Adults With Congenital Heart Disease Patients Research Articles

The Appraisal of Adults with Congenital Heart Disease: Lesson from Comparison of Surgical Outcomes.

The population of adults with congenital heart disease (ACHD) is constantly growing. There seems to be a consensus that these patients are difficult to manage especially if compared to patients with acquired heart disease. The aim of this study is to compare outcomes and results of cardiac surgery in ACHD patients with a reference population of adults with acquired cardiac disease. Retrospective study of 5053 consecutive patients older than 18years hospitalized for cardiac surgery during a 5-years period in our Institution. Two groups of patients were identified. Group I: 419 patients operated for congenital heart disease; Group II: 4634 patients operated for acquired heart disease. In each Group were identified low, medium, and high-risk patients, according to validated scores. Right ventricular outflow tract surgery was the most frequent procedure in Group I, while coronary artery by-pass grafting was the most common in Group II. Patients with ACHD were younger (37.8 vs. 67.7years), with higher number of previous operations (32.1% vs. 6.9%), had longer post-ICU hospital stay (11 vs. 8days) but had lower ICU stay (1 vs. 2days), shorter assisted mechanical ventilation (12 vs. 14h) and lower surgical mortality (1 vs. 3.7%) (all p < 0.001). No differences were found in term of post-operative complications (12.4 vs. 15%). The surgical treatment of ACHD patients can be done with excellent results and if compared with acquired cardiac disease patients they have better results with shorter ICU stay and lower mortality.

PREVALENCE OF ELEVATED LOW DENSITY LIPOPROTEIN CHOLESTEROL (LDL–C) LEVELS IN ADULT PATIENTS WITH COMPLEX CONGENITAL HEART DISEASE

Abstract Background Thanks to advances in both medical care and surgical techniques, survival for adult patients with congenital heart disease (ACHD) has significantly increased over recent years, with about 90% of them now reaching adulthood. However, as CHD patients grow older, their relative risk of developing coronary artery disease (CAD) increases. Elevated low–density lipoprotein cholesterol (LDL–C) levels play a pivotal role in the genesis of CAD in the general population. Limited data exist on the prevalence of hyperlipidemia in the ACHD population. Patients with complex CHD should be considered at increased risk for CAD, mainly related to to the severity of the CHD and aging. For this reason, it could be reasonable to apply targets used for patients at high risk of CAD. Our study aimed at describing the overall prevalence of elevated LDL–C levels in a contemporary cohort of adult patients with complex CHD. Materials and Methods This is a single–centre retrospective study. All adults with complex CHD who attended the outpatient clinic at our tertiary centre from January 2000 to April 2023 were included. Demographic, clinical and laboratory data were obtained in all patients from medical records and our dedicated databases. Results A total of 230 patients with complex CHD were included in the present study (mean age 35±11 years, 124 male, mean follow up of 117±80 months). One hundred forty–three (63%) of them showed biventricular physiology, whereas 86 (37%) patients showed univentricular heart physiology. Eighty patients from our cohort underwent Fontan operation. Twenty patients (9%) showed oxygen saturation at rest lower than 90%. Mean levels of LDL–C was 87±28 mg/dl, with 184 patients (78%) showing LDL–C levels ≥ 55 mg/dl. Conclusion Our study demonstrated that, despite their relatively younger age, elevated LDL–C levels are not uncommon in patients with severe CHD. ACHD patients with complex anatomy, such as patients after Fontan operation, should be considered at increased risk of ischaemic events and treated accordingly. Further research and international collaboration are therefore paramount to understand whether and if these patients should be treated with lower lipid drugs as for the general population.

B-type natriuretic peptide levels predict long-term mortality in a large cohort of adults with congenital heart disease.

Many adult patients with congenital heart disease (ACHD) are still afflicted by premature death. Previous reports suggested natriuretic peptides may identify ACHD patients with adverse outcome. The study investigated prognostic power of B-type natriuretic peptide (BNP) across the spectrum of ACHD in a large contemporary cohort. The cohort included 3392 consecutive ACHD patients under long-term follow-up at a tertiary ACHD centre between 2006 and 2019. The primary study endpoint was all-cause mortality. A total of 11 974 BNP measurements were analysed. The median BNP at baseline was 47 (24-107) ng/L. During a median follow-up of 8.6 years (29 115 patient-years), 615 (18.1%) patients died. On univariable and multivariable analysis, baseline BNP [hazard ratio (HR) 1.16, 95% confidence interval (CI) 1.15-1.18 and HR 1.13, 95% CI 1.08-1.18, respectively] and temporal changes in BNP levels (HR 1.22, 95% CI 1.19-1.26 and HR 1.19, 95% CI 1.12-1.26, respectively) were predictive of mortality (P < .001 for both) independently of congenital heart disease diagnosis, complexity, anatomic/haemodynamic features, and/or systolic systemic ventricular function. Patients within the highest quartile of baseline BNP (>107 ng/L) and those within the highest quartile of temporal BNP change (>35 ng/L) had significantly increased risk of death (HR 5.8, 95% CI 4.91-6.79, P < .001, and HR 3.6, 95% CI 2.93-4.40, P < .001, respectively). Baseline BNP and temporal BNP changes are both significantly associated with all-cause mortality in ACHD independent of congenital heart disease diagnosis, complexity, anatomic/haemodynamic features, and/or systolic systemic ventricular function. B-type natriuretic peptide levels represent an easy to obtain and inexpensive marker conveying prognostic information and should be used for the routine surveillance of patients with ACHD.

