Factors Associated With Nonattendance at the Adult Congenital Heart Disease Clinic.

Adults with congenital heart disease (CHD) are a complex population for whom adult CHD-specific care results in better outcomes. Our objective was to identify factors associated with no-shows and cancelations in an adult CHD (ACHD) clinic and evaluate the efficacy of a social worker intervention to promote ambulatory follow-up. The medical record identified adults with a scheduled appointment in the adult CHD clinic from January 2017 to March 2021. Social worker intervention was performed between March 2020 and May 2021 and consisted of phone calls to those who did not show up. Logistic regression and descriptive statistics were performed. Of 8,431 scheduled visits, 56.7% were completed, 4.6% were no-shows, and 17.5% were canceled by patients. The factors associated with no-shows were Medicaid (odds ratio [OR] 1.63, 95% confidence interval [CI] 1.26 to 2.12, p <0.001), previous no-show (OR per 1% increase in previous no-show rate 1.13, 95% CI 1.12 to 1.15, p <0.001), satellite clinic location (OR 3.15, 95% CI 2.06 to 4.74, p <0.001), virtual visit (OR 1.97, 95% CI 1.28 to 2.92, p = 0.001), and Hispanic ethnicity (OR 1.48, 95% CI 1.03 to 2.10, p = 0.031). The factors associated with cancelations were female gender (OR 1.45, 95% CI 1.25 to 1.68, p <0.001) and virtual visits (OR 2.24, 95% CI 1.50 to 3.40, p <0.001). Social worker outreach calls did not impact frequency of rescheduling. No patients accepted additional support. In conclusion, Medicaid insurance, previous number of no-shows, and Hispanic ethnicity were found to be associated with a higher risk of no-show, identifying a high-risk population that may benefit from targeted interventions. Social worker outreach did not have an appreciable impact on the rescheduling rates.

Substance Use Disorders Are Prevalent in Adults With Congenital Heart Disease and Are Associated With Increased Healthcare Use.

Adults with congenital heart disease (CHD) represent a heterogeneous group with significant long-term health risks. Previous studies have demonstrated a high prevalence of psychiatric disorders among adults with CHD; however, little is known about the frequency of co-morbid substance use disorders (SUDs) in patients with CHD. The Oregon All Payer All Claims (APAC) database for the years 2014 to 2017 was queried for adults aged 18 to 65 years with International Classification of Diseases, Ninth or Tenth Revision codes consistent with CHD. Alcohol and substance use were identified by International Classification of Diseases codes for use or dependence and classified in mutually exclusive categories of none, alcohol only, and other drugs (with or without alcohol). Descriptive statistics were used to characterize prevalence and chi-square tests were used to test for associations between variables. A total of 12,366 adults with CHD were identified. The prevalence of substance use was 15.7%. The prevalence of isolated alcohol use was 3.9%. A total of 19% of patients used tobacco. Insurance type, presence of a concurrent mental health diagnosis, and age were associated with substance use, whereas CHD complexity was not. Cardiovascular co-morbidities were more common in patients with reported substance use. Inpatient and emergency care use were higher in those with SUD. In conclusion, this study of substance and alcohol use among adults with CHD demonstrates high rates of co-morbid SUD, particularly among patients with mental health disorders and Medicaid insurance, associated with increased healthcare utilization. We identify a population in need of targeted interventions to improve long-term health.

Echocardiographic changes and impact on clinical management in pregnant women with heart disease.

BACKGROUND: While guidelines recommend echocardiography for pregnant women with heart disease, there are limited data on its effect on clinical practice. In this study, we investigated pregnancy-associated echocardiographic changes and their impact on management. METHODS: This was a retrospective study of pregnant women with heart disease followed at an academic medical centre from 2016 to 2020. Data on maternal intrapartum and postpartum echocardiograms were collected and the impact on management analysed. RESULTS: 421 echocardiograms in 232 pregnancies were included in the study. The most common cardiac diagnosis was CHD (60.8% of pregnancies), followed by cardiomyopathy (9.9%). The frequency of baseline echocardiographic abnormalities varied by diagnosis, with abnormal right ventricular systolic pressure being the most common (15.0% of pregnancies in CHD and 23.1% of pregnancies with cardiomyopathy). 39.2% of the 189 follow-up echocardiograms had a significant change from the prior study, with the most common changes being declines in right ventricular function (4.2%) or left ventricular function (3.7%), and increases in right ventricular systolic pressure (5.3%) and aortic size (21.2%). 17.8% of echocardiograms resulted in a clinical management change, with the most common change being shorter interval follow-up. CONCLUSIONS: Echocardiographic changes in pregnant women with heart disease are common, in particular increases in aortic size. Echocardiography results in changes in management in a small but significant proportion of patients. Further studies are needed to determine how other factors, including patient access and resource allocation, factor into the use of echocardiography during pregnancy.

