Adult Patients With Congenital Heart Disease Patients Research Articles

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.

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.

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.

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.

SCUBA Diving in Adult Congenital Heart Disease.

Conventionally, scuba diving has been discouraged for adult patients with congenital heart disease (ACHD). This restrictive sports advice is based on expert opinion in the absence of high-quality diving-specific studies. However, as survival and quality of life in congenital heart disease (CHD) patients have dramatically improved in the last decades, a critical appraisal whether such restrictive sports advice is still applicable is warranted. In this review, the cardiovascular effects of diving are described and a framework for the work-up for ACHD patients wishing to engage in scuba diving is provided. In addition, diving recommendations for specific CHD diagnostic groups are proposed.

Patients with congenital heart disease over the age of 60: experiences of a single tertiary centre

Abstract Background We are currently facing a shift in adult patients with congenital heart disease (ACHD) to older and more complex patients. The growing population of senior patients with congenital heart disease (CHD) is prone to acquired comorbidities. Purpose Purpose of our study was to identify the main comorbidities in ACHD patients ≥60 years. Methods 2726 patients had at least one visit at our ACHD centre between 2010 and 2021, 287 patients of them being 60 or older. Clinical data about the patients' last visit were obtained retrospectively from digital medical records. Based on the complexity of the CHD patients were classified as mild, moderate, and severe. Survival difference was analysed using the Kaplan-Meier method and the log rank test. Results Senior patients included in this study were predominantly female (73.2%) with the median age of 69 years. We included 287 patients with mild (47.4%), moderate (39.4%), and severe (13.2%) underlying heart disease. Median age at first visit in our centre was 60 years (IQR 54–67). Over the median follow-up time of 7.9 years (IQR: 3.2–13.2), 20 patients (7%) died with the incidence of 0.8 deaths/100 patient years. The most common complication in this populations were Heart failure (43.2%) and arrhythmia (12.5%). Meanwhile the highest prevalence of heart failure was seen in patients with Eisenmenger syndrome/Pulmonary Arterial Hypertension (84.8%), Tetralogy of Fallot patients had the highest prevalence of arrhythmias (77.8%). 51% of all patients had at least one cardiovascular risk factor (diabetes, hypertension, hyperlipidaemia, or chronic kidney disease). 42% of the patients underwent a non-coronary intervention over the age of 60. The highest mortality was seen in patients with severely complex lesions (p&amp;lt;0.002). As of the comorbidities, significantly higher mortality was observed in seniors with heart failure (p=0.001) or NYHA functional class III/IV (p&amp;lt;0.002). Conclusions Although the number of ACHD patients is growing constantly, the population of elderly patients with CHD is still mainly consists of patients with mild or moderate heart defects. Half of the patients had at least one cardiovascular risk factor by the age of 60. As expected, a significantly higher mortality was seen in those with severe heart defects. Funding Acknowledgement Type of funding sources: None.

Treatment of heart failure in adult congenital heart disease

The progressive shifting in adult patients with congenital heart disease (ACHD) epidemiology with aging and superimposed acquired cardiac pathology on top of complex congenital heart defects is leading to an increase of hospitalizations for heart failure (HF), which nowadays represents one of the main causes of death in this patient population. Although there is a theoretical evidence to support the use of conventional drugs indicated for the general population with HF, randomized controlled trials do often exclude ACHD patients. Anatomical and physiological heterogeneity makes it difficult to define the role of cardiac resynchronization, and indications are less established. Timing of assessment for heart transplantation is challenging and referral often occurs too late. The present review wants to offer a summary of current therapeutic strategies and discuss future perspectives for ACHD-related HF treatment.

Heart failure hospitalization in adult patients with congenital heart disease: risk factors for repeated admissions

