Adult Congenital Heart Disease Centers Research Articles

Electrophysiological assessment of atrial tachycardia substrates in patients with repaired tetralogy of Fallot

Abstract Introduction Adults with repaired tetralogy of Fallot (rToF) are most often referred to Adult Congenital Heart Disease centers for atrial tachycardia (AT) and radiofrequency catheter ablation (RFCA) is often required. A systematic evaluation of the mechanisms and recurrences of arrhythmias in these patients is lacking. Methods 28 patients with rToF referred for catheter ablation to the Interventional Electrophysiology Unit of our center between January 2013 and January 2023 were evaluated. Pts underwent electrophysiologic study (EPS) with 3D electroanatomic high density mapping. Evaluation of right atrial bipolar voltage and activation mapping of the AT was performed. Intra-atrial re-entrant tachycardias (IART), focal AT (FAT), other. Activation mapping was performed to identify the critical isthmus for IART. FATs were localized according to the earliest unipolar signal. Whenever possible, all the induced tachycardias were treated besides the arrhythmia which represented the clinical indication to EPS. RFCA was aimed at the earliest activation point for FAT and at the critical isthmus for IART, anchoring lesion to fixed obstacles (valve annuli or atriotomy scar). All patients provided written informed consent for participating in the study. Results Among 28 adult (age 47±13 years) patients (females n=14, 50%), 53 AT were documented: 34 (64%) IART, 11 (21%) FAT and 8 (15%) atrial fibrillation (AF). Mean tachycardia cycle length (excluding AF) was 307±95 ms. At least 2 ATs were induced in 12 pts during index EPS. Among IARTs, incisional IART (IARTinc, atriotomy and superior and inferior vena cava orifices, SVC and IVC, respectively) was the prevalent critical part of the circuit (n=22, 52%), while CTI was the second mechanism (n=20, 48%). In three pts, due to non-inducibility, a pre-emptive lesion set comprising in one case CTI and in two cases CTI+SVC-atriotomy-IVC line was performed. 2 patient underwent scheduled pulmonary veins isolation and CTI ablation. Among FAT, in 3 cases the AT was mapped in the coronary sinus, in two at the tricuspid annulus, in the remaining at the crista terminalis, at the right appendage base, at the posterolateral incision scar or between atriotomy and SVC. In 6 patients AF was induced but not ablated. During a median follow up of 23 months (interquartile range, 6-37) recurrence occurred in 8 pts (18% of pts, 15% of tachycardias). Patients with recurrences were younger (43±7 vs. 48±17 y, p=0.04); no differences were found according to critical isthmus location (4 CTI and 4 IARTinc, 50%, p=ns). Conclusions Among rToF pts with AT, IART is the prevalent mechanism and atriotomy scar is the prevalent critical isthmus. FAT location is much more variable. Atrial fibrillation burden is not negligible. Long-term freedom from AT in this clinical setting is encouraging.

Characteristics and Outcomes of a Single-Centre Cohort of Adult Congenital Heart Disease Patients Referred for Heart Transplant

Adult congenital heart disease (ACHD) services increasingly encounter heart failure (HF) in the ageing ACHD population. Optimal timing of referral for heart transplant (HTx) evaluation in this heterogeneous population is complex and ill-defined. We aim to outline the characteristics and outcomes of ACHD patients referred for HTx from a large Australian ACHD centre. Retrospective review of ACHD patients referred for HTx from a primary ACHD centre (1992-2021). Database analysis of patient demographics, characteristics, wait-listing, and transplantation outcomes was performed. A total of 45 patients (mean age 37±9.9 years old; 69% male) were referred for HTx with a mean follow-up of 5.9±6.3 years. Of these, 22 of 45 (49%) were listed and transplanted, including one heart-lung transplant. The commonest diagnosis was dextro-transposition of the great arteries (13/45, 29%). Most patients, 33 of 45 (73.3%) had undergone at least one cardiac surgery in childhood. Indications for HTx referral included HF in 34 of 45 (75%), followed by pulmonary hypertension in 7 of 45 (11%). Median transplant wait-list time was 145 days (interquartile range, 112-256). Of the 23 patients not wait-listed, the reasons included clinical stability in 13 of 45 (29%), psychosocial factors in 2 of 45 (4.4%) and prohibitive surgical risk, including multiorgan dysfunction, in 8 of 45 (17.7%). Transplant was of a single organ in most, 21 of 22 (95.5%). Overall mortality was 5 of 22 (22.7%) in those after HTx, and 14 of 23 (60.9%) in those not listed (p=0.0156). Increasingly, ACHD patients demonstrate the need for advanced HF treatments. HTx decision-making is complex, and increased mortality is seen in those not wait-listed. Ultimately, the referral of ACHD patients for HTx is underpinned by local decision-making and experience, wait-list times and outcomes.

