 385 895 individuals, a total of 8679 (0.6%) were diagnosed with CHD. Men and women with simple or moderate CHD had no increased risk of infertility when compared with the reference population. Estimates for complex CHD groups were too imprecise for evaluation. Individuals with CHD were more often childless with consequently lower birth rates compared with unaffected individuals. However, those becoming parents had the same number of children as the reference population. Conclusions Men and women with simple or moderate CHD had the same risk of infertility as the reference population. Despite patients with CHD more often being childless, those becoming parents had the same number of children as parents without CHD. The current findings increase the knowledge regarding fertility in the CHD population.

Ambivalence is associated with decreased physical activity and cardiorespiratory fitness among adolescents with critical congenital heart disease

BackgroundAdolescents with congenital heart disease (CHD) are insufficiently physically active. Given that increasing physical activity may reduce their cardiovascular risk, it is important to identify correlates of this behavior. Perceived benefits of and barriers to physical activity are associated with physical activity engagement. Existing research has only considered these constructs separately. This population may be ambivalent toward physical activity (i.e., perceive both strong benefits and barriers). The association of ambivalence and physical activity related outcomes is unknown among this at-risk population. ObjectiveDetermine the association of ambivalence and sedentary behavior, moderate-to-vigorous physical activity (MVPA), and cardiorespiratory fitness (VO2Peak) among adolescents with CHD. MethodsThe present study is an analysis of data from an eligibility assessment for a randomized clinical trial of an intervention to promote MVPA among adolescents aged 15 to 18 years with moderate or complex CHD. Participants (N = 84) completed a survey assessing perceived benefits and barriers from which ambivalence toward physical activity was calculated, an exercise stress test to measure VO2Peak, and wore an accelerometer for one week to determine their engagement in sedentary behavior and MVPA. Linear regression analyses determined associations between ambivalence and physical activity related outcomes. ResultsGreater ambivalence toward physical activity was associated with increased sedentary behavior, decreased MVPA, and reduced VO2Peak, adjusting for demographic and clinical covariates. ConclusionsAmbivalence is associated with objectively measured physical activity (sedentary behavior, MVPA) and a biomarker of cardiovascular health (VO2Peak). Screening for ambivalence may help clinicians identify those most likely to benefit from physical activity-related education.

Aerobic Capacity in Adults with Congenital Heart Disease: More than VO2peak, a Follow-Up Study.

To control the development of people with congenital heart disease (CHD), it is important to follow their aerobic capacity (AC), especially when they exercise. This research aimed to study the progress of AC during a follow-up of adults with CHD. This is a longitudinal study which involved 127 adults with a mean age of 33.8 (11.1) years (57.5% female; 75 moderate CHD and 52 complex CHD) who had undergone two cardiopulmonary exercise tests (CEPT) in at least one year between the first and the second test. The AC and exercise performance (EP) (duration of exercise time, velocity and percentage of grade) were assessed using a ramp protocol over a treadmill. In a mean of 4.5 (2.0) years of follow-up, there was a significant decrease in AC. The VO2peak at baseline was 27.8 (27.7) mL/kg/min (82.9% (20.3%) predicted) versus 26.6 (7.8) mL/kg/min (79.3% (20.8%) predicted) at the end of follow-up. This decline was independent of the body weight increase. There was no significant difference in HRpeak and EP among periods. These results suggest a sign of favorable evolution of adults with CHD. More research is needed to study different factors that could contribute to AC reduction.