Delayed Sternal Closure vs Emergency Sternal Reopening in Adults with Congenital Heart Disease

Limited data exist regarding outcomes of delayed sternal closure (DSC) in adults with congenital heart disease (ACHD). We reviewed 159 ACHD patients undergoing cardiac surgery 1993-2023, who required DSC (open sternum at the end of operation, n=112) or ESR (sternum emergently reopened, n=47). Regression models were performed to determine factors associated with outcomes. Of 112 patients undergoing DSC, 87 patients (77.6%) underwent DSC ≤4 days, and 25 patients (22.3%) >4 days. The most common operations were valve (n=35, 31.2%), aortic (n=33, 29.4%), and right ventricular outflow tract procedures (n=23, 20.5%). Median time to chest closure was 2 days (interquartile range 1, 5). Apart from gender, baseline characteristics were similar between DSC groups. A stepwise increase in early mortality was observed from DSC≤4 days to DSC>4 days (6.8% vs 32%), as well as incidence of early complications, except sternal infection. Risk factors associated with early mortality were age (p=0.02), DSC >4 days (p<0.001), hemodynamic indication (p=0.03), and single ventricle (p=0.02). On multivariable analysis, lower ejection fraction (p=0.04), hemodynamic indication (p=0.02), single ventricle (p=0.004), and diabetes mellitus (p=0.03) were predictors of prolonged time to chest closure. Amongst hospital survivors, there was no difference in late survival between patients undergoing DSC ≤4 days vs >4 days (p=0.48). Brief duration of DSC in ACHD patients is associated with low morbidity and mortality. Higher early mortality and complications were observed among patients who did not achieve chest closure within 4 days.

Prognostic significance of echocardiographic deformation imaging in adult congenital heart disease.

Due to medical and surgical advancements, the population of adult patients with congenital heart disease (ACHD) is growing. Despite successful therapy, ACHD patients face structural sequalae, placing them at increased risk for heart failure and arrhythmias. Left and right ventricular function are important predictors for adverse clinical outcomes. In acquired heart disease it has been shown that echocardiographic deformation imaging is of superior prognostic value as compared to conventional parameters as ejection fraction. However, in adult congenital heart disease, the clinical significance of deformation imaging has not been systematically assessed and remains unclear. According to the Preferred Reporting Items for Systematic Reviews checklist, this systematic review included studies that reported on the prognostic value of echocardiographic left and/or right ventricular strain by 2-dimensional speckle tracking for hard clinical end-points (death, heart failure hospitalization, arrhythmias) in the most frequent forms of adult congenital heart disease. In total, 19 contemporary studies were included. Current data shows that left ventricular and right ventricular global longitudinal strain (GLS) predict heart failure, transplantation, ventricular arrhythmias and mortality in patients with Ebstein's disease and tetralogy of Fallot, and that GLS of the systemic right ventricle predicts heart failure and mortality in patients post atrial switch operation or with a congenitally corrected transposition of the great arteries. Deformation imaging can potentially impact the clinical decision making in ACHD patients. Further studies are needed to establish disease-specific reference strain values and ranges of impaired strain that would indicate the need for medical or structural intervention.

Single-Chamber and Dual-Chamber Pacemaker Devices in Adults with Moderate and Complex Congenital Heart Disease: A Single Tertiary Referral Center Experience.