Adult congenital heart disease training in Europe: current status, disparities and potential solutions

ObjectivesThis study aimed to determine the status of training of adult congenital heart disease (ACHD) cardiologists in Europe.MethodsA questionnaire was sent to ACHD cardiologists from 34 European countries.ResultsRepresentatives from 31...

Current State of Cardiac Implantable Electronic Device Remote Monitoring in Pediatrics and Congenital Heart Disease: A PACES-Sponsored Quality Improvement Initiative.

Cardiac implantable electronic device (CIED) remote transmissions are an integral part of longitudinal follow-up in pediatric and adult congenital heart disease (ACHD) patients. To evaluate baseline CIED remote monitoring(RM) data among pediatric and ACHD centers prior to implementation ofa Pediatric and Congenital Electrophysiology Society (PACES)-sponsored quality improvement (QI) project. This is a cross-sectional study of baseline CIED RM. Centers self-reported baseline data: individual center RM compliance was defined as high if there was > 80% achievement and low if < 50%. A total of 22 pediatric centers in the USA and Australia submitted baseline data. Non-physicians were responsible for management of the RM program in most centers: registered nurse (36%), advanced practice provider (27%), combination (23%), and third party (9%). Fifteen centers (68%) reported that > 80% of their CIED patients are enrolled in RM and only two centers reported < 50% participation. 36% reported high compliance of device transmission within 14 days of implant and 77% of centers reported high compliance of CIED patients enrolled in RM. The number of centers achieving high compliance differed by device type: 36% for pacemakers, 50% for ICDs, and 55% for Implantable Cardiac Monitors(ICM). All centers reported at least 50% adherence to recommended follow-up for PM and ICD, with 23% low compliance rate for ICMs. Based on this cross-sectional survey of pediatric and ACHD centers, compliance with CIED RM is sub-optimal. The PACES-sponsored QI initiative will provide resources and support to participating centers and repeat data will be evaluated after PDSA cycles.

Current practice of care for adolescent and adult patients after Fontan operation in Poland

Abstract Introduction For the recent decades advances in surgical and medical care have led to rapid growth in the population of adults after Fontan operation. These patients require regular surveillance testing for both cardiovascular status and multiorgan complications and comorbidities. The practice of care for patients after Fontan operation in Poland has not been systematically evaluated. We conducted an observational study in which frequency and selection of surveillance testing at large centers specialized in congenital heart diseases across Poland were investigated on the basis of multicenter survey. Material and methods Eight centers were included into the study - 5 adult congenital heart disease (ACHD) centers and 3 adolescent centers. To objectify the comparison between the centers and facilitate the interpretation of the results we have developed a model that assesses the quality of patient care - the Fontan Surveillance Score (FSS) (Table 1). The higher score is consistent with better care with the maximum of 19 points. Results The overall number of 398 Fontan patients (176 females, 222 males, mean ages: 22.5±7.9 ys) were included into the study. The group consisted of 243 adults (age &amp;gt; 18 ys) and 155 adolescents (age: 15-18 ys). There was difference in the number of Fontan patients cared in each center. The median FFS was 13 points with considerable variability between the centers (interquartile range 7-14 pts). Centers providing continuous care from pediatric period until 18 years of age achieved a higher FFS as compared to ACHD centers (median: 14 pts vs 12 points, p&amp;lt; 0.001). The majority of patients, both in ACHD (82,3%) and pediatric centers (89%) were seen annually and had respectively: physical examination (83.1% and 90.3%, p&amp;lt;0.05), electrocardiogram (83.1% vs 90.3%, p&amp;lt;0.05) and echocardiogram (82.3% and 90.3%, p=0.052) performed at each visit. Nevertheless, unsatisfactory utilization of tests identifying early stages of Fontan – associated complications was observed. The percentage of, respectively, adult and adolescent patients in whom the diagnostic tests were performed were as follows: the cardio-pulmonary exercise tests (45.7% vs 41.3%, p=0.039), cardiac magnetic resonance (7.8% vs 1.3%, p=0.004), liver biochemical (51.9% vs 67.1%, p=0.003) and morphological (6.6% vs 7.7%, p=0.68) assessment and screening for protein-losing enteropathy (31.7% vs 77.4%, p&amp;lt;0.001). Summary The results of the study proved that there is no systemic surveillance approach for Fontan patients in Poland what according to the previous data seems to be a worldwide problem. The practice for care of adolescent Fontan patients differed from the one of adults patients on many levels. The introduction of systemic solutions for the transition period and organization of ACHD centers dedicated to care for Fontan patients is needed.Table 1

