Adults With Congenital Heart Disease Patients Research Articles

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<0.001). Also, the F group had significantly higher EAA for mortality predictor Grimage (p<0.001) and PhenoAge (p<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<0.05). The F group has statistically significant higher Grimage and Phenoage EAA than the controls (p<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<0.001), sex (41% vs 28% male, p<0.001), age (34.8 +/-12.5 years vs 44.5 +/- 14.3 years, p<0.001), and education (33% vs 22% high school equivalent or less, p<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.

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). 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 < 0.001; RV wall average LS, n = 34: r = -0.49, P = 0.002; RV lateral wall LS, n = 56: r = -0.45, P < 0.001; fractional area change, n = 67: r = 0.48, P < 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 < 45% (area under the curve: 0.84, P < 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. In ACHD patients, advanced echocardiographic RV functional parameters are moderately correlated with CMR-RVEF, although significant differences exist between indices measurable by both modalities.

Cardiogenic shock in adults with congenital heart disease: Insights from the FRENSHOCK registry

BackgroundData on cardiogenic shock in adults with congenital heart disease (ACHD) are scarce. AimWe sought to describe cardiogenic shock in ACHD patients in a nationwide cardiogenic shock registry. MethodsFrom the multicentric FRENSHOCK registry (772 patients with cardiogenic shock from 49 French centres between April and October 2016), ACHD patients were compared with adults without congenital heart disease (non-ACHD). The primary outcome was defined by all-cause mortality, chronic ventricular assist device or heart transplantation at 1year. ResultsOut of the 772 patients, seven (0.9%) were ACHD, who were younger (median age: 56 vs. 67years), had fewer cardiovascular risk factors, such as hypertension (14.3% vs. 47.5%) and diabetes (14.3% vs. 28.3%), and no previous ischaemic cardiopathy (0 vs. 61.5%). Right heart catheterization (57.1% vs. 15.4%), pacemakers (28.6% vs. 4.6%) and implantable cardioverter-defibrillators (28.6% vs. 4.8%) were indicated more frequently in the management of ACHD patients compared with non-ACHD patients, whereas temporary mechanical circulatory support (0 vs. 18.7%) and invasive mechanical ventilation (14.3% vs. 38.1%) were less likely to be used in ACHD patients. At 1year, the primary outcome occurred in 85.7% (95% confidence interval: 42.1–99.6) ACHD patients and 52.3% (95% confidence interval: 48.7–55.9) non-ACHD patients. Although 1-year mortality was not significantly different between ACHD patients (42.9%) and non-ACHD patients (45.4%), ventricular assist devices and heart transplantation tended to be more frequent in the ACHD group. ConclusionsCardiogenic shock in ACHD patients is rare, accounting for only 0.9% of an unselected cardiogenic shock population. Despite being younger and having fewer co-morbidities, the prognosis of ACHD patients with cardiogenic shock remains severe, and is similar to that of other patients.

Changes in the Cath Lab in the Treatment of Adult Patients with Congenital Heart Disease: A 12-Year Experience in a Single Referral Center with the Establishment of a Dedicated Working Group.

Adults with congenital heart disease (ACHD) are a growing population needing ongoing care. The aim of this study was to investigate if a dedicated ACHD team impacted the timing and indication of invasive cardiology procedures in these patients at our hospital. Our retrospective single-center study enrolled adult patients with moderate or complex congenital heart disease and with at least one cardiac catheterization between January 2010 and December 2021. According to the period, procedures were labeled as group A (2010 to 2015) or group B (2016 to 2021) and further divided into diagnostic (DCC) and interventional cardiac catheterizations (ICC). 594 patients were eligible for the study. Both DCC (p < 0.05) and ICC increased between groups A and B (p < 0.05). In group B: Fontan patients accounted for the majority of DCC (p < 0.001), while DCC decreased in arterial switch repair (p < 0.001). In Fontan patients, conduit stenting was prevalent (p < 0.001), while fenestration closures dropped (p < 0.01). In patients with tetralogy of Fallot and native outflow tract, percutaneous pulmonary valve implantations (PPVI) increased, with a concurrent reduction in pulmonary valve replacements (p < 0.001 vs. surgical series). In right ventricular conduits, ICC increased (p < 0.01), mainly due to PPVI. Among Mustard/Senning patients, baffle stenting increased from Group A to Group B (p < 0.001). In patients with pulmonary atresia and biventricular repair, ICC often increased for pulmonary artery stenting. A dedicated working group could improve ACHD patients' indications for interventional procedures, leading to tailored treatment, better risk stratification and optimizing time until heart transplantation.