Abstract Background Heart failure hospitalizations in adult patients with congenital heart disease (ACHD) are increasing and are associated with higher healthcare-related costs. We aimed to evaluate factors that are associated with repeated heart failure hospitalizations and whether heart failure hospitalizations are related to adverse outcome in ACHD patients with heart failure (ACHD-HF). Methods Out of 3995 patients under active follow-up in our institution (last visit &amp;gt;2010), 256 patients (mean age 49.5±16.7 years) had ACHD-HF and were included in the study. Medical records were reviewed, including heart failure hospitalization prior and after study inclusion. A combined endpoint of death, ventricular assist device and transplantation was defined. Results Overall, 136 ACHD-HF patients (53%) had a prior heart failure hospitalization. Over a mean follow-up of 2.5±2.3 years, 44 patients (17%) had repeated heart failure hospitalizations. Of these, 31 patients (12%) had 1; 9 patients (4%) 2 and 4 patients (2%) 3 repeated heart failure hospitalizations. Patients with repeated heart failure hospitalizations had higher NYHA class (p=0.031), were more likely to have end-organ dysfunction (p=0.025) and more likely to have a prior heart failure hospitalization (p&amp;lt;0.001). In multivariable Cox regression analysis, only end-organ dysfunction (HR 2.431 95% CI 1.516–43.896 p&amp;lt;0.001) was related to repeated hospitalization. Seventy patients (27%) reached the combined endpoint of death, VAD or transplantation (event-rate 11% per year). Repeated heart failure hospitalizations was not related to the combined endpoint in Cox regression analysis. Conclusions Heart failure hospitalizations are frequent in ACHD-HF. End-organ dysfunction appears to be a strong determinant of repeated heart failure hospitalizations. Event-rate is high in patients with ACHD-HF, but repeated heart failure hospitalizations were not related to outcome in this short-term follow-up study. Funding Acknowledgement Type of funding sources: None. Table 1.1Table 1.2

Treatment of symptomatic atrial tachycardia in complex anatomic conditions: use and outcome of ablation in patients after damus-kaye-stansel surgery

Abstract Funding Acknowledgements Type of funding sources: None. Background Atrial tachycardias (AT) are amongst the most frequent complications in adult patients with congenital heart disease (ACHD). Their incidence mainly depends on age and complexity of underlying defect. AT has a marked impact on morbidity and mortality and is associated with sudden cardiac death particularly in complex ACHD patients. The arrhythmic substrate can be variable from re-entry to multifocal resulting in demanding requirements for electrophysiologic ablation procedures (EP) and thus, relatively high rates of recurrence. Methods and results Retrospective analysis of ACHD patients with prior Damus-Kaye-Stansel (DKS) surgery treated at one of the largest specialized tertiary centers for ACHD patients worldwide. Analysis focussed on occurrence of AT, respective treatment and outcome of EP. Over a median observation time of 15 years, 13 patients with prior DKS surgery and complex congenital heart defects of different types but uniformly Fontan circulation, were identified (median age 22.0 years (IQR 20.5 – 25.5), 76.9% male, 92.3% NYHA Class I, median systemic ventricular ejection fraction 58.5%, 69.2% extracardiac tunnel). Seven patients (53.8%) presented with symptomatic AT (median cyclic length 440 ms (IQR 360.0 – 517.5)). Of these, six patients underwent EP. Median procedure time was 180.5 min (IQR 160.8 – 274.5), median fluoroscopy time 1.56 min (IQR 0.51 – 4.73) and radiofrequency time 851 sec (265.0 -2005.0). Remote magnetic navigation was used to access ablation site through a retrograde access via the aortic as well as the neo-aortic valve. Three-dimensional image integration aided in mapping and ablation. Throughout the procedure as well as in follow-up visits, no reportable complications were observed. After a mean of 1.5 procedures, all analysed patients stayed complaint-free throughout a median time-interval of 13 months. Conclusion AT are frequent in complex univentricular situations after Damus-Kaye-Stansel surgery (53.8%). Although more than one procedure (mean 1.5) was necessary to achieve satisfactory results even in experienced hands, EP appears to be an appropriate treatment strategy in this young and otherwise healthy patient cohort. According to our analysis, EP is feasible and safe in centers with knowledge of specific anatomic characteristics of congenital heart disease and when the appropriate technical infrastructure is present. Access to ablation site was performed by remote magnetic navigation in the majority of procedures. The floppy catheter was navigated through the neo-aortic as well as through the aortic valve without observable peri- or postinterventional complications. Mapping with three-dimensional image integration facilitated EP and led to a relatively low radiation exposure with the known benefits for the patient as well as for the healthcare professionals involved in the procedure. Abstract Figure.