Adult Congenital Heart Disease Care in Canada: Has Quality of Care Improved in the Last Decade?

BackgroundPatients with adult congenital heart disease (ACHD) are at increased risk of comorbidity and death compared with the age-matched population. Specialized care is shown to improve survival. The purpose of this study was to analyze current measures of quality of care in Canada compared with those published by our group in 2012. MethodsA survey focusing on structure and process measures of care quality in 2020 was sent to 15 ACHD centres registered with the Canadian Adult Congenital Heart Network. For each domain of quality, comparisons were made with those published in 2012. ResultsIn Canada, 36,708 patients with ACHD received specialized care between 2019 and 2020. Ninety-five cardiologists were affiliated with ACHD centres. The median number of patients per ACHD clinic was 2000 (interquartile range [IQR]: 1050, 2875). Compared with the 2012 results, this represents a 68% increase in patients with ACHD but only a 19% increase in ACHD cardiologists. Compared with 2012, all procedural volumes increased with cardiac surgeries, increasing by 12% and percutaneous intervention by 22%. Wait time for nonurgent consults and interventions all exceeded national recommendations by an average of 7 months and had increased compared with 2012 by an additional 2 months. Variability in resources were noted across provincial regions. ConclusionsOver the past 10 years, ACHD care gaps have persisted, and personnel and infrastructure have not kept pace with estimates of ACHD population growth. Strategies are needed to improve and reduce disparity in ACHD care relative to training, staffing, and access to improved care for Canadians with ACHD.

Tachyarrhythmias in congenital heart disease patients: diagnosis, clinical presentation and treatment

Tachyarrhythmias are a major cause of morbidity and mortality in adult congenital heart disease patients. There is a strong association between tachyarrhythmias and the specific congenital heart disease and the patient's surgical history. Health care for adults with congenital heart disease and arrhythmias should be coordinated by adult congenital heart disease centers of excellence that serve as a resource for consultation and referral. A complete and multidisciplinary evaluation of patients with tachyarrhythmias, including a detailed hemodynamic and electrophysiological evaluation, is mandatory to define the best treatment strategy.

Clinical and invasively-measured predictors of high exercise capacity in Fontan patients

IntroductionFontan patients have variable exercise capacity. Contemporary understanding as to which factors predict high tolerance is limited. MethodsRecords from the Ahmanson/University of California, Los Angeles Adult Congenital Heart Disease Center were reviewed for adult Fontan patients who underwent CPET. Patients were considered “high performers” if their maximum oxygen uptake (VO2 max/kg)-predicted was greater than 80%. Cross-sectional clinical, hemodynamic, and liver biopsy data was gathered. High-performers were compared to control patients across these parameters via associations and regression. ResultsA total of 195 adult patients were included; 27 patients were considered “high performers”. They had lower body mass indices (BMI, p < 0.001), mean Fontan pressures (p = 0.026), and cardiac outputs (p = 0.013). High performers also had higher activity levels (p < 0.001), serum albumin levels (p = 0.003), non-invasive and invasive systemic arterial oxygen saturations (p < 0.001 and p = 0.004), lower New York Heart Association (NYHA) heart failure class (p = 0.002), and were younger at Fontan completion (p = 0.011). High performers had less severe liver fibrosis (p = 0.015). Simple regression found Fontan pressure, non-invasive O2 saturation, albumin level, activity level, age at Fontan surgery, NYHA class, and BMI to predict significant changes in VO2 max/kg %-predicted. These associations persisted in multiple regression for non-invasive O2 saturation, NYHA class II, activity level, and BMI. ConclusionsThin Fontan patients who exercise more had better exercise capacity, Fontan hemodynamic profiles, and less liver fibrosis.