Transseptal Puncture Guided by Three-Dimensional Electroanatomical Mapping: Early Experience Using a Simplified Approach in Adults with Congenital Heart Disease.

The widespread use of three-dimensional (3D) mapping systems and echocardiography in the field of cardiac electrophysiology has made it possible to perform transseptal punctures (TSP) with low or no fluoroscopy. However, such attempts in adults with congenital heart disease (ACHD) who have previously undergone surgical or interventional treatment are limited. Therefore, we sought to explore the feasibility and safety of an approach to perform zero- or low-fluoroscopy TSP in ACHD patients undergoing left atrial cardiac ablation procedures. This study included 45 ACHD patients who underwent TSP for ablation of left-sided tachycardias (left atrium or pulmonary venous atrium). Computed tomography (CT) of the heart was performed in all patients prior to ablation. 3D mapping of the right-sided heart chambers before TSP was used to superimpose the registered anatomy, which was subsequently used for the mapping-guided TSP technique. TSP was performed with zero-fluoroscopy in 27 patients, and the remaining 18 patients had a mean fluoroscopy exposure of 315.88 ± 598.43 μGy.m2 and a mean fluoroscopy duration of 1.9 ± 5.4 min. No patient in this cohort experienced TSP-related complications. Our study describes a fluoroscopy-free or low-dose fluoroscopy approach for TSP in ACHD patients undergoing catheter ablation of left-sided tachyarrhythmias who had been previously treated surgically or interventionally due to congenital heart defects. By superimposing 3D electroanatomic mapping with cardiac CT anatomy, this protocol proved to be highly effective, feasible and safe.

Trends in cardiac implantable electronic device utilization in adults with congenital heart disease: a US nationwide analysis.

Adults with congenital heart disease (ACHD) have increased risk of arrhythmias warranting implantation of cardiac implantable electronic devices (CIEDs), which may parallel the observed increase in survival of ACHD patients over the past few decades. We sought to characterize the trends and outcomes of CIED implantation in the inpatient ACHD population across US from 2005 to 2019. A retrospective analysis of the Nationwide Inpatient Sample (NIS) identified 1,599,519 unique inpatient ACHD admissions (stratified as simple (85.1%), moderate (11.5%), and complex (3.4%)) using the International Classification of Diseases 9/10-CM codes. Hospitalizations for CIED implantation (pacemaker, ICD, CRT-p/CRT-d) were identified and the trends analyzed using regression analysis (2-tailed p < 0.05 was considered significant). A significant decrease in the hospitalizations for CIED implantation across the study period [3.3 (2.9-3.8)% in 2005 vs 2.4 (2.1-2.6)% in 2019, p < 0.001] was observed across all types of devices and CHD severities. Pacemaker implantation increased with each age decade, whereas ICD implantation rates decreased over 70years of age. Complex ACHD patients receiving CIED were younger with a lower prevalence of age-related comorbidities, however, had a greater prevalence of atrial/ventricular tachyarrhythmias and complete heart block. The observed inpatient mortality rate was 1.2%. In a nationwide analysis, we report a significant decline in CIED implantation between 2005 and 2019 in ACHD patients. This may either be due to a greater proportion of hospitalizations resulting from other complications of ACHD or reflect a declining need for CIED due to advances in medical/surgical therapies. Future prospective studies are needed to elucidate this trend further.

Very high power- short duration radiofrequency ablation in patients with congenital heart disease and atrial fibrillation: safety and outcome