COVID-19 in adults with congenital heart disease: early morbidity and mortality in two northern Italian specialist centers

There is no information on coronavirus disease 2019 (COVID-19)-related outcomes for adult patients with congenital heart disease (ACHD). We aim to assess the clinical burden of COVID-19 on ACHD patients during the initial stages of the pandemic in Europe. ACHD patients followed in 2 tertiary centers in the Lombardy region were screened for COVID-19-related symptoms and complications. 661 patients (52.3% male, mean age 34.9±11.7 years) with CHD of moderate (58.5%) or high (32.8%) complexity were contacted. Of these, 145 (21.9%) reported typical COVID-19 symptoms, but only 4.8% required hospitalization. We did not record confirmed COVID-19-related deaths. Patients hospitalized were elderly (P=0.03), more likely to be receiving diuretics (P=0.03) or beta-blockers (P=0.02), and have been admitted over the previous year (P=0.003). Of the 7 patients hospitalized, 2 had a systemic RV and 2 a Fontan circulation. COVID-19-related morbidity and mortality have been low in ACHD patients 6 weeks from the start of the outbreak, in one of the worse hit European regions.

Invasive electrophysiological studies in adult congenital heart disease- evolving use and impact of specialized centre therapy on recurrence of arrhythmia: a nationwird analysis based on 2.433 cases

Abstract Background Arrhythmias are a major contributor to morbidity and mortality in adult patients with congenital heart disease (ACHD). Advances in ablation technology have contributed to improved management and reduction of symptoms in this population. However, only limited data exist on the use and outcome of these technologies in large community-based cohorts of ACHD patients and on the impact of specialized centres on recurrence of arrhythmias. Purpose We performed a retrospective analysis based on data from one of the largest German Health Insurance Companies (approx. 9 million insured members), acquiring real-world data on frequency of invasive electrophysiological studies (EPS) in Germany between 2005 and 2018. ACHD patients were identified based on ICD codes and the spectrum of disease as well as the impact of EPS being performed at specialized ACHD centres was analysed. Results Out of 45,761 eligible ACHD patients in the database, we identified 2,433 EPS performed in 1,706 ACHD (51% female, median age 55.4 years, complexity of CHD mild, moderate and severe in 50.6%, 33.2%,16.2%, respectively). Over the study period the annual number of EPS increased by 207%. The majority of procedures were for supraventricular tachycardias (85.9%). Of these procedures atrial fibrillation accounted for 35.1%, atrial flutter for 29.4% and other supraventricular tachycardias/pre-excitation syndromes for 29.9% of cases. The majority of EPS (64.3%) was performed at non-specialized centres including 40.2% of cases in patients with complex disease. Overall, the re- intervention rate within 12 months of the primary EPS was 14.5%. Whereas in ACHD patients with simple and moderate complexity disease no obvious difference in 12-months re- intervention rate was observed between specialized and non-specialised centres (15.5% vs. 15.0%), in patients with complex disease the reintervention rate was 41.6% higher for non-specialized centres (13.6% vs. 9.6%). Conclusion Our large real- world analysis shows an increasing need for invasive electrophysiological studies in ACHD patients. The vast majority of procedures was performed for supraventricular arrhythmias including in atrial fibrillation and flutter ablation. Re-interventions were frequently required in this unique and anatomically challenging population. Alarmingly, many procedures were performed at non-specialized centres, although current guidelines recommend that interventions in complex patients should remain reserved for experienced high-volume centres. Funding Acknowledgement Type of funding source: None

Maternal and neonatal complications in women with congenital heart disease: results from a nationwide analysis including 7,231 pregnancies