Referral Order Placement Decreases Time to Transfer to Adult Congenital Heart Disease Care.

Pediatric patients with moderate and great complexity congenital heart disease (CHD) may benefit from coordinated transfer to adult congenital heart disease (ACHD) centers to reduce the risk of complications; however, there are a variety of transfer practices. We examined the impact of referral order placement at the last pediatric cardiology visit on time to transfer to an ACHD center. We analyzed data collected from pediatric patients with moderate and great complexity CHD who were eligible to transfer to our tertiary center's accredited ACHD center. We examined transfer outcomes and time-to-transfer between those with a referral order placed at the last pediatric cardiology visit and those without using Cox proportional hazards modeling. The sample (n = 65) was 44.6% female and mean age at study start was 19.5years (± 2.2). Referral orders were placed for 32.3% of patients at the last pediatric cardiology visit. Those who had a referral order placed at the last visit had significantly higher number of successful transfers to the ACHD center compared to those who did not (95% vs 25%, p < 0.001). In a Cox regression model, placement of a referral order at the last pediatric cardiology visit was associated significantly with a sooner time to transfer (HR 6.0; 95% CI 2.2-16.2, p > 0.001), adjusting for age, sex, complexity, living location, and pediatric cardiology visit location. Placement of a referral order at the last pediatric cardiology visit may improve transfer occurrence and time to transfer to accredited ACHD centers.

SPECIAL CONSIDERATIONS IN CRITICAL CARE OF THE CONGENITAL HEART DISEASE PATIENT.

Outcomes of congenital heart disease have improved markedly over the last 20 years, with survival to adulthood now close to 90%. The mean age of admission to an intensive care unit (ICU) is 40 years. The incidence of hospital and critical care admissions have increased significantly as a consequence of this improved survival. Intensivists are now confronted with the management of complex adult congenital heart disease (ACHD) lesions not only from a cardiac perspective but also extra-cardiac organ consequences of years of abnormal circulation following surgical or palliative correction. Kidney and liver dysfunction, respiratory and hematological abnormalities are very common in this population. ACHD patients can present to the ICU for a vast number of reasons, these are classified in this review as medical non-cardiac, medical cardiac and surgical. Community/hospital acquired infections, cerebrovascular accidents, respiratory failure, alongside arrhythmias and heart failure are responsible for medical admissions. Surgical admissions include post-operative management after correction or palliation, but also medical optimization and work-up for advanced therapies. ICU management of this large heterogeneous group requires a thorough understanding of the pathophysiology in order to apply conventional adult critical care modalities; left ventricular or right ventricular dysfunction, pulmonary hypertension, intracardiac, extracardiac, palliative surgical shunts can be present and require additional consideration. This review focuses on the pathophysiology, long term sequelae, and different treatment modalities to supply a framework for the ICU physician caring for these patients. Successful outcome, especially in complex lesions is dependent on early involvement of specialized ACHD centers.

Common diagnostic errors in adults with congenital heart disease.

Owing to the great advances in the care for children with congenital heart disease by paediatric cardiac surgeons and cardiologists, there are ever increasing numbers of patients with congenital heart disease who reach adult life. At some stage during the late teenage years or soon after, these patients 'transition' from paediatric cardiac care to surveillance by cardiologists who look after adults. Many such specialists, however, are more familiar with commoner acquired heart problems such as coronary disease, heart failure, and arrhythmia in structurally normal hearts and less familiar with congenital heart disease. For this reason, international guidelines have suggested that the care of young adults with congenital heart disease take place in designated specialist adult congenital heart disease centres. It remains very important, however, for general cardiologists to have a good understanding of many aspects of adult congenital heart disease, including common pitfalls to avoid and, importantly, when to refer on, to a specialist centre. To help healthcare providers across the spectrum of cardiology practice to address common themes in adult congenital heart disease, this state-of-the-art review provides a series of case vignettes to illustrate frequent diagnostic problems that we have seen in our tertiary-level adult congenital heart disease centres, which are sometimes encountered in general cardiology settings. These include commonly 'missed' diagnoses, or errors with diagnosis or management, in these often very complex patients.

