Adults With Congenital Heart Disease Population Research Articles

Effect of Sodium-Glucose Cotransporter 2 Inhibitors in Adults With Congenital Heart Disease

BackgroundHeart failure (HF) is the principal cause of morbidity and mortality in adults with congenital heart disease (ACHD). Robust evidence-based treatment options are lacking. ObjectivesThis study aims to evaluate the safety, tolerability, and short-term HF-related effects of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in a real-world ACHD population. MethodsAll patients with ACHD treated with SGLT2i in 4 European ACHD centers were included in this retrospective study. Data were collected from 1 year before starting SGLT2i to the most recent follow-up. Data on side effects, discontinuation, mortality, and hospitalizations were collected. ResultsIn total, 174 patients with ACHD were treated with SGLT2i from April 2016 to July 2023. The mean age was 48.7 ± 15.3 years, 72 (41.4%) were female, and 29 (16.7%) had type 2 diabetes mellitus. Ten (5.7%) patients had mild, 75 (43.1%) moderate, and 89 (51.1%) severe congenital heart disease. HF was the most frequent starting indication (n = 162, 93.1%), followed by type 2 diabetes (n = 11, 6.3%) and chronic kidney disease (n = 1, 0.6%). At median follow-up of 7.7 months (Q1-Q3: 3.9–13.2 months), 18 patients (10.3%) reported side effects, 12 (6.9%) permanently discontinued SGLT2i, and 4 (2.3%) died of SGLT2i-unrelated causes. A significant reduction in the HF hospitalization rate was observed from 6 months before to 6 months after starting SGLT2i (relative rate = 0.30; 95% CI: 0.14-0.62; P = 0.001). ConclusionsSGLT2i generally seem safe, well-tolerated, and potentially beneficial in patients with ACHD. SGLT2i was associated with a 3-fold reduction in the 6-month HF hospitalization rate. These results warrant prospective randomized investigation of the potential benefits of SGLT2i for patients with ACHD.

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 (<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.

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.

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

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.

COVID-19 Infections in Adults with Congenital Heart Disease-A Prospective Single-Center Study in an Outpatient Setting.

Background: COVID-19 might pose a risk for adults with congenital heart disease (ACHD). However, data regarding the rate of infection as well as myocardial involvement in ACHD patients are currently lacking. Methods: During the study period from January to June 2021, all consecutive outpatients from our ACHD clinic were eligible to participate. Clinical data were collected. An antibody test for COVID-19 was performed in all patients. Cardiovascular magnetic resonance imaging (CMR) was offered to those with a positive antibody test. Results: Overall, 420 patients (44.8% female, mean age 36.4 ± 11.6 years) participated. Congenital heart defect (CHD) complexity was simple in 96 (22.9%), moderate in 186 (44.3%), complex in 117 (27.9%), and miscellaneous in 21 (5.0%) patients. Altogether, 28 (6.7%) patients had a positive antibody test. Out of these, 14 had an asymptomatic course. The others had mainly mild symptoms and were managed as outpatients. Furthermore, 11 patients (39.3%) had even not been aware of their infection. Fourteen patients underwent a CMR without signs of myocardial involvement in any of them. Conclusions: We observed a number of undetected cases of COVID-19 infections in our ACHD population. Reassuringly, in all cases, the infection had a mild clinical course.

Impact of left atrial size and strain on new atrial arrhythmias during a 5-year follow-up in adults with congenital heart disease