Abstract 11040: Disparities in Inpatient Intensity of End-of-Life Care for Children With Advanced Heart Disease

Introduction: Children dying of advanced heart disease experience high intensity end-of-life care (EOLC). However, little is known about disparities in EOLC intensity, which is critical for EOLC equity. Methods: We conducted a population-based retrospective analysis using the California Office of Statewide Health Planning and Development private inpatient discharge database to assess disparities in inpatient EOLC intensity by examining the terminal admission for children (0-21y) with cardiomyopathy and moderate/complex congenital heart disease (CHD) who died in 2005-2018. We examined rates of medical intensity (intubation, CPR, etc.) and cardiac intensity (ECMO, ventricular assist device interventions, other cardiac surgeries, or ≥ 2 cardiac catheterization, biopsy, or electrophysiology procedures). Clinical and sociodemographic factors, including death at a California Children’s Services designated cardiac special care center, associated with medical intensity and cardiac intensity were examined. Results: The 2,794 children died and 76% were &lt; 1 year old. Of them, 26% had cardiomyopathy, 44% moderate CHD, and 46% complex CHD. The majority were male (55%) and publicly insured (64%). The largest racial/ethnic group was Hispanic (50%) followed by non-Hispanic white (26%). Mean length of terminal admission was 31±50d, and 82% died at a cardiac special care center. During the terminal admission, the majority (79%) were intubated, 39% had cardiac surgery, 30% received ECMO, 28% received CPR, and 42% had a cardiac intensity marker. Black children and children with cardiomyopathy were less likely to have a cardiac intensity marker (aOR=0.6 [95% CI: 0.39-0.91]; reference, non-Hispanic white, aOR=0.46 [95% CI: 0.34-0.61]; reference, moderate CHD, respectively). Children who died at a cardiac special care center were more likely to have a cardiac intensity marker (aOR=6.79 [95% CI: 4.95-9.32]; reference, non-specialty center). Conclusion: Children with advanced heart disease in California exhibited high intensity EOLC with significant sociodemographic variation. It is critical to determine if this variation represents patient preferences, provider biases, or systemic factors such as resource availability.

Abstract 12062: Additional Burden of Esophageal and Gastric Varices on Hospitalized Patients With Moderate or Severe Congenital Heart Disease

Introduction: Elevated central venous pressure (CVP) occurs in many forms of congenital heart disease (CHD) which may result in esophageal or gastric varices. We sought to describe the incidence of varices and acute outcomes for hospital admissions with moderate or severe CHD compared to those without CHD. Methods: Vizient Clinical Database was queried from 10/2018-12/2021 for admissions aged 1-89 years with ICD-10 codes for moderate or severe CHD with and without ICD-10 codes for esophageal (I85-I85.11) or gastric (I86.4) varices. For comparison (NO-CHD), admissions with no CHD codes with and without varices were queried. Data included demographics, hospital outcomes, principal procedure and costs. Results: There were 54,979 CHD admissions, 489 (0.9%) with varices and 28,282,750 NO-CHD admissions, 231,633 (0.8%) with varices ( p = 0.069); summary in Table 1. Compared to the NO-CHD population, admission with CHD and varices were younger, had longer lengths of stay, higher complication rates and hospital costs with no difference in in-hospital mortality. Table 2 shows the top 5 principal procedures for each group. Conclusions: Hospitalized patients with moderate or severe CHD and varices have worse acute hospital outcomes and higher costs compared to those without CHD. These findings suggest a potential additional disease burden in this already fragile population. Future work to identify and potentially treat modifiable factors could improve care for these patients.

Abstract 11061: Incidence of Cancer in Hospitalized Adults With Congenital Heart Disease