Abstract 15217: Intrahepatic Gene Expression Profile Differences in Patients With Fontan Associated Liver Disease

Introduction: Fontan associated liver disease (FALD) develops shortly after the Fontan palliation as the long-term consequence of chronically elevated central venous pressures. The molecular pathways underlying FALD remain largely unknown. We aimed to assess intrahepatic gene expression profile differences among these patients with advanced vs early fibrosis. Hypothesis: Advanced fibrosis in patients with Fontan circulation shows a specific gene expression profile with specific enriched pathways. Methods: This retrospective cohort study included adults with the Fontan circulation who had tissue available from at least one liver biopsy at the UCLA Adult Congenital Heart Disease Center. Clinical, laboratory, imaging and hemodynamic data prior to the liver biopsy were extracted from medical records. Patients were classified into early (F1-F2) or advanced fibrosis (F3-F4). RNA was isolated from formalin-fixed paraffin embedded liver tissue, RNA libraries were constructed using ribosomal RNA depletion method and sequencing was performed on Illumina Novaseq 6000. Differential gene expression and gene ontology analyses were carried out using DESeq2 and Metascape. Results: Seventy-seven patients were included, 53% women, 50% White and 20% Hispanic. Most patients had either a lateral tunnel (40%) or atriopulmonary (24%) Fontan. Four patients had 2 liver biopsies and 1 had hepatocellular carcinoma. Early and advanced fibrosis were present in 43 (54%) and 37 (46%) samples, respectively. Both groups had similar age, comorbidities, NYHA class, liver function tests and liver ultrasound features. Patients with advanced fibrosis had higher BNP, Fontan, mean pulmonary artery and capillary wedge pressures. Samples with advanced fibrosis had 921 up-regulated genes compared to the ones with early fibrosis. The up-regulated genes were enriched in extracellular matrix organization, regulation of cell adhesion, response to wounding, TGF-beta signaling pathway and blood vessel morphogenesis. Conclusions: Advanced fibrosis in patients with Fontan circulation exhibits up-regulated genes compared to early fibrosis, including pathways related to inflammation, congestion and angiogenesis, adding further insight into FALD pathophysiology.

Abstract 9972: Sex-Related Differences and the Influence of Pregnancy on Cardiac Outcomes in Adults With a Systemic Right Ventricle and Biventricular Physiology

Introduction: Transposition of the great arteries (TGA) with a systemic right ventricle (sRV) and biventricular physiology is associated with increased morbidity and mortality. There is a paucity of data regarding sex-related differences in outcomes in the context of a sRV. Moreover, pregnancy has been associated with deterioration of sRV function in short-term post-partum follow-up, but the long-term impact remains largely unknown. Methods and Results: A retrospective cohort study was conducted on 214 adults, age 44.7±12.3 years, with a sRV and biventricular physiology followed for a median of 13 years at an adult congenital heart disease center. No sex-related difference was identified in the prevalence of atrial or ventricular arrhythmias, permanent pacemaker implantation, hospitalization for heart failure, systemic atrio-ventricular valve intervention, heart transplant, or cardiac death. Among the 82 (38.3%) women, age 44.0±12.5 years, 43 (52.4%) had at least one full-term pregnancy. Women had a lower prevalence of moderate to severe sRV dysfunction than men (21% vs 42.6%, p=0.001) despite similar ages. Beta-blockers (p=0.008), furosemide (p=0.012), and mineralocorticoid receptor antagonists (p=0.028) were less frequently prescribed to women than men. Women had fewer implantable cardioverter-defibrillators (ICDs) for primary prevention than men (3.7% vs 13.6%, p=0.016), with no difference in the prevalence of secondary prevention ICDs (1.2% vs 2.3%, p=1). The four women with a prohibitive maternal mortality risk (modified WHO class IV) complied with recommendations to avoid pregnancy. After excluding these 4 women, no differences regarding frequency of adverse cardiac events, age at the time of event, and proportion with moderate or severe sRV dysfunction were observed in women with (N=43) and without (N=35) pregnancies during 14 years of follow-up. Conclusions: Women with TGA and a sRV had a lower prevalence of moderate to severe systemic ventricular dysfunction than men, along with a lower proportion of primary prevention ICDs. Following risk assessment and counselling with contraindication of pregnancy in the highest risk subgroup, pregnancy had no impact on long-term cardiac outcomes.