The number of device implantation procedures has increased in adult patients with congenital heart disease (ACHD). Despite significant improvements in materials and implantation techniques, these patients are exposed to higher risk of device related complications than general population. Herein, we describe our single tertiary referral center experience on transvenous pacemaker (PM) implantation and follow-up in adult patients with moderate and complex congenital heart disease (CHD) as limited data are available on long-term outcome. We considered all adults with moderate and complex CHD aged more than 16 years who underwent transvenous single-chamber and dual-chamber PM implant for sinus node dysfunction or atrioventricular block between January 2013 to December 2022 at our Unit. Seventy-one ACHD patients were included in the study (mean age 38.6 ± 15.2 years, 64% with moderate CHD, 36% with complex CHD). Among 32 patients implanted with a dual chamber PM (DDD PM), 4 devices were reprogrammed in VDD mode, 3 in VVI and 2 in AAI mode during follow-up because of lead dysfunction or permanent atrial arrhythmia. In addition, 26 patients had a single chamber PM (AAI or VVI PM) and 13 patients had single-lead pacing system with a free-floating atrial electrode pair (VDD PM). Just one of 13 single-lead VDD PM was reprogrammed in VVI mode due to a low atrial sensing. In DDD PM group, 10 re-interventions were needed due to lead dysfunction (8 cases) and lead-related infective endocarditis (2 cases). Only 3 patients in the single-lead PM group developed lead dysfunction with 2 re-interventions needed, but no infective endocarditis was reported. The rate of long-term complications is high in moderate and complex ACHD with transvenous PM devices, and it is mainly lead-related. In our experience, the less leads implanted, the less complications will occur. Considering the heterogeneity of the ACHD population, transvenous single-chamber or dual-chamber PM device implantation should always be tailored on the single patient, balancing risks and benefits in this complex population.

Abstract WP279: Stroke in Young Adults With Congenital Heart Disease a Single Center Case Control Study

Introduction: Adults with congenital heart disease (ACHD) are at risk for stroke, but risk factors within this population are not well characterized. Methods: We reviewed medical records of patients evaluated in an ACHD clinic from 8/1/1995- 3/1/2022 who underwent brain imaging for any reason. We categorized patients by anatomical complexity based on American Heart Association guidelines (simple, moderate or high complexity). The primary outcome was ischemic or hemorrhagic stroke. TIA and silent infarcts were excluded. We created logistic regression models to calculate odds ratios (OR) and 95% confidence intervals (CI) for the association between anatomic complexity and stroke. Results: Out of 184 patients, 48 (26.1%) had any stroke (62% ischemic, 38% hemorrhagic). Median age at first stroke was 26.2 years (IQR 19.2-38.5) (Figure). Those with stroke had higher proportion of Black race, more major cardiac surgeries, and more use of antithrombotic medications prior to stroke (see Table). Adjusting for age, sex, race, number of cardiac surgeries, and use of antithrombotic medications, those with highly complex lesions had a trend towards higher odds of any stroke (adjusted OR 2.0, 95% CI 0.89 - 4.4). Effect size was higher for ischemic stroke (adjusted OR 2.6, 95% CI 0.99-6.7). Conclusion: In our sample of 184 ACHD patients who underwent brain imaging, stroke was common with half of strokes occurring before the age of 26 years. High complexity cardiac lesions were associated with more than double the odds of ischemic stroke. Larger studies are needed to develop effective stroke prevention strategies in this growing young adult population.

Coronary artery disease in older adults with congenital heart defects: risk factors and pharmacotherapy.

With advancing age, adults with congenital heart disease (ACHD) are at a higher risk of developing atherosclerotic coronary artery disease (CAD). We aimed to determine the prevalence of CAD, its risk factors, and use of guideline‑directed pharmacotherapy among older patients with ACHD. Patients and methods: We studied all ACHD patients aged 60 years or older hospitalized in our department between the years 2013 and 2020. CAD was defined as a history of acute coronary syndrome or coronary revascularization, or more than 50% diameter stenosis on coronary angiography. Data regarding the underlying heart defect, prevalence of cardiovascular risk factors, and drug prescriptions were collected. A total of 198 patients with known coronary artery status (mean [SD] age, 66.2 [5.3] years; 43.3% men) were included in the analysis. Of them, 54 (27.3%) had CAD. The individuals with CAD were more often men, and they were more likely to have a mild heart defect, dyslipidemia, and a history of hypertension and tobacco use. Multivariable analysis showed that male sex (P = 0.001), dyslipidemia (P = 0.003), and hypertension (P = 0.04) were positive independent predictors of CAD, whereas overweight / obesity was identified as a negative independent predictor (P = 0.04). The proportion of CAD patients on antiplatelet and / or anticoagulant drugs was 92.6%. β‑Blockers were prescribed to 87% of the patients, and a lipid‑lowering agent to 96% of the study population. CAD is common in older patients with ACHD. Our results underline the importance of identification and treatment of modifiable CAD risk factors in individuals with ACHD. The obesity paradox might also play a role in this population. The rate of guideline‑recommended pharmacotherapy implementation seems to be satisfactory.

