Adults With Congenital Heart Disease Patients Research Articles

Mortality and survival rate in a scottish cohort of adults with congenital heart disease

Abstract Background Adult patients with congenital heart disease (ACHD) experience higher rates of morbidity and reduced long-term survival in comparison to the general population. The care of this cohort of patients requires specialist expertise which is mainly delivered at tertiary centers. Aims We aim to report the clinical characteristics, mortality rates and causes of death in a contemporary cohort of ACHD patients followed up in a North- East Scottish Health Board. Methods We prospectively followed up ACHD patients aged ≥17 years (n=645) under review at the Regional NHS ACHD Clinic in a North- East Scottish Health Board between the years 2020 and 2023. We recorded the number and the causes of death as documented on our electronic records and on correspondence with primary care physicians. Survival analysis was performed according to the 2020 ESC Guidelines classification of congenital heart disease complexity (Table 4) classes using Kaplan-Meier method with log-rank (Mantel-Cox) test done in IBM SPSS v.24. Results Our ACHD cohort (n=645, 54% females and 46% males) included patients of all three ESC complexity classes (30% mild, 60% moderate and 10% severe). Between 2020 and 2023, 18 patients (2.8%) died, with a total incidence of 0.93 deaths/100 patient years. Median age of death was 59 years (IQR 45–71), and the majority of deceased patients were males (56%). There were 2 deaths in the mild complexity group, 11 deaths in the moderate complexity group and 5 deaths in the severe complexity group (See Table 1). This was found to correspond to an incidence of 0.34, 0.95 and 2.64 deaths/100 patient years respectively. The leading causes of death were heart failure (28%) and malignancy (22%). Using Kaplan-Meier survival analysis we found that the survival distributions for the mild, moderate and severe ACHD complexity groups were statistically significantly different by log-rank test (χ2(2) = 11.47, p < .005) (See Figure 1). Patients with moderate ACHD complexity had a median survival age 67 years (95% CI 58.1-75.9), patients with severe ACHD had median survival age 57 years (95% CI 44.7-69.2). Median survival could not be calculated for the mild group due to lack of data points. Conclusion The ACHD patient group is characterised for a variety of pathophysiology and clinical presentations. They require lifelong medical input and in spite of the local and national expertise, the mortality and morbidity rates are still high. Heart failure was the leading cause of death in this cohort which is similar to previous reported data. Mortality rates were proportional to the complexity of the underlying ACHD diagnosis. The survival rate of patients in the severe complexity group was ten years less than the moderate complex group. ACHD patients in the moderate and severe complexity groups have significantly reduced life expectancy and require access and clinical expertise from National ACHD Specialty Centers.

Short-term mechanical circulatory support in adults with congenital heart disease: indications and outcomes