Abstract Introduction Atrial arrhythmias are a common and important cause of morbidity and mortality in adults with congenital heart disease (ACHD). In acquired heart disease, left atrial (LA) strain has been shown to predict supraventricular tachyarrhythmias (SVT). This study sought to investigate if LA strain is also a reliable predictor of SVT in the ACHD population. Method We retrospectively obtained baseline clinical and echocardiographic data, including LA function parameters and strain, in 206 ACHD patients. Only patients with sinus rhythm at baseline and 5-years follow-up were included (median age 29, IQR 22–41 years). 157 participants had a left heart defect (aortic stenosis or aortic coarctation, with or without correction) and 49 a right heart defect (Fallot physiology). Diagnosis of sustained SVT was determined from clinical reports during the follow-up period (standard 12-lead ECG, ECG Holter). Results During a median follow-up of 6.2 years, a new or recurrent sustained SVT occurred in 16 patients (7.8%). Patients baseline characteristics are depicted in Table 1. Patients who developed SVT were older, had larger LA dimensions and left ventricular mass, more likely diastolic dysfunction on echo, and a lower peak LA longitudinal strain (PALS). PALS was a good predictor of SVT risk in patients with left and right heart defects with an area under the receiver-operating-curve of 0.857. By Cox regression analysis, patient in the lowest quartile for PALS had a 16.7-fold higher hazard ratio of SVT (95% confidence interval, 4.7 to 59.0, p&amp;lt;0.001) in comparison with the top three quartiles. Overall freedom from arrhythmia after 1, 3 and 5 years of follow-up was 98.1%, 96.1% and 94.2%, respectively. The freedom from SVT as a function of PALS quartiles is shown in Figure 1. Conclusion PALS provides predictive information about the occurrence of SVT in the ACHD population, regardless of the type of the lesion. Including the measurement of LA strain in the follow-up of these patients may permit to better identify patients at risk of future atrial arrhythmias. Funding Acknowledgement Type of funding sources: Public hospital(s). Main funding source(s): Universitätsklinik für Kardiologie, Inselspital Bern

PO-674-03 INTRAVENOUS SOTALOL FOR ACUTE CONVERSION OF INTRA-ATRIAL-REENTRANT-TACHYCARDIA IN ADULTS WITH CONGENITAL HEART DISEASE

Intra-atrial-reentrant-tachycardia (IART) is a common source of morbidity in adults with congenital heart disease (ACHD). Most patients (pts) that present with IART are treated with direct current cardioversion (DCCV). There is limited data on the efficacy of intravenous (IV) anti-arrhythmics for acute conversion of IART in the ACHD population.

Assessment of Hypertension, Guideline-Directed Counseling, and Outcomes in the ACHD Population.

Guidelines for the diagnosis and treatment of hypertension were published by the American Heart Association (AHA) in 2017. The prevalence of hypertension in adults with congenital heart disease (ACHD) under these guidelines has yet to be characterized. We sought to assess the prevalence, impact, and provider response to hypertension under current guidelines. Data were obtained retrospectively from records of routine clinic visits over a 10year period. Potential hypertension-related adverse outcomes including stroke, myocardial infarction, surgical intervention for aortic aneurysm, aortic dissection, atrial fibrillation or flutter, cardiac transplantation and death were recorded. The 1070 patients who met inclusion criteria had a mean age of 30.8 ± 10.0years. The prevalence of hypertension under the 2017 guidelines was 46.6%. Multivariate modeling identified cyanosis, male gender, older age, and overweight/obesity as independent risk factors for hypertension. Guideline-directed management of hypertension in ACHD patients occurred more frequently in ACHD and adult cardiology clinics than in pediatric cardiology clinics (44.1% and 45.1% vs. 24.0%, p < 0.01, respectively). Adverse outcomes were reported in 217 (20%) patients, the most prevalent of which was atrial fibrillation or flutter (11%). Multivariable modelling for any adverse outcome identified older age, hypertension, cyanosis, greater complexity ACHD, and obesity as risk factors. Modifiable risk factors for atherosclerotic cardiovascular disease are common and often under addressed in the ACHD population.