Ebstein's anomaly of the tricuspid valve management – the importance of right ventricle assessment

Abstract Background Ebstein's anomaly (EA) is a rare congenital heart disease (CHD) characterized by well known echocardiographic (ECHO) features. The golden standard method for evaluating these patients is cardiac magnetic resonance (CMR) although not easily accessible. The aim of the study was to evaluate the correlation between ECHO and CMR parameters and to determine the optimal parameters for long-term follow-up. Performed was a retrospective cross-section analysis from our registry, which we consider national, as we serve as the only national adult CHD centre. Material and methods Of the 48 patients in the EA national registry, 28 non-operated adults (60% male, median age 39 (18–64) years) with performed CMR were included. Analyzed were ECHO and CMR right ventricular (RV) characteristics, NTproBNP and 6-minute walking distance (6MWD). ECHO parameters were evaluated as follows: 1. morphometric [tricuspid valve septal cusp displacement (mm), long-axis RV end-diastolic dimension (RVEDD,mm), long-axis end-diastolic right to left ventricle diameter ratio (RV/LV)] and 2. functional [tricuspid annular plane systolic excursion (TAPSE,mm), RV fractional area change (FAC,%), RV tissue Doppler myocardial velocity (m/s)]. CMR-derived analyzed paramaters were: ejection fraction (EF,%) and end-diastolic volume (EDV,ml) indexed per body surface area (BSA,m2). According to CMR-derived EDV, patients were divided into groups: 1. normal size RV (55–105ml/m2), 2. dilated RV (&amp;gt;105ml/m2) and 3. small RV (&amp;lt;55ml/m2). Results For ECHO morphometric measurements, Spearman's correlation (R) showed strong, statistically significant correlation of CMR-derived EDV with ECHO-based RV/LV ratio (R=0.6989, P&amp;lt;0.0001); and moderate, statistically significant correlation with ECHO-based RVEDD (R=0.55, P=0.004); No significant correlations between CMR-derived EF and ECHO parameters were found. CMR-derived EF and EDV were independent of the severity of septal leaflet displacement; CMR-derived EF was shown to be conditional to RV EDV, with mean EF in Groups 1/2/3 as follows: 57%/36.5%/25.8% (P=0.0013). CMR-derived Group 3 (small RV) compared to Groups 1 and 2 exhibited worst 6MWD (mean 416m) and highest NTproBNP (mean 1021ng/l) though without statistical significance. Conclusions The assessment of RV in EA seems more critical than the morphology of the tricuspid valve itself. Therefore, as in the context of our results, more emphasis needs to be placed on RV size. In particular, small-capacity RV, as we found, showed strong relation to reduced RV function and some (though not significant) to NTproBNP and 6MWD. CMR-derived EDV and EF are accepted as the gold standard method for RV evaluation. Since we detected significant correlation of CMR with simply gained ECHO parameters (RV/LV ratio and RVEDD), we assume that easily accessible ECHO remains a pertinent method in routine follow-up, able to reflect the complex hemodynamic situation in EA. Funding Acknowledgement Type of funding sources: None.

ADULT CONGENITAL HEART DISEASE HEALTH SERVICES IN CANADA-WHERE HAVE WE COME IN THE PAST 15 YEARS

ADULT CONGENITAL HEART DISEASE HEALTH SERVICES IN CANADA-WHERE HAVE WE COME IN THE PAST 15 YEARS

Cardiopulmonary Exercise Test Outcomes in Fontan Patients With Right Versus Left Single Ventricle Morphology.