Hospital Care for Adult Patients with Congenital Heart Diseases.

OBJECTIVE: The ideal type of hospital to care for adult congenital heart disease (ACHD) patients is not well known. Hospital competitiveness, clinical volume and market structure can influence clinical outcomes. We sought to understand how hospital competitiveness affects clinical outcomes in ACHD patients in the era prior to the Adult Congenital Heart Association accreditation program. METHODS: Patient discharges with ACHD diagnosis codes were filtered between 2006-2011 from an all-payer inpatient healthcare database. Hospital-level data was linked to market structure patient flow. A common measure of market concentration used to determine market competitiveness-the Herfindahl-Hirschman Index (HHI)-was stratified into: more competitive (HHI ≤25th percentile), moderately competitive (HHI 25th to <75th percentile), and less competitive (HHI ≥75th percentile) hospital. Any complication, home discharge and mortality were analyzed with clustered mixed effects logistic regression. The combined impact of HHI and any complication on mortality by interaction was assessed. RESULTS: A total of 67,434 patient discharges were isolated. More competitive hospitals discharged the least number of patients (N = 15,270, 22.6%) versus moderately competitive (N = 36,244, 53.7%) and less competitive (N = 15,920, 23.6%) hospitals. The adjusted odds of any complication or home discharge were not associated with hospital competitiveness strata. Compared to more competitive hospitals, mortality at moderately competitive hospitals (Adjusted Odds Ratio (AOR) 0.79, 95% CI: 0.66-0.94) and less competitive hospitals (AOR 0.79, 95% CI: 0.63-0.98) were lower (p = 0.025). Age, race, elective admission, transfer status, and payer mix were all significantly associated with adjusted odds of any complication, home discharge and mortality (p ≤ 0.05). Having any complication independently increased the adjusted odds of mortality more than 6-fold (p < 0.001), and this trend was independent of HHI strata. Failure to rescue an ACHD patient from mortality after having any complication is highest at less competitive hospitals. Sensitivity analysis which excluded the transfer status variable, showed that any complication (p = 0.047) and mortality (p = 0.01) were independently associated with HHI strata. CONCLUSIONS: Whether lower competition allow hospitals to focus more on quality of care is unknown. Hospital competitiveness and outcome seem to have an inverse trend relationship among ACHD patients. Since medical care is frequently provided away from the home area, hospital selection is an important issue for ACHD patients. Further research is needed to determine why competitiveness is linked to surgical outcomes in this population.

Racial Disparity: The Adult Congenital Heart Disease Surgery Perspective.

The influence of race and ethnicity on clinical outcomes in medicine are widely acknowledged. However, the effect of race on adult congenital heart disease (ACHD) surgery is not known. We sought to evaluate the possible association between race and outcomes following ACHD operations. Discharge records for patients who underwent ACHD surgery between 2005 and 2014, were isolated from an all-payer voluntary database in the United States. Hierarchical case-mix regression models and sensitivity analyses examined any complication, in-hospital mortality, and discharge disposition (home/non-home) by race (white-WP, black-BP, non-white non-black-NWNB). Of the 174,370 patients (WP: 80.8%, BP: 5.8%, NWNB: 13.4%), black patients were youngest to undergo surgery (WP: 57.9 ± 15.8 years, BP: 50.2 ± 16.1 years, NWNB: 51.6 ± 16.9 years, P < 0.0001), the most likely to have a comorbidity (WP: 70.3%, BP: 74.3%, NWNB: 68.6%, P < 0.0001), and most likely to have had a post-operative cardiac complication (WP: 9.4%, BP: 15.3%, NWNB: 10.9%, P < 0.0001). BP had similar odds of having any complication (AOR = 0.99, 95%CI = 0.94-1.04), while NWNB had significantly decreased odds of a major complication (AOR = 0.90, 95%CI = 0.87-0.93). BP had equivalent in-hospital mortality compared to WP (AOR = 1.03, 95%CI = 0.91-1.18), while NWNB had significantly increased odds of in-hospital mortality (AOR = 1.29, 95%CI = 1.18-1.41). Among survivors, BP were less likely to discharge home (AOR = 0.88, 95%CI = 0.82-0.94), and NWNB were more likely to discharge home than WP (AOR = 1.26, 95%CI = 1.19-1.33). Race and clinical outcomes are associated among patients undergoing surgery for ACHD. Understanding why and how these factors are impactful will help improve care for this complex population.