Abstract Background The number of adult patients with congenital heart disease (ACHD) is increasing. Many ACHD women aim to start a family and require adequate pre-pregnancy counselling and pregnancy care. Limited population-based data on maternal and neonatal outcome and complications are available. Purpose We analysed the association between ACHD and maternal or neonatal outcomes based on data from one of the largest German Health Insurance Companies (approx. 9 million members). All pregnancies in women with ACHD and an age-matched non-congenital control group were included. The data covered all pregnancies and deliveries (in and out of hospital) between 2005 and 2018. Results The study population comprised 25,528 ACHD patients of childbearing age. Overall, 7,231 pregnancies occurred in 3,868 ACHD women (mean age at delivery 30.7±5.4 years). An age matched group of 6,513 women without ACHD (11,247 pregnancies, mean age 30.0±5.6 years) served as controls. Miscarriages/abortions occurred in 2,501 (34.6%) and 3,586 (31.9%) cases in the ACHD and non-ACHD cohort, respectively. Overall, there were 4,551 successful deliveries (99.5% in hospital) and 59 stillbirths in ACHD patients. Cesarean deliveries (CS) were significantly more common in ACHD patients (40.4% vs. 31.5% in the control group; p&amp;lt;0.0001) and the CS rate increased with disease complexity (Bethesda class I/II/III: 39.2/42.3/44.4%; p=0.035). There was one successful maternal resuscitation in each group, but no maternal deaths during pregnancy and up to 90 days postpartum. ACHD patients had a higher rate of maternal cerebral complications (1.0% vs. 0.17%; p&amp;gt;0.0001) compared to the control group. Neonatal mortality was low but significantly higher in the ACHD group (0.76% vs. 0.22% within the first 30 days of life; p&amp;lt;0.0001). Neonates to ACHD mothers were also significantly more often afflicted by low or extremely low birth weight or extreme immaturity (p&amp;lt;0.0001), and required resuscitation (1.04% vs. 0.34%; p&amp;lt;0.0001), mechanical ventilation (8.7% vs. 3.5%; p&amp;lt;0.0001) or blood transfusions (3.11% vs. 1.08%; p&amp;lt;0.0001) significantly more often compared to offspring from non-ACHD mothers. In addition, recurrence of congenital heart disease was 6.1 times higher in infants to ACHD mothers compared to controls (p&amp;lt;0.0001). Overall, 5.87% of all children born to ACHD mothers required cardiac surgical procedures in the first 6 years of life compared to only 0.39% of children to non-ACHD mothers (p&amp;lt;0.0001). Conclusion Our analysis confirms that maternal morbidity and neonatal morbidity and mortality are significantly increased in women with ACHD and their offspring compared to non-ACHD controls. Although, maternal mortality appears limited in high resource health care systems, the elevated maternal complication rate and particularly the high mortality and complication rate in neonates justifies specialized care to address specific risks and requirements of the condition. Funding Acknowledgement Type of funding source: None

Amiodarone-associated thyroid dysfunction in adult patients with congenital heart disease: a nationwide health insurance data analysis including &amp;gt;50.000 individuals

Abstract Background Arrhythmias are common in adult patients with congenital heart disease (ACHD). Amiodarone is widely used as an antiarrhythmic agent. Thyroid dysfunction represents a serious complication of amiodarone treatment. Systematic data on the prevalence of thyroid dysfunction, risk factors for complications and treatment options are lacking. Purpose Based on data from one of the largest German Health Insurance Companies (BARMER GEK, approx. 9 million members), we performed a retrospective analysis investigating the rate of thyroid complications under active amiodarone therapy in ACHD patients and a comparison group of cardiac patients without congenital heart disease on amiodarone between 2005 and 2018. Result Overall, 910 ACHD (34% female; median age 66 y; CHD complexity mild, moderate, severe in 64.6%, 23.6%,11.8%, respectively) and 49,782 non-ACHD patients (37% female, median age 73.4 y) received prescriptions for amiodarone without documented pre-existing thyroid disease or use of thyroid medication. Over a treatment period of 184,770 patient-years, 10,874 incidents of thyroid dysfunction occurred in the non-ACHD and 201 in the ACHD cohort, corresponding to an event-rate of 6% and 5.3% per patient year, respectively. Overall, 23.5% of the ACHD patients developed thyroid dysfunction (56.71% hypothyroidism, 43.3% hyperthyroidism). Risk factors for developing thyroid dysfunction on time dependent Cox-analysis were female gender (hazard ratio [HR] 1.44, 95% CI: 1.39–1.50; p&amp;lt;0.001), lower patient age (HR: 0.96 per 10 years, 95% CI: 0.94–0.98, p&amp;lt;0.001) renal dysfunction (HR 1.24, 95% CI: 1.19–1.29, p&amp;lt;0.001), history of alcohol abuse (HR 1.17, 95% CI: 1.07–1.27, p,0.001) and smoking (HR 1.12, 95% CI: 1.06–1.19, p&amp;lt;0.001). Congenital heart disease itself was not associated with a higher risk of thyroid dysfunction (HR 0.96, 95% CI: 0.83–1.10, p=0.53). Within the ACHD group, patients with complex disease had a significantly higher risk of thyroid dysfunction (HR 1.5, 95% CI: 1.00–2.25, p=0.049) compared to patients with simple diagnoses. Once thyroid disease occurred, 48.5% of patients were continued on amiodarone therapy, 12.8% of patients underwent an electrophysiologic procedure and only 2.1% of patients received class I antiarrhythmics. Specific thyroid therapy included thyroxine (62.6% of hypothyroid patients) or thiamazole (22.6% of hyperthyroid patients). Only 2.3% of patients required surgery or radiotherapy within 6 months after thyroid dysfunction. Conclusion Amiodarone-associated thyroid dysfunction is a frequent complication in ACHD patients. Overall, one in 4 ACHD patients on amiodarone developed thyroid dysfunction in our study. In itself, ACHD does not seem to increase the risk of thyroid dysfunction. However, female gender, complexity of disease, younger age and renal dysfunction emerged as independent risk factors. When amiodarone therapy can not be avoided, close follow-up and regular thyroid function tests are recommended. Funding Acknowledgement Type of funding source: None