COVID-19–Related Thrombotic and Bleeding Events in Adults With Congenital Heart Disease

BackgroundAltered coagulation is a striking feature of COVID-19. Adult patients with congenital heart disease (ACHD) are prone to thromboembolic (TE) and bleeding complications. ObjectivesThe purpose of this study was to investigate the prevalence and risk factors for COVID-19 TE/bleeding complications in ACHD patients. MethodsCOVID-19-positive ACHD patients were included between May 2020 and November 2021. TE events included ischemic cerebrovascular accident, systemic and pulmonary embolism, deep venous thrombosis, myocardial infarction, and intracardiac thrombosis. Major bleeding included cases with hemoglobin drop >2 g/dl, involvement of critical sites, or fatal bleeding. Severe infection was defined as need for intensive care unit, endotracheal intubation, renal replacement therapy, extracorporeal membrane oxygenation, or death. Patients with TE/bleeding were compared to those without events. Factors associated with TE/bleeding were determined using logistic regression. ResultsOf 1,988 patients (age 32 [IQR: 25-42] years, 47% male, 59 ACHD centers), 30 (1.5%) had significant TE/bleeding: 12 TE events, 12 major bleeds, and 6 with both TE and bleeding. Patients with TE/bleeding had higher in-hospital mortality compared to the remainder cohort (33% vs 1.7%; P < 0.0001) and were in more advanced physiological stage (P = 0.032) and NYHA functional class (P = 0.01), had lower baseline oxygen saturation (P = 0.0001), and more frequently had a history of atrial arrhythmia (P < 0.0001), previous hospitalization for heart failure (P < 0.0007), and were more likely hospitalized for COVID-19 (P < 0.0001). By multivariable logistic regression, prior anticoagulation (OR: 4.92; 95% CI: 2-11.76; P = 0.0003), cardiac injury (OR: 5.34; 95% CI: 1.98-14.76; P = 0.0009), and severe COVID-19 (OR: 17.39; 95% CI: 6.67-45.32; P < 0.0001) were independently associated with increased risk of TE/bleeding complications. ConclusionsACHD patients with TE/bleeding during COVID-19 infection have a higher in-hospital mortality from the illness. Risk of coagulation disorders is related to severe COVID-19, cardiac injury during infection, and use of anticoagulants.

Subcutaneous implantable cardioverter-defibrillator in adults with congenital heart disease

Abstract Background Subcutaneous implantable cardioverter-defibrillators (S-ICD) are an established treatment option for the prevention of sudden cardiac death (SCD). However, data in adults with congenital heart disease (ACHD), especially in direct comparison to other patient groups (e.g. cardiomyopathies) are scarce. Methods Retrospective single-center study of all patients receiving a S-ICD in the time-period 2012-2020. ACHD patients were compared to non-ACHD patients. Primary outcome was all-cause mortality. Secondary outcome was a combined endpoint of all-cause mortality and appropriate shocks. Results Altogether, 79 patients (11 ACHD, 68 non-ACHD patients) with a mean age of 40.6 ± 16.0 years (29.1% female) were included. There was no significant difference for age and sex between both groups. Secondary prevention of SCD was more often the indication for a S-ICD in ACHD (81.8%) than in non-ACHD patients (48.5%; p=0.040). Betablockers were more commonly used in ACHD patients (100% vs. 64.7%; p=0.018). There was no significant difference for other heart failure medications. During a median follow up of 3.4 years [IQR 1.7-5.2], the primary endpoint occurred in 36.4% of ACHD vs. 2.9% of non-ACHD patients (p&amp;lt;0.001). The secondary endpoint occurred also more often in the ACHD patient group compared to non-ACHD patients (54.5% vs. 17.6%, p=0.007). There was a numerically higher number of inappropriate shocks in the ACHD group (27.3% vs. 9.0%), albeit without a statistically significant difference (p=0.078). Conclusions In ACHD patients with a S-ICD, mortality as well as a combined endpoint of mortality and appropriate shocks were more common than in non-ACHD patients. The numerically higher number of inappropriate shocks deserves further attention.

Safety of cardiopulmonary exercise testing in a large contemporary cohort of adults with congenital heart disease: implications on levels of test supervision