Prognostic Relevance of Thyroid Disorders in Adults With Congenital Heart Disease

Adults with congenital heart disease (ACHD) are frequently affected by thyroid diseases (TDs). However, the clinical relevance of TD in ACHD remains unknown. We aimed to describe the prevalence of TD in the ACHD population and to ascertain whether TD are associated with worse outcome. Patients with ACHD >18 years attending our tertiary center for a day-case between 2014 and 2019 were included. Clinical data between patients' first visit and December 2020 were collected. Primary end point was a combination of death, hospitalization for heart failure (HF), and new-onset of arrhythmic events. Secondary end points were each part of the primary outcome as separate end points. A total of 495 patients with ACHD (32.2 [24.5 to 45.6] years; 54% women) were included. Median follow-up was 9.4 (4.5 to 13.1) years. The prevalence of TD was 30%. TD group showed worse clinical status, as demonstrated by N-terminal pro b-type natriuretic peptide values (243.5 [96.5 to 523] vs 94 [45 to 207] pg/ml, p <0.001) and New York Heart Association class (27% vs 13% in class III to IV, p <0.0001) with higher incident rate of adverse events at follow-up (4.45 [3.43 to 5.69] % vs 1.29[0.94 to 1.75] % per person-year, p <0.001). TD were independently associated with higher risk of death (hazard ratio [HR] 4.1, p = 0.009), arrhythmic events (HR 3.8, p <0.0001), and hospitalization for HF (HR 8.02, p <0.0001). There was a fourfold increased risk of primary end point in the TD group even after propensity score matching for clinical variables including age, gender, disease complexity, physiological stage, previous palliative surgery, ventricular function, pulmonary arterial hypertension, cyanosis, and presence of systemic right ventricle (HR 4.47, p <0.0001). In conclusion, TD are predictive of adverse outcome in the ACHD population. Routine screening of thyroid function during follow-up in this population may be helpful to identify those with higher risk of death, arrhythmias, and HF.

Assessment of Current Society of Thoracic Surgeons Data Elements for Adults With Congenital Heart Disease

BackgroundTo identify opportunities for enhanced data collection for adults with congenital heart disease (ACHD), a structured review of existing variables in The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (CHSD) and the STS Adult Cardiac Surgery Database (ACSD) was conducted. MethodsA working group was assembled representing multiple STS Workforces and Task Forces. The ACSD was reviewed systematically over eight 90-minute calls. ACSD version 4.20.2 and CHSD version 3.41 were used, and the ACSD was approached in sections. ACSD variables were classified as (1) represented in identical form in the CHSD (no further discussion), (2) represented in similar form in the CHSD (discussed for potential harmonization of definitions), or (3) not represented in the CHSD (discussed for potential inclusion). Variables felt to be relevant to ACHD were noted, and special consideration was given to STS required fields and variables used in existing STS adult risk models. Other factors that were examined were the frequency, use, and capture of existing ACSD variables. ResultsOver 22 weeks (8 calls), the existing 1069 variables in version 4.20.2 of the ACSD were discussed. Ultimately, 539 total variables were found to be both (1) relevant to ACHD and (2) not currently collected in the CHSD. These were recommended for inclusion in the next CHSD upgrade for patients aged ≥18 years. ConclusionsFor adult patients having case records entered into the CHSD, the inclusion of a limited set of additional data fields from the ACSD should enhance capture of comorbidities and other clinical data relevant to the ACHD population.

Anticoagulation management in adult patients with congenital heart disease: a narrative review

With improvements in treatment of congenital heart disease more paediatric patients are surviving with palliative or corrective interventions during childhood, thus becoming adults with congenital heart disease (ACHD). Overall, the ACHD population is at a higher risk of arrhythmias and stroke. The abnormal structure and function of their corrected hearts suggests that in addition to established stroke risk factors, such as prior stroke or older age, additional stroke risk factors need to be considered to determine the risk and establish the indication for oral anticoagulation (OAC) in ACHD patients. In structurally normal hearts non-vitamin-K oral anticoagulants (NOACs) offer at least equal stroke prevention with a better safety profile compared to vitamin K antagonists (VKA) in patients with atrial fibrillation (AF) or pulmonary embolism. Current guidelines recommend NOACs in ACHD patients with simple lesions and indication for OAC, while there is less certainty about their safety in ACHD patients with moderate or complex congenital heart disease such as patients with transposition of the great arteries (TGA) after atrial switch operation (Senning or Mustard operation), Fontan circulation or congenital corrected transposition of the great arteries (ccTGA). This review summarises the available evidence characterising stroke risk in patients with ACHD and the use of anticoagulants and interventional therapies to reduce that risk.