Adult Congenital Heart Transplantation: Learning to Surf This Growing Wave?

Adult patients with congenital heart disease (ACHD) undergoing transplantation are a high-risk population. Based on current estimates, nearly 90% of children with repaired CHD survive into adulthood, approximately at 1 million.1 According to the most recent International Society of Heart and Lung Transplantation report, 3.3% of adult heart transplantations are done in CHD patients, representing a growth of over 40% in the past 2 decades.2,3 In this issue of The Annals of Thoracic Surgery, the current report by Kainuma and colleagues4 tries to study the differences between ACHD and non-ACHD patients with previous sternotomies undergoing heart transplantation.

Risk prediction models for heart failure admissions in adults with congenital heart disease

BackgroundHeart failure (HF) is the leading cause of death in adult patients with congenital heart disease (ACHD). No risk prediction model exists for HF hospitalization (HFH) for ACHD patients. We aimed to develop a clinically relevant one-year risk prediction system to identify ACHD patients at high risk for HFH. MethodsData source was the Quebec CHD Database. A retrospective cohort including all ACHD patients aged 18–64 (1995–2010) was constructed for assessing the cumulative risk of HFH adjusting for competing risk of death. To identify one-year predictors of incident HFH, multivariable logistic regressions were employed to a nested case-control sample of all ACHD patients aged 18–64 in 2009. The final model was used to create a risk score system based on adjusted odds ratios. ResultsThe cohort included 29,991 ACHD patients followed for 648,457 person-years. The cumulative HFH risk by age 65 was 12.58%. The case-control sample comprised 26,420 subjects, of whom 189 had HFHs. Significant one-year predictors were age ≥ 50, male sex, CHD lesion severity, recent 12-month HFH history, pulmonary arterial hypertension, chronic kidney disease, coronary artery disease, systemic arterial hypertension, and diabetes mellitus. The created risk score ranged from 0 to 19. The corresponding HFH risk rose rapidly beyond a score of 8. The risk scoring system demonstrated excellent prediction performance. ConclusionsOne eighth of ACHD population experienced HFH before age 65. Age, sex, CHD lesion severity, recent 12-month HFH history, and comorbidities constructed a risk prediction model that successfully identified patients at high risk for HFH.

Inspiratory muscle dysfunction and restrictive lung function impairment in congenital heart disease: Association with immune inflammatory response and exercise intolerance