Abstract Background Exercise intolerance is common in adults with congenital heart disease (ACHD). Cardiopulmonary exercise testing (CPET) is used routinely as an objective assessment of the exercise capacity, the severity and mechanisms of exercise intolerance, and the effect of intervention. Little is known on the safety of CPET in ACHD, resulting in the American Heart Association recommending physician supervision of all patients with intracardiac shunts. Purpose To assess the safety of CPET in ACHD. Methods We retrospectively examined 9309 CPETs performed on ACHD patients in a tertiary referral Centre over 20 years to document major or minor events. All tests were performed in-hospital, with an emergency alarm system on site, and supervised routinely by 2 clinical exercise physiologists. Major events were classified as death within 48 hours and cardiac arrest, syncope, sustained/symptomatic ventricular tachycardia (VT) and sustained/symptomatic supraventricular tachycardia (SVT) during the test. Minor events were classified as non-sustained/asymptomatic VT, non-sustained/asymptomatic SVT, bradyarrhythmia, chest pain and significant ST segment ECG changes, new onset of left or right bundle branch block). Results During the study period, 9309 CPETs were performed on 4941 ACHD patients (age 33.0±14.7 years, 52.3% male), of varying complexity by Bethesda classification (18.3% simple, 51.2% moderate, 27.4% great) Figure 1. A major event occurred in 56 (0.6%) tests and a minor event in 870 (9.3%) tests. There were no deaths within 48 hours, nor cardiac arrests during the test. There were 6 (0.1%) cases of sustained or symptomatic VT, 4 (&amp;lt;0.1%) syncopal episodes and 46 (0.5%) instances of sustained or symptomatic SVT. A total of 31 (0.3%) episodes of bradyarrhythmia, 377 (4%) significant ECG changes and 267 (2.8%) episodes of chest pain were recorded. Moreover, there were 107 (1.2%) cases of non-sustained or asymptomatic VT and 88 (1%) cases of non-sustained or asymptomatic SVT. Patient fatigue (3701, 40%), shortness of breath (2246, 24.1%) and leg discomfort (1286, 13.8%) were common reasons for stopping the test, whereas only 95 (1%) tests were stopped due to chest pain. Conclusions Our data on a large ACHD population undergoing CPET demonstrate a potential risk for arrhythmic complications during exercise and recovery, mostly due to sustained or symptomatic SVT, while the risk of all the other major cardiovascular events is low. Our data support the use of CPET in routine ACHD practice, with quick recognition and management of potential adverse events in a controlled environment by appropriately trained cardiac physiologists.

Abstract 17646: Maternal and Neonatal Outcomes in Pregnant Women With Congenital Heart Disease: The Magee Obstetric Maternal and Infant (MOMI) ACHD Substudy

Introduction: Adult patients with congenital heart disease (ACHD) have a life-long burden of cardiovascular morbidity that contributes to increased maternal and neonatal risk during pregnancy and the peripartum period. Hypothesis: Women with ACHD and their neonates have a higher risk of adverse pregnancy and peripartum outcomes as compared to women without ACHD. Methods: We matched 565 deliveries from women with ACHD and 1130 deliveries from women without cardiac disease (1:2 matching), for a delivery year, race and maternal age as a substudy of the Magee Obstetric Maternal and Infant (MOMI) study, of women who received perinatal care at the Magee-Womens Hospital at the University of Pittsburgh Medical Center between 1995-2020. We evaluated for in-hospital maternal and neonatal outcomes. Results: Of the 565 women with ACHD, 24.0% had aortic outflow and mitral valve abnormalities, 19.5% had pulmonary outflow and tricuspid valve abnormalities, 28.1% had isolated atrial or ventricular septal defects, 0.3% had single ventricle physiology, and 1.4% had outflow vessel abnormalities. Maternal and neonatal outcomes are summarized in Table 1. Women with ACHD had a higher rate of pregnancy loss (32.4% vs 26.2% p&lt;0.001), induction of labor, and cesarean section. Neonates from ACHD women were delivered at an earlier gestational age, with a lower birth weight, and had a higher incidence of respiratory distress syndrome. ACHD women had a higher incidence of cardiovascular events (predominantly arrhythmias and pulmonary edema) and required intensive care more frequently (10.5% vs 1.2%, p&lt;0.001) and for longer periods (2.1±1.8 vs. 1.1±0.3 days; P=0.027) after delivery. Conclusion: Women with ACHD are at higher risk for adverse clinical outcomes during pregnancy and delivery. Neonates of ACHD women are delivered slightly earlier by induction of labor and have consequent sequelae after birth.

Abstract 15341: A Prospective Study on the Safety and Effectiveness of Apixaban for the Prevention of Thromboembolism in Adults With Congenital Heart Disease and Atrial Arrhythmias: The PROTECT-AR Study

Introduction: In adults with congenital heart disease (ACHD), atrial arrhythmias (AA) confer an increased risk of thromboembolic events. Limited data exist on non-vitamin K oral anticoagulant (NOAC) treatment for ACHD. We aimed to assess the effectiveness and safety of apixaban in ACHD patients with AA. Methods: PROTECT-AR (NCT03854149) was a prospective, multicenter, observational study conducted from 2019 to 2023. ACHD patients with atrial fibrillation, atrial flutter, or intra-atrial re-entrant tachycardia, who were routinely treated with apixaban, were included. The primary efficacy endpoint was the composite of stroke or thromboembolism. The primary safety endpoint was major bleeding. Patients who were previously on vitamin K antagonists (VKAs) before transitioning to apixaban served as a historical control group. Results: In total, 218 patients with ACHD and AA on apixaban (previous VKA users 34.9%) were included (mean age 51±17 years; 45.9% male; predominantly moderate complexity). Over a mean follow-up of 2.4±1.3 years, the rate of stroke or thromboembolism was 0.57% [95% confidence interval (CI): 0.15-1.55] and the rate of major bleeding was 1.52% [95%CI: 0.71-2.88] per patient-year, respectively. In the subset of patients who were previously on VKA, the risk of the primary endpoints did not differ significantly between apixaban and VKA-treatment periods (Figure). Conclusion In ACHD patients with AA on routine apixaban treatment, the risk of major thromboembolic and bleeding events was low. Among prior VKA users, the risk of adverse events during the apixaban-treatment period was comparable to that during the VKA-treatment period. Prospective studies directly comparing NOAC and VKA-treated patients are needed.