Background: Congenital heart defects (CHDs) palliated with Fontan surgery often result in a functional single ventricle that is either a morphologically right or left ventricle, and much less commonly undefined. Given this departure from normal physiology, especially for systemic right ventricle Fontan patients, our study sought to compare cardiopulmonary exercise test (CPET) results of adult patients with single right ventricle (SRV) and single left ventricle (SLV) morphology. Methods: Of 237 Fontan patients from the Ahmanson/UCLA Adult Congenital Heart Disease Center database, 135 patients met the inclusion criteria and were split into 2 groups: SRV (n = 44) and SLV (n = 91). Data were collected on baseline demographics, cardiac history, and CPET results. The 2 groups were compared using unpaired t-test, Mann-Whitney, or Chi-square test. Results: Regarding baseline demographics, SRV patients underwent CPET at a slightly younger age than the SLV group (26.5 ± 6.2 vs 29.6 ± 8.5 years, P = .03). There were no significant differences in CPET parameters (including peak heart rate, oxygen saturation, and maximum VO2/kg) between the SRV and SLV groups. When evaluated subsequent CPET at 3 to 4 years, there was no difference in CPET peak heart rate, peak oxygen saturation, and maximum VO2/kg between the 2 groups. Conclusions: This single-center retrospective analysis suggests that dominant single ventricle morphology may not be associated with an appreciable difference in exercise performance in adult survivors with a Fontan palliation.

Clinical complications of liver disease in adults after the Fontan operation

BackgroundThe natural history and complications of Fontan-associated liver disease (FALD) are not well-characterized. We aim to describe the incidence and prevalence of and risk factors for adverse liver-related complications in FALD. MethodsThis is a single-center, retrospective cohort study of adult Fontan patients followed in an adult congenital heart disease center from 2006 to 2019. The primary outcome was clinical complications of liver disease (CCLD) defined as portal hypertension (pHTN)-related ascites, pHTN-related gastrointestinal bleeding, hepatic encephalopathy, or hepatocellular carcinoma. We also explored the composite outcome of CCLD, transplantation, or death. ResultsAmong 182 adult Fontan patients (46% female), median time from Fontan to first liver evaluation was 25.5 years (interquartile range [IQR] 22.3–30.4) with median subsequent follow-up of 4.1 years (IQR 2.5–7.6). There were 14 CCLD, 6 heart-liver and 1 heart transplant, and 16 deaths. The cumulative incidence of CCLD and the composite outcome were 2.6 per 1000-person years and 6.2 per 1000 person-years, respectively. Median time from Fontan to CCLD was 26.8 years (IQR 23.1–31.2), and to the composite outcome 26.6 years (IQR 23.1–30.5). Prior diuretic use was associated with the development of CCLD (hazard ratio [HR] 10.03, 95% confidence interval [CI] 1.25–80.22, P 0.030). Diuretic use (HR 16.47, 95% CI 3.90–69.57, P < 0.001), thrombocytopenia (HR 2.30, 95% CI 1.05–5.01, P 0.036), and cyanosis (HR 5.68, 95% CI 2.47–13.08, P < 0.001) were associated with the composite outcome. ConclusionWe identified clinical factors associated with adverse outcomes in FALD that may help guide early recognition of complications and clinical management.

Abstract 12850: The Effect of Regionalization on End-of-Life Hospitalizations for Adult Congenital Cardiology Patients

Introduction: Adult congenital heart disease (ACHD) patients may have shortened life expectancy due to complications of interventions and accrued comorbidities. Specialized ACHD care has become standard of care. The effect of regionalization on ACHD patient death is not known. Methods: The Office of Statewide Health Planning and Development database identified ACHD patients ≥ 18 years who died in California hospitals from 1995 - 2018. Specialized ACHD centers were self-designated, so high and low ACHD surgical volume centers were also compared. Results: Of 3,724 patients, when comparing by volume, more deaths occurred at low (85.2%) than high volume centers (14.8%); when comparing by specialization, more deaths occurred at non-ACHD (78.8%) than ACHD centers (21.2%). Moderate (9.4%) and high complexity (4.4%) lesions were rare. Common causes of death were sepsis (14.6%), myocardial infarction (9.3%), stroke (7.5%), and heart failure (7.1%). High volume centers were more likely the location of death than low volume centers for patients aged 18-64, with private insurance, and who lived &gt;20 miles away (p &lt;0.0001 for all). These held true for ACHD vs non-ACHD centers (p &lt;0.0001 for all). Low volume centers were more likely the location of death than high volume centers for patients from zip codes with relative poverty (p 0.0243). This held true for non-ACHD vs ACHD centers (p 0.0182). Over 24 years, the proportion of patients dying at ACHD centers increased (Figure 1), while the proportion of patients dying at high volume centers decreased. Conclusion: ACHD patients who die at high surgical volume and ACHD self-designated centers are more likely to be young, have private insurance, be referred to centers far from home, and have higher socioeconomic status. These likely reflect disparities in access to specialized ACHD care. Despite regionalization with an increase in specialized ACHD centers over time, the majority of ACHD patients continue to die at non-specialized centers.