Abstract Funding Acknowledgements Type of funding sources: None. Background It has been shown recently that ablation of paroxysmal and persistent atrial fibrillation (AF) using very high-power-short duration (vHPSD) ablation protocol is safe and effective. Data for vHPSD ablation protocol in adult patients with congenital heart disease (ACHD) are lacking. We searched our database for ACHD patients undergoing AF ablation using vHPSD ablation regarding periprocedural safety and outcome. Methods The data of 62 consecutive ACHD patients with mild (n=43; 69.4%), moderate (n=15; 24.2%) or complex (n= 4; 6.4%) CHD were analysed (Table 1 ). Mean age was 60,43 ± 12,7 years ( 46% male) with a Cha2ds2-VASc2 Score median of 2. Patients were diagnosed with paroxysmal AF (n= 24; 38%) or persistent AF (n= 38; 61%). In 6 patients (9,6 %), a previous conventional pulmonary vein isolation (PVI) had been performed. Settings used for vHPSD ablation were 70 W/5-7 s or 60 W/7-10 s using irrigated-tip catheters. Circumferential PVI was performed in all 62 patients; in 30 patients (48%) additional substrate ablation was performed. The 30-day incidence of adverse events was analyzed and patients followed-up in regular visits at our outpatient clinic using repetitive 7 days Holter ECGs every 3 months. Results No adverse events such as cardiac tamponade, pericardial effusion &amp;gt;10mm, transient ischemic attack/stroke, atrio-esophageal fistula, cardiac arrest or death occurred. Vascular access complications were detected in 7/62 patients (11%), including 4 patients with a need of intervention (surgical n=2, thrombin injection n=2). Complete PVI was performed in all patients (mean procedure time 129 ± 42min, mean fluoroscopy time 9,09 ± 5.44 min. Radiofrequency (RF) time was 127.27 ± 7,25 min (111,43 ± 27,83 min PVI only, 148,76 ± 46,07 min PVI + substrate ablation). After a mean follow up of 249 days, freedom of any atrial arrhythmia off antiarrhythmic drugs (AAD) after a single ablation procedure was present in 26/47 patients (55.3%). Success rate was significantly higher in patients with paroxysmal AF (n=16/18; 88%) compared to patients with persistent AF (n=10/29; 34.5%; p&amp;lt; 0.001; Figure 1) Conclusion: In this first study using vHPSD in ACHD patients of variable complexity with paroxysmal or persistent AF, the technique was safe and effective. Major periprocedural complications were rare and limited to vascular access problems. No vHPSD ablation modality-related complications occurred. vHPSD was fast in all patintes regardless of CHD complexity. Results in the paroxysmal CHD group were excellent and encouraging for persistent AF.

Telemedicine in Adult Congenital Heart Disease: Usefulness of Digital Health Technology in the Assistance of Critical Patients.

The number of adults with congenital heart disease (ACHD) has progressively increased in recent years to surpass that of children. This population growth has produced a new demand for health care. Moreover, the 2019 coronavirus pandemic has caused significant changes and has underlined the need for an overhaul of healthcare delivery. As a result, telemedicine has emerged as a new strategy to support a patient-based model of specialist care. In this review, we would like to highlight the background knowledge and offer an integrated care strategy for the longitudinal assistance of ACHD patients. In particular, the emphasis is on recognizing these patients as a special population with special requirements in order to deliver effective digital healthcare.

Atrial fibrillation in adult congenital heart disease and the general population

Atrial fibrillation (AF) in adult patients with congenital heart disease (ACHD) may appear early, depending on individual characteristics. The goals of this study were to investigate the epidemiological spectrum of AF in the entire cohort of ACHD and compare it with that in the general population. A retrospective study was performed in the nationwide cohort 2000-2014 with AF onset during 2003-2014. In the cohort of ACHD, 2350 patients had AF; the incidence increased with age, plateauing around age 70. In patients aged 25-29, 45-49, 65-69, 75-79, and ≥80 years, the annual incidence was 1.3, 7.9, 20.6, 23.7, and 21.4/1000 per year, respectively. In the general population without CHD, 347,979 patients had AF; the annual incidence was <1/1000 per year in those aged <55 years but increased steadily with age (3.6, 8.6, and 14.2/1000 per year in aged 65-69, 75-79, and ≥80 years, respectively). Compared with individuals without ACHD, ACHD patients aged <50 years and those aged both 50-54 and 55-59 years exhibited a 20-fold and 10-fold higher incidence of AF, respectively. Patients with complex congenital heart disease and Ebstein's anomaly had the highest risk of AF (cumulative risk >10% by age 50 and >20% by age 60), followed by those with tetralogy of Fallot, tricuspid atresia, endocardial cushion defect, and secundum atrial septal defect (cumulative risk >5% by age 50 and >10% by age60). Compared with individuals without ACHD, AF in patients with ACHD likely appeared 30 years earlier, with a 10- to 20-fold higher incidence plateauing around age 70. Yet, incidence in individuals without ACHD continued to increase. AF burden in patients with ACHD is not expected to increase in a never-ending way.