Abstract 17447: Effects of Inflammatory Biomarkers on Epigenetic Age Acceleration in Adult Congenital Heart Disease

Introduction: DNA methylation (DNAm) changes reflect alterations in the immune response to cardiac surgery in older adults. However, the role of inflammation on epigenetic age acceleration (EAA) in adults with congenital heart disease (ACHD) who underwent palliative cardiac surgery during childhood is unknown. Hypothesis: ACHD patients with Fontan(F) procedures would have advanced EAA due to higher DNAm predicted IL-6 and CRP levels compared to healthy adults or ACHD patients who underwent single surgery as an infant, likely capturing chronic inflammation associated with the abnormal pathophysiologic state. Methods: A case-control study in young adults (aged 20-30 years) with Fontan circulation or single surgery (SS) as an infant at a tertiary ACHD hospital. DNAm were measured by the EPIC Array. DNAm predicted IL-6 and CRP level differences were tested by robust linear model (rlm) adjusted for age, sex, cell types, and self-reported ethnicity. EAAs were calculated by extracting residuals by regressing epigenetic ages on chronological age in linear regression models. EAA differences were tested by rlm adjusted for the same covariates. Contribution analysis was performed to determine contribution of IL-6 and CRP to EAA. Results: Thirteen Fontan patients (6 female: 7 male, median age: 23) and 5 SS patients (3 female: 2 male, median age: 23) were compared with 20 age-matched controls (15 female: 5 male, median age: 27). Each subgroup had a predominant white race and non-Hispanic ethnicity. The F group has a statistically higher predicted IL-6 and CRP level (p&lt;0.001). Also, the F group had significantly higher EAA for mortality predictor Grimage (p&lt;0.001) and PhenoAge (p&lt;0.001). Conclusions: Patients who underwent Fontan procedure experience advanced EAA contributed by inflammatory biomarkers at a young age. Future studies evaluating the key predictors that drive EAA difference is needed to understand the impact of chronic alteration of physiology.

Abstract 17374: Epigenetic Age Acceleration in Adult Congenital Heart Disease

Introduction: DNA methylation (DNAm) changes reflect alterations in the immune response to cardiac surgery in older adults. However, whether adults with congenital heart disease (ACHD) who underwent palliative cardiac surgery during childhood experience epigenetic age acceleration (EAA) is unknown. Hypothesis: ACHD patients with Fontan(F) procedures would have advanced EAA compared to healthy adults. Methods: A case-control study in young adults (aged 20-30 years) with Fontan circulation at a tertiary ACHD hospital with age-matched healthy adults. DNAm were measured by the EPIC Array. EAAs were calculated by extracting residuals by regressing epigenetic ages on chronological age in linear regression models for four epigenetic clocks. DNAm-based cell type proportions and EAA differences between the two groups were tested by robust linear models (rlm) adjusted for age, sex, and self-reported ethnicity. Results: Thirteen Fontan patients (6 female: 7 male) were compared with 20 age-matched controls (15 female). The median age of the F group was 23 years, compared to 27 years in control. Both subgroups had a predominant white race and non-Hispanic ethnicity. The F group has lower B memory cells and naïve CD4+ cells than controls (adjusted p&lt;0.05). The F group has statistically significant higher Grimage and Phenoage EAA than the controls (p&lt;0.01). Conclusions: Patients who underwent the Fontan procedure experience advanced EAA at a young age. Future studies evaluating the key predictors that drive EAA difference is needed to understand the impact of chronic alteration of physiology.