Introduction: Many patients with congenital heart disease (CHD) are repeatedly exposed to low-dose ionizing radiation for diagnosis, treatment and long-term management of their care. Hypothesis: We hypothesized that these repeated exposures put CHD patients at higher risk for radiation-associated cancers (RAC). Methods: Review of Vizient (a national, administrative database) from 12/2018 - 12/2021 for admissions aged 18-85 years with ICD-10 codes for moderate or severe CHD and RAC (bladder, breast, colon, esophageal, leukemia/myeloma, liver, lung, ovarian and stomach). Demographics, hospital outcomes and costs were compared to admissions with RAC and no CHD (NO-CHD). Results: Out of 34,793 CHD admissions, 6.4% had RAC, and out of 24,999,735 NO-CHD admissions, 11.2% had RAC (p&lt;0.001; Table 1). CHD with RAC were younger, had longer length of stay, higher complications, in-hospital mortality and costs. Comparisons for each RAC type are in Table 2. There were higher rates of breast, liver and lung cancer in CHD (p&lt;0.05 for all) and higher costs for all CHD RAC except esophageal. Conclusions: This national database review shows increased incidence of some types of RAC (lung, liver and breast) in adults with CHD, with worse hospital outcomes and higher costs for several cancer types. While frequent radiation in the CHD population may contribute to these findings, it will be important to explore further. These findings also highlight the importance of adherence to the “as low as reasonably achievable” (ALARA) principle in the care of children and adults with CHD and stresses the importance of imaging modalities that do not use ionizing radiation.

Abstract 15588: Effect of Covid-19 on the Mortality of Patients With Congenital Heart Disease

Introduction: Cardiovascular conditions have been recognized as risk factors for poor outcomes in association with COVID-19. However, the effect of the COVID-19 pandemic on different severity types of congenital heart disease (CHD) patients remains unknown. This study aims to examine whether patients with history of interventions for CHD experienced a higher mortality risk during the COVID-19 pandemic. Methods: This is a retrospective cohort study using data from the Pediatric Cardiac Care Consortium (PCCC). We included patients having US residence and direct identifiers, who were enrolled between 1982 and 2003 after undergoing transcatheter or surgical interventions for CHD. The observation window (2017-2020) was divided to a pre-SARSV2 and SARSV2 exposure period defined around the state specific onset of COVID-19 disease in 2020. Patients were categorized as having mild, moderate, severe-2 ventricle (2V) or single ventricle (SV) CHD. Standardized mortality ratios (SMRs) were used to describe the observed risk of mortality in CHD patients compared to the age- and sex-adjusted rates from the general U.S. population. Multivariable analysis was conducted using time-varying covariate Cox proportional hazards model with adjustment for age, sex and severity of CHD. Results: A total of 50,138 patients with history of interventions for CHD were included. Pooled pre-SARSV2 SMR was 2.38 [95% CI: 2.06, 2.75], while the SARSV2-exposure SMR was 2.07 [95% CI: 1.78, 2.35] (not significantly different). No significant differences in pooled SMRs were found by severity of CHD. The adjusted hazard risk for all-cause mortality in the SARSV2-exposure vs pre-SARSV2 period did not increase for patients with CHD (aHR: 0.59, 95%CI:0.19 - 1.84), and we did not find any differences in risk by severity subgroups or sex of patients. Conclusion: In this large cohort study monitoring long term outcomes after interventions for CHD, we could not detect any evidence of increased risk for all-cause mortality among this population during the early part of the COVID-19 pandemic. Additional data will be required over longer period to fully understand the impact of COVID-19 on adults with repaired CHD.

Acute Hospital Outcomes for Renal Transplantation in Patients With Moderate or Severe Congenital Heart Disease

Children and adults with congenital heart disease (CHD) are increasingly recognized to be at risk for acute and chronic renal injury. Some of these may progress to the need for renal transplantation. We hypothesized that patients with underlying moderate or severe CHD who undergo renal transplantation will have worse acute hospital outcomes. Using a national administrative database, we queried for admissions aged 0 to 50 years with moderate or severe CHD and renal transplantation and compared these to admissions without CHD. There were 56 admissions for renal transplantation in the CHD group (0.04%) and 26,285 admissions in the group without CHD (0.21%, p<0.001). The CHD group were younger, had a higher proportion of Whites, longer length of stay, higher complication rates, higher in-hospital mortality, and higher costs. In conclusion, although renal transplantation is still relatively uncommon in the CHD population, there is an increasing recognition of severe chronic renal disease in the setting of CHD, making it important to understand the potential implications of these findings.