Abstract Background An increasing number of adults with congenital heart disease (ACHD) present with heart failure (HF), which may require short- or long-term mechanical circulatory support (MCS), often around cardiac surgery or other interventions. Even though MCS has entered ACHD practice over the last few years, little is known on its indications and outcomes in this population. Purpose To assess the use and outcomes of short-term MCS in ACHD patients in a specialist centre, and assess the predictive power of the Survival After Veno-Arterial Extracorporeal Membrane Oxygenation (SAVE) score in this population. Methods Data on all ACHD patients who received MCS since 2008 in a single tertiary centre were collected. Immediate, short and longer-term outcomes were also collected, including death, complications, and weaning success. Logistic regression and ROC analysis were performed to identify predictors of in-hospital mortality. Result There were 65 episodes of MCS in 63 patients during the study period: age 36.9+-13.6 years, 61.5% male. Most patients had CHD of moderate or severe complexity (49.2% moderate and 10.8% severe according to ESC guidelines). The most common anatomical diagnoses were left outflow tract obstruction in 35.4%, Marfan syndrome in 20.0% and tetralogy of Fallot or pulmonary atresia in 10.8%. The most common indications for ECMO were perioperative support (not endocarditis) in 55.6%, HF in 14.3%, pneumonia in 12.7% and endocarditis-related surgery in 7.9%. A critical preoperative status was present in 26.8%; median PEACH score (a validated ACHD peri-operative risk score) was 1.0[1.0-3.0] and ACHS score was 0.6[0.6-1.0]. Median SAVE score was -4.0[-9.0–2.0], with 21(43.8%) in SAVE class 4 or 5. VA-ECMO was used in 69.2%, VV-ECMO in 21.5%, BiVAD-ECMO in 4.6% and RVAD support in 4.6% of patients. The median duration of ECMO support was 198.6[133.0-354.2] hours. Complications included ischemic stroke (15.4%), intracerebral bleeding (9.2%), and other thromboembolism (23.8%). Successful weaning was achieved in 56.2%, whereas 4.6% were switched to more definitive MCS. In-hospital mortality was 47.7%, and this was higher in patients receiving VA support (OR 4.92, 95%CI:1.32-23.93, p=0.03). For patients on VA-ECMO or BiVAD support, the presence of Marfan syndrome (OR 4.92, 95%CI:1.32-23.93, p=0.03) and a lower (worse) SAVE score (OR 1.3 per point reduction, 95%CI:1.11-1.59, p=0.004) were associated with in-hospital mortality. An adjusted SAVE score that accounted for the presence of heritable thoracic aortic diseases (HTAD, -3 points) performed well at predicting in-hospital mortality (AUC increasing from 0.73 to 0.8, Figure). Conclusions Short-term MCS is a useful temporary rescue intervention in patients with ACHD, as a bridge to recovery or durable support, with 1 in 2 patients surviving to hospital discharge. The SAVE score adjusted for HTAD can reliably predict outcomes in patients with VA-ECMO and requires external validation.Figure

Cardio-pulmonary exercise testing in adult congenital heart patients: a new data-focused approach to predict mortality

Abstract Background/Introduction The cardio-pulmonary exercise test (CPET) has been a pivotal tool for functional and prognostic evaluation in adults with congenital heart disease (ACHD). During a routine CPET test, a myriad of variables are collected reflecting the cardiovascular, pulmonary, and skeletal muscle systems. However, clinicians typically use only a few easily and accessible variables to establish prognosis, which can come at an accuracy cost. Machine learning algorithms have undergone significant development in recent years and have found applications in the medical field. One of their main advantages is the ability to handle a large number of variables and extract information from their various combinations, while excluding redundant data, thus offering a higher level of prediction accuracy. Purpose Our objective was to develop a machine learning model to predict death during follow-up in a large population of ACHD patients who have undergone routine CPET in a large-volume single specialist centre. Methods All available CPET studies for adult ACHD patients (age > 15 years) performed from December 1999 to December 2021 at our centre were included, and all standard available variables were extracted. The primary outcome was all-cause mortality since the CPET, collected up to November 2023. Data exploration, data cleaning, and feature engineering steps were conducted using Python (v3.8). Continuous variables were standardised. The supervised machine learning algorithm XGBoost was used for classification (all-cause mortality), with the best hyperparameters selected through cross-validation. For the final model, a feature importance analysis based on permutation techniques was used to explore the variables with more impact on model accuracy. Results A total of 6361 studies were included, with median age of 31 years (IQR 23-43), 56% male. During follow-up, a total of 491 deaths were recorded. Of available demographic, clinical and exercise parameters (n=129 variables), 21 were deemed sufficient in terms of completeness, absence of significant correlation and clinically relevant for the subsequent analysis. These included demographic variables (age and sex) and CPET variables (peak VO2, FEV1, peak heart rate, exercise time, etc.) and were used to train the model. The accuracy of the model in predicting death after CPET in the test set had an accuracy of 93.5% and an area under the ROC curve (AUC) of 85.0% (Figure1). The parameters with the highest relative feature importance were FEV1, body mass index and percent predicted peak VO2 (Figure 2). Conclusions Machine learning models can be effectively employed in specialised cardiac populations, such as ACHD, potentially providing a high level of prediction accuracy by efficiently harnessing the potential of available data. However, its applicability to mortality prediction for ACHD patients requires further external validation.ROC curve AUC for the model predictionsFeature importance using permutation