Preclinical atherosclerotic burden in carotid and lower extremity arteries in adults with congenital heart disease.

Background: Over 90% of patients with congenital heart defects now reach adulthood, due to significant medical advances in recent decades. With advancing age, the risk of acquired cardiovascular diseases increases in addition to the already existing risk due to the congenital defect. The aim of this study was to evaluate the prevalence of atherosclerotic lesions in carotid and lower extremity arteries in adults with congenital heart disease (ACHD). Patients and methods: A total number of 108 ACHD patients (40.6±15.0 years, 50.0% male) and 22 healthy controls (39.3±16.6 years, 40.9% male) were included in this prospective study and underwent a comprehensive angiological examination that included vascular strain analysis on the common carotid artery. Results were stratified by the underlying ACHD lesion groups: shunt lesions (n=26), left-sided obstructive lesions (n=29), right-sided lesions (n=26) and complex lesions (n=27). Results: Colour-coded duplex sonography revealed atherosclerotic lesions in lower extremity arteries in 19 ACHD patients (17.6%). This prevalence did not significantly differ from the one assessed in controls (13.6%, p=0.77). All cases were asymptomatic and therefore classified as Fontaine stage I. 20.4% of ACHD patients presented atherosclerotic lesions in extracranial carotid arteries; amongst controls, the corresponding proportion was 18.4% (p=1.00). No significant differences were observed in atherosclerotic burden in extracranial carotid and lower limb arteries across the four ACHD patient groups (p=0.67 and p=0.89, respectively). Vascular strain analysis revealed no differences between patients and controls. Though circumferential strain varied over ACHD groups (p<0.05), comparison of strain measurements across all specific underlying defect subgroups revealed no significant difference for any of the studied strain parameters. Conclusions: ACHD patients present an atherosclerotic burden in extracranial carotid and lower limb arteries and a vascular stiffness that is comparable to healthy controls.

Cardiogenic shock in adults with congenital heart disease: Insights from the FRENSHOCK registry

Data on cardiogenic shock (CS) in adults with congenital heart disease (ACHD) are scarce. We sought to describe CS in ACHD in a nationwide CS registry. From the multicentric FRENSHOCK registry (n = 772 CS from 49 French centers between April and October 2016), ACHD CS were compared with non-ACHD ones. The primary outcome was defined by all-cause mortality, chronic ventricular assist device (VAD) or heart transplantation at 1-year. Out of the 772 patients, 7 (1%) were ACHD. ACHD patients were younger (54 vs. 66 years old), with less cardiovascular risk factors, such as hypertension (14.2 vs. 47.5%) and diabetes (14.3 vs. 36.1%), and no ischemic cardiopathy (0 vs. 61.5%). Right heart catheterization (57.1 vs. 15.4%), pacemaker (28.6 vs. 4.6%) and ICD (28.6 vs. 4.8%) were more frequently indicated in the ACHD CS management compared to non-ACHD CS, whereas temporary MCS (0 vs. 7.2%) and invasive mechanical ventilation (14.3 vs. 38.1%) were less likely used in ACHD. At 1-year, primary outcome occurred in 85.7% ACHD and 52.2% non-ACHD (P = 0.127) (Fig. 1). Although 1-year mortality was similar (45.4 vs. 42.9% respectively for ACHD and non-ACHD patients, P = 1), VAD (14.3 vs. 5.4%, P = 0.325), and heart transplantation (28.6 vs. 5.2%, P = 0.05) seemed more frequent in ACHD group. CS in ACHD is rare accounting for only 1% of an unselected CS population. Despite a younger age and less comorbidities its prognosis remains severe and similar to others patients.