Thrombolytic therapy for mechanical aortic valve thrombosis in pregnancy: case report.

Background: Mechanical heart valves require long-term anticoagulation strategies to prevent valve thrombosis. Pregnant women with mechanical heart valves are especially susceptible to valve thrombosis, given their procoagulant state and the complexity of anticoagulation strategies during pregnancy. We describe a case of prosthetic valve thrombosis in a pregnant woman treated successfully with low-dose slow infusion of thrombolytic therapy. Case Summary: A 23-year-old pregnant woman with a mechanical aortic valve on subcutaneous enoxaparin presented to the maternal cardiac clinic for a follow-up visit. Her physical exam was notable for a loud grade three crescendo decrescendo murmur and follow-up transthoracic echocardiography revealed peak and mean gradients of 87 and 58 mmHg, respectively. The Doppler velocity index (DVI) was 0.24 with an acceleration time of 130 ms. Fluoroscopy confirmed a stuck leaflet disk. Thrombolysis was performed using a low-dose ultra-slow infusion of thrombolytic therapy (1 mg/h of tissue-type plasminogen activator) with the restoration of normal valve function after 8 days. A repeat transthoracic echocardiography showed a decrease in the peak and mean gradients to 37 and 21 mmHg, respectively, with an improvement in the DVI to 0.53. Repeat fluoroscopy confirmed the opening of both leaflet disks. Discussion: Treatment options for mechanical aortic valve thrombosis are either slow-infusion, low-dose thrombolytic therapy or emergency surgery. The hypercoagulable state of pregnancy makes adequate anticoagulation, proper monitoring, and medication adherence even more critical to prevent valve thrombosis. Physicians should educate pregnant patients on anticoagulation strategies and participate in shared decision-making.

Operational and Ethical Considerations for a National Adult Congenital Heart Disease Database.

As more adults survive with congenital heart disease, the need to better understand the long-term complications, and comorbid disease will become increasingly important. Improved care and survival into the early and late adult years for all patients equitably requires accurate, timely, and comprehensive data to support research and quality-based initiatives. National data collection in adult congenital heart disease will require a sound foundation emphasizing core ethical principles that acknowledge patient and clinician perspectives and promote national collaboration. In this document we examine these foundational principles and offer suggestions for developing an ethically responsible and inclusive framework for national ACHD data collection.

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 18-65 years 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 < 1 h from the ACHD center, 37.5% 1-4 h, and 10.1% > 4 h. Increased distance from the ACHD center was associated with a lower rate of ACHD-specific follow-up [< 1 h: 13.0% vs. > 4 h: 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 4 h from the ACHD center had less inpatient visits at urban hospitals (55.5% vs. 93.9% in those < 1 h) 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.

Barriers to care in urban and rural dwelling adults with congenital heart disease.

INTRODUCTION: Adults with congenital heart disease (CHD) face a unique set of medical, psychological, and social challenges, and access to specialised adult congenital heart disease care has been associated with improved outcomes. Rural adults with CHD may represent a uniquely disadvantaged group given additional challenges when accessing specialised care. The aim of this study was to investigate the challenges faced by adults with CHD in accessing outpatient cardiac care, with a specific focus on understanding differences between urban- and rural-dwelling patients. METHODS: This cross-sectional, survey-based study took place in the adult congenital heart disease clinic at an urban academic medical center. Additional medical information was abstracted in a retrospective manner from the electronic health record. In addition to descriptive statistics, t-tests and Chi-square tests were performed to investigate differences between urban and rural dwelling patients. RESULTS: A total of 100 patients participated in the study (mean age 40 ± 13 years, 60% female, 18% rural dwelling). Across the total sample, the median driving distance to clinic was 20 miles (interquartile range 12-77); it was 15 miles for urban dwellers and 77 miles for rural dwelling patients (p < 0.001). The most commonly identified barriers to cardiac clinic visits were financial losses related to taking time off from work (39%), distance of clinic from home (33%), and weather (33%). Compared to urban dwelling patients, on average those who were rural dwelling had a lower level of education (p = 0.04), more difficulty paying insurance premiums (p < 0.001) and copays (p = 0.005), and were more likely to identify the distance from clinic (p = 0.05) and having to go into the city (p = 0.02) as barriers to clinic appointments. CONCLUSIONS: The financial impact and distance to clinic were the most commonly identified barriers to outpatient cardiac care in this cohort of adults with CHD. These barriers, along with difficulty paying insurance premiums, are more common in rural dwelling patients. Initiatives such as telemedicine visits or providing financial subsidies for travel and treatment could help to expand specialty adult congenital heart disease care and better serve this growing patient population.