Exploring frailty: muscle strength, functional capacity, activities of daily living and cognition in adult congenital heart disease

Purpose The study aimed to assess frailty in adults with congenital heart disease (ACHD) and to compare muscle strength, functional capacity, activities of daily living (ADL), and cognition between frail and non-frail ACHD patients. Materials and methods A cross-sectional study design was used. Sixty people with ACHD aged between 18 and 45 years were included. Frailty was determined according to the Fried criteria. Peripheral muscle strength was assessed with a digital dynamometer, functional capacity with the 6-min walk test (6MWT), ADL with the Glittre ADL test, and cognition with the Montreal Cognitive Assessment (MoCA) test. Results Frailty was seen in 38.33% (frail = 23 and non-frail = 37) of the participants. In the frail patients, dominant knee extensor strength (p = 0.002), shoulder abductor strength (p = 0.005), 6MWT distance (p = 0.021), and MoCA score (p = 0.005) were significantly lower than those in the non-frail patients. Glittre ADL test (p = 0.002) was significantly higher in the frail patients. Conclusions Muscle strength, functional capacity, ADL, functional mobility, and cognition were lower in the frail participants with ACHD. Early assessment of frailty in ACHD and planning individualized exercise training programs for frail individuals may be a strategy to reduce the impact of frailty on adverse clinical outcomes.

Impact of an Adult Congenital Heart Disease Order Set in a Pediatric Cardiac Intensive Care Unit.

Background: Patients with congenital heart disease (CHD) are increasingly surviving to adulthood. Given their age- and condition-specific needs, the optimal postoperative setting for these patients is not yet determined. This study aims to evaluate the utility of an adult-specific order set in improving clinical outcomes for these patients in a pediatric cardiovascular intensive care unit (ICU). Methods: Adults with CHD (ACHD) admitted to the cardiovascular ICU following cardiac surgery at a pediatric tertiary care center were identified for a retrospective cohort study. Health care delivery metrics and clinical outcomes of participants who received the adult-specific order set were compared with control patients admitted prior to the intervention. Categorical outcomes were compared using the χ2 test of independence or Fisher exact test, and continuous outcomes were assessed using the t test. Results: A total of 130 ACHD patients received the intervention, with no significant differences observed in clinical outcomes when compared with 89 controls. While there was additionally no reduction in hospital-related mortality (2.3% vs 7.8%, RR 0.3; P = .1), clinician ordering behaviors were better aligned with best-practices following the intervention. Among patients with moderate or greater anatomic complexity, a post hoc analysis demonstrated reduced in-hospital mortality from those who received the adult order set. Conclusions: The implementation of an adult-specific order set in a pediatric care setting did not improve clinical outcomes for all ACHD patients. Patients with moderate and greater anatomic complexity did see a mortality benefit, suggestive that targeted electronic tools may most benefit those who have the highest risk.

Association of reduced peak left atrial strain with supraventricular arrhythmia in adults with congenital heart disease.

Atrial arrhythmias are an important cause of morbidity and mortality in adults with congenital heart disease (ACHD). In acquired heart disease, the left atrial (LA) strain has been shown to predict supraventricular tachyarrhythmias (SVT). This study aimed to investigate whether reduced LA strain is associated with SVT in ACHD patients. This retrospective, single-center cohort study collected baseline clinical and echocardiographic data of 206 ACHD patients (157 left heart defect, 49 right heart defect). Patients with sinus rhythm at baseline and a 5-year follow-up (median age 29, IQR 22-41 years) were included. Diagnosis of sustained SVT was determined from clinical reports during the follow-up period. New or recurrent sustained SVT occurred in 16 patients (7.8%, median follow-up of 6.2 years). Patients who developed SVT were older, more likely to have diastolic dysfunction, and had larger LA dimensions, left ventricular mass, and a lower peak LA longitudinal strain (PALS). Lower PALS was associated with higher risk of SVT in patients with left and right heart defects. Patients in the lowest quartile for PALS had a 15.9-fold higher hazard ratio of SVT (95% confidence interval, 4.5 to 56.0, p < 0.001) in comparison with the top three quartiles. PALS provides information about the occurrence of SVT in the ACHD population. Including measurement of LA strain in the follow-up of these patients may allow to better identify patients at risk of future atrial arrhythmias.