Abstract 15763: Increasing Diversity Among Research Participants Through Patient Engagement in the Congenital Heart Initiative

Background: The Congenital Heart Initiative (CHI) is the first patient-powered digital registry for adults with congenital heart disease (ACHD). The CHI-Redefining Outcomes and Navigation to Adult-Centered Care (CHI-RON) study is built upon the CHI as a unique collaboration with PCORnet. Goal: Recruitment of populations that are racially diverse, younger, out-of-care, and male has been challenging in ACHD studies. Our goal was to design patient engagement and recruitment strategies to increase these underrepresented populations. Methods: Launched in December 2020, patients across the globe can self-enroll in the CHI registry. The CHI-RON study, initiated in May 2022, recruits ACHD patients at 12 PCORnet centers. Patients are involved in all aspects of the study including recruitment (see Figure 1). Results: As of May 2023, a total of 2,520 participants were recruited by CHI-RON centers while 1,494 self-enrolled in the CHI. Most participants report complex CHD anatomy (n=1,660, 49%). A total of 762 (36%) participants are considered out of care. CHI-RON recruitment methodologies increased representation when compared to self-enrolled CHI participants in terms of ethnicity (10.3 % vs 7.4% Hispanic, p=0.005), race (5.4% vs 2.6%, African American, p&lt;0.001), sex (41% vs 28% male, p&lt;0.001), age (34.8 +/-12.5 years vs 44.5 +/- 14.3 years, p&lt;0.001), and education (33% vs 22% high school equivalent or less, p&lt;0.001). Most patients involved in the study team (n=11, 83%) reported a very to extremely high degree of meaningfulness (average score 85.2) per the Patient Engagement in Research Scale (PEIRS-22). The mean scores of each of the seven subdomains demonstrated significant meaningfulness, especially in the subdomains of convenience, contributions, and benefits. Conclusions: Patient engagement is critical to improving the inclusion of underrepresented populations in clinical research and ensuring alignment with the needs of ACHD patients.

Risk factors and early outcomes of repeat sternotomy in 1960 adults with congenital heart disease: A 30-year, single-center study

ObjectivePatients with congenital heart disease (CHD) increasingly live into adulthood, often requiring cardiac reoperation. We aimed to assess the outcomes of adults with CHD (ACHD) undergoing repeat sternotomy at our institution. MethodsReview of our institution's cardiac surgery database identified 1960 ACHD patients undergoing repeat median sternotomy from 1993 to 2023. The primary outcome was early mortality, and the secondary outcome was a composite end point of mortality and significant morbidity. Univariable and multivariable logistic regression models were used to determine factors independently associated with outcomes. ResultsOf the 1960 ACHDs patient undergoing repeat sternotomy, 1183 (60.3%) underwent a second, third (n = 506, 25.8%), fourth (n = 168, 8.5%), fifth (n = 70, 3.5%), and sixth sternotomy or greater (n = 33, 1.6%). CHD diagnoses were minor complexity (n = 145, 7.4%), moderate complexity (n = 1380, 70.4%), and major complexity (n = 435, 22.1%). Distribution of procedures included valve (n = 549, 28%), congenital (n = 625, 32%), aortic (n = 104, 5.3%), and major procedural combinations (n = 682, 34.7%). Overall early mortality was 3.1%. Factors independently associated with early mortality were older age at surgery, CHD of major complexity, preoperative renal failure, preoperative ejection fraction, urgent operation, and postoperative blood transfusion. In addition, sternotomy number and bypass time were independently associated with the composite outcome. ConclusionsDespite the increase in early mortality with sternotomy number, sternotomy number was not independently associated with early mortality but with increased morbidity. Improvement strategies should target factors leading to urgent operations, early referral, along with operative efficiency including bypass time and blood conservation.

Risk of Infective Endocarditis Post-transcatheter Pulmonary Valve Replacement Versus Surgical Pulmonary Valve Replacement: A Systematic Review.

Pulmonary valve replacement (PVR) is the most common cardiac operation in adult patients with congenital heart disease (ACHD). It can improve right ventricular outflow tract (RVOT) obstruction, typically due to pulmonary valve stenosis or regurgitation. PVR can be performed surgically (open-heart) and through a transcatheter (percutaneous) method, which is minimally invasive and is associated with shorter hospitalization stays. However, following PVR, infectious endocarditis (IE) can complicate the recovery process and increase mortality in the long term. IEis a rare but deadly multi-organ system condition caused by microorganisms traversing the bloodstream from a specific entry point. It can have many presentations, such as splinter hemorrhages, fevers, and vegetation on valves that lead to stroke consequences. This paper aims to evaluate the differences in the rate, etiology, manifestations, treatment, and outcomes of IE following surgical and transcatheter PVR, as the goal is to perform a procedure with few complications. In both approaches, Staphylococcusaureus was the most common microorganism that affected the valves, followed by Streptococcus viridians. Research has shown that surgical pulmonary valve replacement (SPVR) has a decreased risk of IE following surgery compared to TPVR. However, TPVR is preferred due to the reduced overall risk and complications of the procedure. Despite this, the consensus on mortality rates does differ. Future research should consider the type of valves used for transcatheter pulmonary valve replacement (TPVR), such as Melody valves versus Edward Sapien valves, as their IE rates vary significantly.