Aortic dissection and ruptures in adult congenital heart disease in Texas from 2009 to 2019.

Acute thoracic aortic dissection and rupture (TADR) has an incidence of 5-7 per 100 000-person years. Today, most children with congenital heart disease (CHD) survive to become adults with congenital heart disease (ACHD). This study evaluates TADR in patients with ACHD in a large, hospitalized patient population over 11 years to evaluate the incidence, risk factors and outcomes associated with TADR. This was a retrospective review of the Texas Inpatient Discharge Data Set from 1 January 2009 to 31 December 2019. All non-trauma discharges of patients ≥18 years were included. ACHD discharges were identified by International Classification of Diseases, 9th edition (ICD-9)/10 diagnosis codes. TADR were identified using 2 definitions: TADR1 is an ICD-9/10 code for TADR, and TADR2 is TADR1 with an ICD-9/10 procedure code for aortic intervention. Descriptive, univariate and logistic regression statistics were used. A total of 22 154 664 eligible discharges were identified, of which 12 584 (0.06%) were TADR1 and a subgroup of 5699 (0.03%) were TADR2. CHD was more prevalent in TADR1 (0.2% vs 0.05%; P < 0.001) and TADR2 (0.3% vs 0.04%; P < 0.001). Adjusting for known TADR risk factors, CHD had an odds ratio of 1.69 (95% confidence interval: 1.09-2.63; P = 0.020) for TADR1 and an odds ratio of 1.69 (95% confidence interval: 0.99-2.88; P = 0.056) for TADR2. No in-hospital deaths were found in patients with CHD with TADR. ACHD discharges had a higher frequency of TADR versus the general population (0.9-1.2 vs 0.3-0.6 per 1000 discharges). There is an indication that CHD confers an increased adjusted odds of TADR. As the ACHD population continues to grow in number as well as age, it will be important to continue to assess the risk of TADR from CHD and how traditional risk factors impact this risk.

Prediction Models and Scores in Adult Congenital Heart Disease.

Nowadays, most patients with congenital heart disease survive to adulthood due to advances in pediatric cardiac surgery but often present with various comorbidities and long-term complications, posing challenges in their management. The development and clinical use of risk scores for the prediction of morbidity and/or mortality in adults with congenital heart disease (ACHD) is fundamental in achieving optimal management for these patients, including appropriate follow-up frequency, treatment escalation, and timely referral for invasive procedures or heart transplantation. In comparison with other fields of cardiovascular medicine, there are relatively few studies that report prediction models developed in the ACHD population, given the small sample size, heterogeneity of the population, and relatively low event rate. Some studies report risk scores originally developed in pediatric congenital or non-congenital population, externally validated in ACHD with variable success. Available risk scores are designed to predict heart failure or arrhythmic events, all-cause mortality, post-intervention outcomes, infective endocarditis, or atherosclerosis-related cardiovascular disease in ACHD. A substantial number of these scores are derived from retrospective studies and are not internally or externally validated. Adequately validated risk scores can be invaluable in clinical practice and an important step towards personalized medicine. Multicenter collaboration, adequate study design, and the potential use of artificial intelligence are important elements in the effort to develop reliable risk scores for the ACHD population.

Hospitalizations in adult patients with congenital heart disease: an emerging challenge.

The vast majority of patients with congenital heart disease (CHD) survive into adulthood, but many face lifelong complications, which often result in a hospital admission. The increasing number of hospitalizations in adults with CHD (ACHD) poses a significant challenge for healthcare systems globally, especially as heart failure (HF) is becoming increasingly common in this population and is the leading cause of morbidity and mortality. Besides HF, other major contributors to this increase in admission volume are hospitalizations related to mild lesions, comorbidities and pregnancies. Ιn-hospital mortality ranges between 0.8 and 6.1%, while hospitalizations related to HF predict medium-term mortality in ACHD population. Understanding the predictors of hospitalization and in-hospital mortality is, therefore, important for ACHD healthcare providers, who should identify patients at risk that require escalation of treatment and/or close monitoring. This article reviews the available literature on hospitalization patterns in ACHD patients, with a focus on HF-related hospital admissions and specific diagnostic subgroups.