BackgroundIn adult patients with congenital heart disease (ACHD), both underlying disease and lung restriction contribute to exercise intolerance. In ACHD the yet incompletely understood mechanism underlying restricted ventilation may be inspiratory muscle weakness. Therefore, this study comprehensively evaluated inspiratory muscle function in ACHD and associations with systemic inflammation and the clinical severity of exercise intolerance. Methods30 ACHD patients (21 men, 35 ± 12 years) and 30 healthy controls matched for age, gender and body mass index underwent spirometry, measurement of mouth occlusion pressures, and diaphragm ultrasound. Six-minute walking distance (6MWD) and New York Heart Association functional class were used to quantify exercise intolerance. Interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α) levels were measured using enzyme-linked immunosorbent assays. ResultsACHD patients showed lower forced vital capacity (FVC), and maximum inspiratory (PImax) and expiratory (PEmax) pressures compared with controls (all p < 0.05). On ultrasound, ACHD patients showed a lower diaphragm thickening ratio (2.3 ± 0.5 vs. 2.8 ± 0.9, p < 0.01) and lower diaphragm excursion velocity during a voluntary sniff maneuver (5.7 ± 2.2 vs. 7.6 ± 2.0 cm/s, p < 0.01). Respiratory parameters, such as FVC (r = 0.53; p < 0.01) and PImax (r = 0.43; p = 0.02), correlated with 6MWD. Furthermore, amino terminal pro B-type natriuretic peptide levels were inversely correlated with FVC (r = −0.54; p < 0.01). Circulating pro-inflammatory cytokines were markedly increased, and IL-6 was correlated with 6MWD, dyspnea, and biomarkers of heart, lung and inspiratory muscle function (all p < 0.05). ConclusionsOur findings show that diaphragm dysfunction is present in ACHD and relates to restrictive ventilation disorder and exercise intolerance, possibly mediated by increased IL-6 levels.

P4161Oral anticoagulation in 13,344 adult patients with congenital heart disease (ACHD) in a longitudinal real-world setting in 2005–2017

Abstract Background The life-span of patients with congenital heart disease strongly increased during the last decades due to advances in diagnostic and therapeutic approaches. Some cardiac malformations may involve an increased risk of thrombo-embolic complications. Further, adult patients with congenital heart disease (ACHD) are at increased risk to develop cardiac arrhythmia, such as atrial flutter or fibrillation. Therefore, various constellations may indicate anti-thrombotic and/ or anti-coagulative preventive or therapeutic regimen. Concomitantly, progress was made in the development of anticoagulative pharmacotherapy in the last decade with the development of the novel oral anticoagulants (NOACs). Purpose Aim of the study is to assess the use of oral anticoagulants, particularly of the NOACs dabigatran, rivaroxaban, apixaban, and edoxaban in ACHD in an unselected real-world scenario. Methods Data are derived from the German BARMER health insurance comprising approx. 9 million insurants. Within the years 2005 - 2017, we identified all adult patients that were hospitalized with a main or secondary diagnosis of a congenital heart disease by ICD-10 (Q20–28). Patients were categorized into simple, moderate, and high complexity cardiac lesions of heart disease. Oral anticoagulants were identified by ATC codes and assigned to the patient if prescribed at least twice. Results Overall, we included 13,344 ACHD patients (137,079 patient years). The use of oral anticoagulants increased from 8.3% in 2005, to 13.1% in 2010, 19.8% in 2015 and up to 22.5% of patients in 2017. Correspondingly, the use of vitamin K antagonists increased, reaching a plateau at approx. 14% since 2012. More interestingly, however, the prescription of NOACs constantly increased from 1.6% in 2012 to 8.4% in 2017. Therefore, NOACs were used for anticoagulation in 37% (n=802) of ACHD patients in 2017. Among those treated with NOACs, apixaban was used in 44.1% of patients, compared to rivaroxaban in 38.5%, edoxaban in 10.5% and dabigatran in 6.9%. With regard to heart disease complexity, oral anticoagulation was used in 21% ACHD with simple defects (1,065 of 5,080 patients), 19% of ACHD with moderate complexity disease (430 of 2,296), and 30% in complex ACHD patients (644 of 2,138). NOACs were applied to 6.2% of simple ACHD patients, 7.7% of ACHD patients with moderate complexity disease and 14.6% of complex ACHD patients. In 2017, 22.5% of ACHD (2,139) were anticoagulated. Thereof, Vit K ant. were used in 62.5%, apixaban in 16.5% and rivaroxaban in 14.4% (see figure). 2017: Use of oral anticoagulants in ACHD Conclusion Over 20% of ACHD patients require anticoagulation in the current era. Interestingly, despite the lack of prospective studies increasingly NOACs are replacing vitamin K antagonists in the ACHD population. In 2017 NOACs accounted for 37% of all anticoagulated patients in our study. The use of NOACs was not restricted to simple lesions but up to 30% of complex ACHD patients received NOACs in the current era.