Cardiac autonomic dysfunction in adult congenital heart disease

PurposeDue to recent advances in diagnosis and treatment, the number of adults with congenital heart disease (ACHD) has substantially increased. This achievement is mitigated by rhythm disorders. Here, we sought to determine alterations in heart rate variability (HRV) and their prognostic value in ACHD.MethodsNinety seven ACHD patients (39.2 ± 14.1 years, 51.5% female) and 19 controls (39.7 ± 15.0 years, 47.4% female) underwent 24-h Holter monitoring.ResultsAs compared to controls, ACHD patients offered a significantly higher burden of premature ventricular contractions (p = 0.02) and decreased HRV indices (natural logarithmic transformation of very low frequency (lnVLF): 7.46 ± 0.76 ms2 vs. 7.91 ± 0.92ms2, p = 0.03; natural logarithmic transformation of low frequency (lnLF): 6.39 ± 0.95ms2 vs. 7.01 ± 1.07ms2, p = 0.01; natural logarithmic transformation of the ratio of low to high frequency spectra (lnLF/HF): 0.81 ± 0.74 vs. 1.17 ± 0.51, p = 0.04). No differences in HRV measures were observed across ACHD lesion groups. NT-proBNP levels were significantly related to both time and frequency domain indices (natural logarithmic transformation of the standard deviation of NN intervals (lnSDNN): Spearman´s rho = -0.32, p = 0.001; natural logarithmic transformation of the standard deviation of the average NN intervals for each 5-min segment of a 24-h Holter monitoring (lnSDANN): Spearman´s rho: -0.33, p = 0.001; natural logarithmic transformation of the total power (lnTP): Spearman´s rho: -0.25, p = 0.01; lnVLF: Spearman´s rho: -0.33, p = 0.001; lnLF: Spearman´s rho: -0.35, p < 0.001; lnLF/HF: Spearman´s rho: -0.34, p = 0.001).After a mean follow-up of 3.9 ± 0.7 years, 8 patients died and 3 patients survived sudden cardiac death (SCD). Several HRV parameters were significantly higher in event-free ACHD patients than in those who died or survived SCD (natural logarithmic transformation of the average of the standard deviations of NN intervals for each 5-min segment of a 24-h Holter monitoring (lnASDNN): p = 0.04; lnPNN30: p = 0.04; lnVFL: p = 0.03; lnLF: p < 0.01). On univariate Cox regression analysis, the time domain indices lnSDNN, lnASDNN and lnPNN30, as well as the frequency domain parameters lnTP, lnVLF and lnLF were associated with death and survived cardiac arrest.ConclusionACHD is accompanied by HRV impairment that carries prognostic implications on ACHD mortality and survived SCD.

Comparison of advanced echocardiographic right ventricular functional parameters with cardiovascular magnetic resonance in adult congenital heart disease

Abstract Aims Advanced transthoracic echocardiography (TTE) using volumetric and deformational indices provides detailed quantification of right ventricular (RV) function in adults with congenital heart disease (ACHD). Two-dimensional multi-plane echocardiography (2D-MPE) has demonstrated regional wall differences in RV longitudinal strain (LS). This study aims to evaluate the association of these parameters with cardiovascular magnetic resonance (CMR). Methods and results One-hundred stable ACHD patients with primarily affected RVs were included (age 50 ± 5 years; 53% male). Conventional and advanced echocardiographic RV functional parameters were compared with CMR-derived RV function. Advanced echocardiographic RV functional parameters were measurable in approximately one-half of the study cohort, while multi-wall LS assessment feasibility was lower. CMR RV ejection fraction (CMR-RVEF) was moderately correlated with deformational, area, and volumetric parameters [RV global LS (lateral wall and septum), n = 55: r = −0.62, P &amp;lt; 0.001; RV wall average LS, n = 34: r = −0.49, P = 0.002; RV lateral wall LS, n = 56: r = −0.45, P &amp;lt; 0.001; fractional area change, n = 67: r = 0.48, P &amp;lt; 0.001; 3D-RVEF, n = 48: r = 0.40, P = 0.005]. Conventional measurements such as TAPSE and RV S′ correlated poorly. RV global LS best identified CMR-RVEF &amp;lt; 45% (area under the curve: 0.84, P &amp;lt; 0.001: cut-off value −19%: sensitivity 100%, specificity 57%). RVEF and LS values were significantly higher when measured by CMR compared with TTE (mean difference RVEF: 5 [−9 to 18] %; lateral (free) wall LS: −7 [7 to −21] %; RV global LS: −6 [5 to −16] %) while there was no association between respective LS values. Conclusion In ACHD patients, advanced echocardiographic RV functional parameters are moderately correlated with CMR-RVEF, although significant differences exist between indices measurable by both modalities.