Cardiac resynchronization and implantable defibrillators in adults with congenital heart disease.

Cardiac resynchronization therapy (CRT) and implantable cardioverter defibrillators (ICDs) are well-established therapies for adult patients with heart failure that have been shown to improve morbidity and mortality. However, the benefits and indications for use in adults with congenital heart disease (ACHD) are less defined with no significant large prospective studies in this population. There are, however, multiple retrospective studies that demonstrate the efficacy of these devices in the ACHD population. These indicate a role for both CRT and ICDs in select patients with ACHD. The clinician and patient must balance the risks and benefits, summarized in complex evidence that reflects the heterogeneity of the ACHD patient group, and apply them in a patient-specific manner to optimize the utility of CRT and ICDs.

78Predictors of perioperative mortality in adults with congenital heart disease (ACHD): utility of the EuroSCORE and creation of an ACHD-specific score

Abstract Introduction Adult patients with congenital heart disease (ACHD) are a heterogeneous population and a variety of procedures are performed in ACHD centers, which differ significantly from those performed in the general adult population. Therefore, ACHD patients have not been included in the development of risk stratification models, such as the EuroSCORE. We assessed the utility of the components of the EuroSCORE in predicting in-hospital mortality around surgery in a large cohort of ACHD patients and tested a modified risk score for the ACHD population. Methods Data were collected retrospectively on all consecutive patients &gt;16 years who underwent congenital heart disease surgery in a large tertiary center in 2015–18. Preoperative characteristics and perioperative outcomes were collected from clinical records and databases. Wilcoxon rank sum test, Fisher's test and Logistic regression analysis were used to identify predictors of in-hospital death. Variables significant on univariate linear regression were used to create a risk score for each patient, either attributing 1 point for each risk factor (unweighted model), or weighing this by its log(Odds ratio) in the logistic regression model (weighted score). Receive operator characteristic (ROC) analysis with calculation of the areas under the curve (AUC) was used to assess the performance of each scoring system in predicting in-hospital mortality. Results A total of 476 operations occurred in 459 patients who underwent cardiac surgery during the study period. Age at surgery was 35.9±14.7 years, 258 (56.2%) were male, 231 (51.3%) patients had a previous sternotomy. There were 19 (4.1%) in-hospital deaths. Certain components of EuroSCORE were very rarely observed in our ACHD patients and were not included in the analysis. Of components of the EuroSCORE, female sex (OR 3.79, 95% CI: 1.42–11.89, p=0.01), functional NYHA class&gt;2 (OR 7.65, 95% CI: 2.35–29.26, p=0.001), left ventricle dysfunction (OR 3.14, 95% CI: 1.22–8.01, p=0.02), previous surgery (OR 5.33, 95% CI: 1.41–34.68, p=0.03), emergency or urgent surgery (OR 7.96, 95% CI: 3.1–21.99, p&lt;0.0001), renal dysfunction (MDRD GFR&lt;60 OR 6.56, 95% CI: 1.73–20.52, p=0.002), endocarditis (OR 7.71, 95% CI: 1.6–28.85, p=0.004), and a critical preoperative state (OR 28.65, 95% CI: 5.11–147.93, p&lt;0.0001) were predictive of an adverse perioperative outcome. Moreover, the number of previous sternotomies was predictive of mortality (OR 2.45, 95% CI: 1.58–3.85, p&lt;0.0001). Both the unweighted (AUC 0.78, 95% CI: 70.6–85.3), but especially the weighted risk score (AUC 0.82, 95% CI: 74.8–89) had an optimal discriminative power (Figure). Conclusions While several components of the EuroSCORE are relevant to ACHD patients, an ACHD-specific scoring system for predicting perioperative mortality is needed. In this analysis, we propose a simplified risk score for ACHD patients undergoing surgery, which performs well in this population.