Mechanical circulatory support devices in adults with congenital heart disease.

Mechanical circulatory support is used frequently as a method of stabilizing patients with end stage heart failure who are unable to safely await allograft availability. While this technology has been fundamentally important in supporting patients with normal cardiac anatomy, it is still used infrequently in adult patients with congenital heart disease and end-stage heart failure. Here, we review the data on mechanical circulatory support technology in this small but growing population of patients with chronic heart disease prone to the development of circulatory failure. Mechanical circulatory support (MCS) has been increasingly employed in adults with congenital heart disease (ACHD) as a bridge to transplant. The new United Network for Organ Sharing listing system favoring temporary MCS use with a higher listing status offers another tool to stabilize ACHD patients and potentially shorten wait times. Both temporary and Durable MCS could help improve transplant candidacy and posttransplant outcomes in select groups of ACHD patients. Durable and temporary MCS have the potential to significantly improve access to transplant and overall transplant outcomes in ACHD patients.

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.

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

BackgroundLimited data exist regarding outcomes of delayed sternal closure (DSC) in adults with congenital heart disease (ACHD). MethodsWe reviewed 159 ACHD patients undergoing cardiac operation from 1993 to 2023 who required DSC (open sternum at the end of operation, n = 112) or sternum emergently reopened (n = 47). Regression models were performed to determine factors associated with outcomes. ResultsOf 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 days). Apart from sex, 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 the incidence of early complications, except sternal infection. Risk factors associated with early mortality were age (P = .02), DSC >4 days (P < .001), hemodynamic indication (P = .03), and single ventricle (P = .02). On multivariable analysis, lower ejection fraction (P = .04), hemodynamic indication (P = .02), single ventricle (P = .004), and diabetes mellitus (P = .03) were predictors of prolonged time to chest closure. Among hospital survivors, late survival was similar between patients undergoing DSC ≤4 days vs >4 days (P = .48). ConclusionsA 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.

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.

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.

Pulmonary valve replacement-A 10-year single-center surgical experience in ACHD patients.

Large-scale analyses of surgical outcomes after surgical pulmonary valve replacement (sPVR) as part of re-do surgery in adults with congenital heart disease (ACHD) are rare. Therefore, we present our outcomes of sPVR in ACHD patients over the last decade and demonstrate our standardized surgical approach. All ACHD patients who underwent sPVR between January 2013 and August 2022 were included. Primary diagnoses, peri-operative data, post-operative echocardiography, pre- and post-operative RV MRI and in-hospital mortality were examined. Pre- and postoperative MRI parameters were compared using paired testing. Standardized surgery was documented. Normality of continuous variables was tested using Shapiro-Wilk test. 79 patients (male 59.5% (n = 47), 71 re-operations (89.9%)) at a median age of 41.7 (52.2-28.8) years were included. Main underlying disease was Tetralogy of Fallot (TOF; n = 47, 59.5%). After removal of degenerated valve/conduit parts, right ventricular outflow tract (RVOT) patch augmentation and implantation of a larger stented bioprosthesis (25mm in 78.5%) were conducted. In 57% of cases, concomitant surgery was performed (mainly tricuspid valve surgery: n = 28, 35.4%). 25 patients (31.6%) were operated with beating heart technique. Echocardiographic outcomes showed no moderate or severe insufficiency (median Vmax of 2 m/s (2.3-1.77 m/s)) upon discharge. Available MRI data showed significantly lower indexed RV-EDV (p = 0.0006) and RV-ESV (P = 0.0017) after surgery. In-hospital mortality was 5.1% (n = 4). SPVR is a safe therapeutic option with low surgical risk and satisfying post-operative results. It can serve as a solid therapeutic option for patients who need future valve-in-valve interventions.

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.