PO-04-147 LEADLESS PACING IN ADULT CONGENITAL HEART DISEASE PATIENTS: MULTI-CENTRE UK EXPERIENCE

A significant number of Adults with Congenital Heart Disease (ACHD) require cardiac rhythm management devices. Implantation of endocardial or epicardial pacemakers presents specific challenges in this population. The Micra leadless pacemakers have potential to overcome some of these challenges. To assess the utility of Micra implant in ACHD population. A retrospective analyses of Micra implants undertaken at Barts Heart Centre, London and Liverpool Heart and Chest Hospital in the ACHD population between 2020-2022. Data was collected from a prospective registry and electronic health records. Patients were contacted for follow-up data where necessary. 26 AV Micras were implanted across the two centres over two years. 52% females with mean age of 37 yrs. 42% were anticoagulated with warfarin or a DOAC. Underlying etiology – Figure 1. Micra indication – Table 1. Mean procedure and fluoroscopy times were 57±21min and 7±1 min. Access site was femoral in 77% and transjugular in 23%. Mean sensing and thresholds were 11±0.4mV and 0.6±0.09V at 0.24ms respectively. There were no acute procedural complications. Over a mean follow-up of 13 months there were no significant programming issues or change in pacing parameters. One patient had high thresholds and one died of non-cardiac causes. Micra implants should be considered in cases where endocardial or epicardial implants are contraindicated or represent an otherwise suboptimal approach. They have advantages in ACHD patients with complex cardiac anatomy, difficult venous access and need for repeat procedures. Larger multicentre studies and longer follow-ups are required.Tabled 1Table 1Micra Indicationn, %Failed endocardial system6,23Failed epicardial system6,23Bridge to CRTP3,12SVC occlusion3,12Other8,30 Open table in a new tab

Incidence and clinical predictors of early and late complications of implantable cardioverter-defibrillators in adults with congenital heart disease.

The Implantable cardioverter-defibrillator (ICD) has been proven to improve survival in adults with congenital heart disease (ACHD), however it is associated with a high rate of complications. We aimed to quantify the incidence of early (≤3 months) and late (>3 months) complications (EC, LC) in ACHD patients implanted with an ICD and identify their clinical predictors. We retrospectively reviewed 207 patients who had ICD follow-up at Toronto General Hospital between 1996 and 2019. Most common diagnoses were Tetralogy of Fallot (32.4%), dextro-transposition of the great arteries (17.9%), congenital corrected transposition of the great arteries (13%). No intraprocedural complications were observed. Median follow up was 3.4 years (0.1-23). 24 patients (12%) developed EC (4 hematomas, 20 lead dislodgments). A total of 56 LC occurred (46% lead failure, 21% infection, 11% prophylactic lead extraction, 9% neurologic pain, 9% erosion, 4% other) with an incidence rate of LC of 18% per 5 person-years. Anatomic complexity (odds ratio 2.9,p=0.02) and CRTD implant(OR 2.5,p=0.04) were associated with EC. Survival free from LC was 92%, 86%, 65% respectively after 1,5 and 10 years. Presence of legacy leads (hazard ratio 2.9,p=0.006) and subpulmonary ejection fraction (5% increase, HR 1.35,p=0.031) were associated with LC. ACHD patients at risk of SCD continue to benefit from newer device technology. However, these patients-particularly those with greater anatomic and device complexity-remain at increased risk of developing complications over their lifetime. Given the life expectancy of this population, careful consideration needs to be given when a device for primary prevention is being contemplated.

Development of a Novel Society of Thoracic Surgeons Adult Congenital Mortality Risk Model

BackgroundOperative mortality risk models for adults with congenital heart disease (ACHD) undergoing cardiac operations are essential, given the growing population of these patients, yet they are currently unavailable. Existing adult Society of Thoracic Surgeons (STS) models exclude congenital procedures, whereas existing congenital models exclude operations for acquired disease. We aimed to develop an STS mortality risk model for ACHD patients undergoing cardiac operations. MethodsLeveraging a comprehensive list of diagnostic and procedure codes, ACHD patients who underwent cardiac operations were identified from the STS Adult Cardiac Surgery Database (versions: v2.73, v2.81, and v2.9) between 2011 and 2019. The model was developed and validated in the ACHD population using a 60/40 development/validation split. Univariate analyses and clinical expertise informed the addition of ACHD-relevant procedure and diagnosis variables to existing STS adult risk model variables. Model performance was assessed overall and in 38 subgroups based on patient demographics, procedures, and diagnoses. ResultsForty-seven procedure and diagnosis variables relevant to ACHD were added to existing STS adult risk model variables. The derived ACHD model for operative mortality was well calibrated within demographic, procedural, and diagnosis subgroups and the overall ACHD population, and discrimination in the validation cohort was excellent (C statistic, 0.815) compared with the model using only existing STS adult risk model variables (C statistic, 0.79; P < .0001). ConclusionsA novel, high-performing STS ACHD mortality risk model has been developed on the basis of contemporary patient data. The ACHD risk model represents an important expansion of the STS portfolio. Implementation with an online risk calculator is planned.