Association of Insurance Status With Emergent Versus Nonemergent Hospital Encounters Among Adults With Congenital Heart Disease.

Background Although the number of hospital visits has exponentially increased for adults with congenital heart disease (CHD) over the past few decades, the relationship between insurance status and hospital encounter type remains unknown. The purpose of this study was to evaluate the association between insurance status and emergent versus nonemergent encounters among adults with CHD ≥18 years old. Methods and Results We used California Office of Statewide Health Planning and Development Database from January 2005 to December 2015 to determine the trends of insurance status and encounters and the association of insurance status on encounter type among adults with CHD. A total 58 359 nonpregnancy encounters were identified in 6077 patients with CHD. From 2005 to 2015, the number of uninsured encounters decreased by 38%, whereas government insured encounters increased by 124% and private by 79%. Overall, there was a significantly higher proportion of emergent than nonemergent encounters associated with uninsured status (13.0% versus 1.8%; P<0.0001), whereas the proportion of nonemergent encounters associated with private insurance was higher than emergent encounters (35.8% versus 62.4%; P<0.0001). When individual patients with CHD became uninsured, they were ≈5 times more likely to experience an emergent encounter (P<0.0001); upon changing from uninsured to insured, they were significantly less likely to have an emergent encounter (P<0.001). After multivariate adjustment, uninsured status exhibited the highest odds of an emergent rather than nonemergent encounter compared with all other covariates (adjusted odds ratio, 9.20; 95% CI, 7.83-10.8; P<0.0001). Conclusions Efforts to enhance the ability to obtain and maintain insurance throughout the lifetime of patients with CHD might result in meaningful reductions in emergent encounters and a more efficient use of resources.

Association of Adults With Congenital Heart Disease-Specific Care With Clinical Characteristics and Healthcare Use.

Background Many adults with congenital heart disease (ACHD) are cared for by non-ACHD specialists, if they receive care at all. Little is known about the differences between those who access care at an ACHD center and those who do not access ACHD-specific care. Methods and Results The Oregon All Payer All Claims database was queried to identify subjects aged 18 to 65 years with an International Classification of Diseases,Ninth Revision (ICD-9) code consistent with ACHD from 2010 to 2015. ACHD center providers were identified using National Provider Identification numbers. Usage rates and percentages were calculated with person-years in the denominator, and rate ratios and odds ratios (ORs) were estimated using negative binomial and logistic regression. Only 11.7% of identified individuals (N=10 199) were seen at the ACHD center. These individuals were younger (median 36 versus 47 years; P<0.0001) and had higher rates of Medicaid insurance (47.8% versus 28.4%; P<0.0001), heart failure (31.4% versus 15.3%; P<0.0001), and arrhythmia (75.5 versus 49.2%; P<0.0001). They had more visits of all types (outpatient: 79% per year versus 64% per year [age-adjusted OR, 2.54; 99% CI, 2.24-2.88]; emergency department: 29% versus 22% per year [adjusted OR, 1.34; 99% CI, 1.18-1.52]; inpatient: 17% versus 12.0% per year [adjusted OR, 1.92; 99% CI, 1.67-2.20]). Rates of guideline-indicated annual echocardiography were low (7.7% overall, 13.4% in patients at the ACHD center). Conclusions Patients at an ACHD center comprise a distinct and complex group with a high rate of healthcare use and a relatively higher compliance with guideline-indicated annual follow-up. These findings underscore the importance of building and supporting robust systems for ACHD care in the United States.

Left ventricular strain and fibrosis in adults with repaired tetralogy of Fallot: A case-control study.