Detection of arrhythmias in adult congenital heart disease patients with LINQTM implantable loop recorder.

Rhythm disorders are the leading cause of morbidity and mortality in adults with congenital heart disease (ACHD). Infrequent or asymptomatic arrhythmias may not be detected by routine monitoring. Implantable loop recorders (ILRs), such as the Reveal LINQTM , have been useful in long-term monitoring for arrhythmias in adults with cryptogenic stroke. We propose the Reveal LINQTM will detect arrhythmias, not documented by other monitoring modalities, resulting in change in management in ACHD patients. This is a single center retrospective review of Reveal LINQTM use in ACHD patients from 2014-2017. Medical records were reviewed to determine cardiac diagnosis, indication for implant, ILR findings, and changes in management. Twenty-two patients, median age 25years, underwent ILR implantation. ILR findings resulted in change in management in nine (41%) patients. One-third (3/9) of the patients with clinically relevant events were asymptomatic. Patients with Fontan palliation had the highest number of pertinent positive events (57%). ACHD physiologic class D patients were more likely to have a positive finding (P=.034) compared to other physiologic classes. Majority (75%) of patients with positive events had arrhythmias documented on ILR which were not demonstrated on prior Holter/event monitors. Pertinent negative event occurred in one patient with Fontan palliation (5%) who had syncope corresponding to sinus rhythm. ILRs are a useful adjunct for arrhythmia monitoring in the ACHD population with clinically relevant events in 41% of patients. A special consideration for ILRs could be made for high-risk asymptomatic patients.

The Adult with Post-operative Congenital Heart Disease: a Systematic Echocardiographic Approach.

This article attempts to review some of the commonly seen repaired congenital heart defects in the adult population (ACHD), with a focus on important echocardiographic findings that may assist a practitioner in recognizing and managing this group of patients. The prevalence of ACHD population is increasing, and currently, there are over 1 million adults with congenital heart disease. At the current time, the total number of ACHD patients exceeds the total number of pediatric patients with CHD. The recently released 2018 American College of Cardiology/American Heart Association guidelines for the management of adults with congenital heart disease (ACHD) recommends transthoracic echocardiography for all ACHD patients for initial assessment and serial assessment as needed (class of recommendation: I) and echocardiography remains the mainstay for diagnosing and managing these patients in adjunct with other available imaging modalities. It is imperative for all cardiologists to be familiar with the echocardiographic features of the commonly seen repaired CHD in adult population.

Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring.

Arrhythmias are a leading cause of death in adults with congenital heart disease (ACHD). While 24-48-hour monitors are often used to assess arrhythmia burden, extended continuous ambulatory rhythm monitors (ECAM) can record 2 weeks of data. The utility of this device and the arrhythmia burden identified beyond 48-hour monitoring have not been evaluated in the ACHD population. Additionally, the impact of ECAM has not been studied to determine management recommendations. To address the preliminary question, we hypothesized that clinically significant arrhythmias would be detected on ECAM beyond 48hours and this would lead to clinical management changes. A single center retrospective cohort study of ACHD patients undergoing ECAM from June 2013 to May 2016 was performed. The number and type of arrhythmias detected within and beyond the first 48hours of monitoring were compared using Kaplan-Meier curves and Cox proportional hazard models. Three hundred fourteen patients had monitors performed [median age 31 (IQR 25-41) years, 61% female). Significant arrhythmias were identified in 156 patients (50%), of which 46% were noted within 48 hours. A management change based on an arrhythmia was made in 49 patients (16%). ECAM detects more clinically significant arrhythmias than standard 48-hour monitoring in ACHD patients. Management changes, including medication changes, further testing or imaging, and procedures, were made based on results of ECAM. Recommendations and guidelines have been made based on arrhythmias on 48-hour monitoring; the predictive ability and clinical consequence of arrhythmias found on ECAM are not yet known.