Abstract 12598: Association Between Insurance Type, Clinical Characteristics, and Healthcare Use in Adults With Congenital Heart Disease

Introduction: Adults with congenital heart disease (CHD) represent a heterogeneous and growing population with high healthcare utilization. We sought to understand the association between insurance type, healthcare use, and outcomes among adults with CHD in Oregon. Methods: The Oregon All Payers All Claims database from 2010-2017 was queried for adults aged 18-65 in 2014 with ICD-9 or 10 codes consistent with CHD; patient demographics, comorbidities, healthcare use, and disease severity were identified. Insurance type was categorized as either public (Medicare and Medicaid) or private (commercial or self-pay). Descriptive statistics were used to compare groups. Use rates and odds ratios were calculated representing probability of at least one event per person-year using logistic regression with clustering on patients. Results: Of 12,978 adults with CHD, 53% were privately insured. More publicly insured patients had moderate-severe anatomic complexity (29.5% vs 22.9%; p &lt; 0.0001), drug and alcohol use (22.4% vs 4.9%; p &lt; 0.0001), and mental health diagnoses (66.6% vs 50.7%; p &lt; 0.0001). They were more likely to reside in a rural area (24.7% vs 16.0%; p &lt; 0.0001), and less likely to be within an hour of an adult congenital heart disease center (46.7% vs 57%; p &lt; 0.0001). Adjusted for age and CHD diagnosis, publicly insured patients were more likely to use ambulatory (aOR 1.71; 99% CI 1.60, 1.83), emergency (aOR 3.44; 99% CI 3.22, 3.69), and inpatient (aOR 2.63; 99% CI 2.42, 2.85) care ( Table ). Differences in use were more pronounced among patients with higher anatomic complexity. Conclusions: Our study shows that publicly insured adults with CHD in Oregon have more anatomically complex disease, more comorbidities, and higher healthcare use despite controlling for CHD diagnosis. Adults with CHD with public insurance represent a population that may benefit from targeted interventions addressing social determinants of health to improve outcomes and decrease healthcare use.

Distance to Care, Rural Dwelling Status, and Patterns of Care Utilization in Adult Congenital Heart Disease.

Many patients with adult congenital heart disease (ACHD) do not receive guideline-directed care. While distance to an ACHD center has been identified as a potential barrier to care, the impact of distance on care location is not well understood. The Oregon All Payer All Claims database was queried to identify subjects 18-65years who had a health encounter from 2010 to 2015 with an International Classification of Diseases-9 code consistent with ACHD. Residence area was classified using metropolitan statistical areas and driving distance was queried from Google Maps. Utilization rates and percentages were calculated and odds ratios were estimated using negative binomial and logistic regression. Of 10,199 identified individuals, 52.4% lived < 1h from the ACHD center, 37.5% 1-4h, and 10.1% > 4h. Increased distance from the ACHD center was associated with a lower rate of ACHD-specific follow-up [< 1h: 13.0% vs. > 4h: 5.0%, adjusted OR 0.32 (0.22, 0.48)], but with more inpatient, emergency room, and outpatient visits overall. Those who more lived more than 4h from the ACHD center had less inpatient visits at urban hospitals (55.5% vs. 93.9% in those < 1h) and the ACHD center (6.2% vs. 18.2%) and more inpatient admissions at rural or critical access hospitals (25.5% vs. 1.9%). Distance from the ACHD center was associated with a decreased probability of ACHD follow-up but higher health service use overall. Further work is needed to identify strategies to improve access to specialized ACHD care for all individuals with ACHD.