BACKGROUND: Left ventricular (LV) systolic dysfunction and myocardial fibrosis have prognostic implications in repaired tetralogy of Fallot (rTOF), but their relationship with myocardial strain is not well understood. We evaluated systolic strain and fibrosis (extracellular volume fraction, ECV) of the left ventricle (LV) using feature tracking with magnetic resonance and determine their association with each other and clinical outcome. METHOD: Adults with rTOF and age-matched controls underwent CMR to measure LV-ECV. Feature-tracking was used to quantify radial, circumferential, and longitudinal strain in both 2 and 3 dimensions. Clinical events (death, arrhythmia and heart-failure hospitalization) were obtained through chart review. Associations between strain, ECV and clinical events were explored. RESULTS: 48 rTOF subjects (age 40.5 ± 14.3, 42% female) and 20 healthy controls were included. Both LV 2D and 3D global circumferential strain (GCS) and global longitudinal strain (GLS) were lower in rTOF subjects (p ≤0.01 for all). There was no association between strain and LV-ECV. Strain parameters correlated with ventricular volumes and function. After a median follow-up of 8.5 years (range 1-10.9 years) there were 5 deaths, 6 hospitalizations and 9 new arrhythmias. By multivariate Cox-regression, GLS was an independent predictor of both hospitalization and death, whereas LV-ECV was an independent predictor of arrhythmia. CONCLUSION: While both LV strain abnormalities and fibrosis are present in rTOF, they are associated with different types of clinical outcome, and not to each other. The findings suggest that these measures reflect different long-term adverse adaptations to abnormal hemodynamics.

Too little too late? Communication with patients with congenital heart disease about challenges of adult life.

OBJECTIVE: To investigate the experiences and communication preferences of adult patients with congenital heart disease (CHD) in the domains of employment, insurance, and family planning. DESIGN: Patients ≥ 18 years of age completed a questionnaire about experiences and communication preferences regarding employment, health insurance, and family planning. RESULTS: Of 152 patients (median age = 33 years, 50% female, 35% with CHD of great complexity), one in four reported work-related problems due to CHD and a quarter also recalled a previous gap in health insurance. Of females, 29% experienced an unplanned pregnancy. The median importance of discussion ratings (on a 0-10 scale) were 3.5 (employment), 6.0 (insurance), and 8.0 (family planning). Few patients recalled discussions about employment (19%) or health insurance (20%). Over half recalled discussions about family planning, although males were less likely to have had these discussions than females (24% vs 86%, P < .001). Across the three domains, patients identified 16-18 years as the most appropriate age to initiate discussion, although for patients who recalled discussions, they typically occurred between 20 and 25 years. CONCLUSIONS: Adults with CHD commonly face employment, health insurance, and family planning challenges. However, discussions about these matters occur with less frequency than recommended and at older ages than patients would prefer. Communication about such issues should be incorporated into a comprehensive educational curriculum for adolescents during the process of transition to adult care.

Safety profile of ultrasound enhancing agents in echocardiography.

INTRODUCTION: Ultrasound enhancing agents (UEAs) are often utilized to enhance ultrasound image quality; however, concerns about adverse reactions have limited their use. Moreover, these agents had been either contraindicated or are labeled with a warning in patients with intra-cardiac shunts because of a theoretic risk of systemic microvascular obstruction. This labeling was recently removed in the United States, but data in these patients are lacking. METHODS: Over a 15-month period, patients receiving three different FDA-approved UEAs at our center were prospectively evaluated for clinically significant adverse events (AEs). RESULTS: A total of 5521 UEA administrations were performed (Definity® : 3306, Lumason® : 2137, Optison® : 78). There were 14 AEs (0.25%) reported (Lumason® : 0.05% [n = 1] vs Definity® : 0.39% [n = 13], P = 0.02). Back pain was the most common complaint (n = 9), followed by headache (n = 2), rash (n = 2), dyspnea (n = 2), and palpitations (n = 1). Among the 33 patients known to have intra-cardiac shunts, there were no AEs. Known right-to-left shunts with positive saline bubble study were present in 20 patients (Lumason® : n = 9, Definity® : n = 11). Left-to-right atrial shunts based on color Doppler were present in 10 patients (Lumason® : n = 5, Definity® n = 5). Three patients were known to have ventricular septal defect with left-to-right flow (Definity® : n = 2, Optison® : n = 1). CONCLUSION: Adverse events were significantly higher with Definity® ; however, overall incidences were low, and AEs were minor. Furthermore, no AEs were reported in patients with known intra-cardiac shunts. UEAs showed a good safety profile in our study and should be afforded to all appropriate patients, including those with known intra-cardiac shunts.

Psychological distress in adults with congenital heart disease: focus beyond depression.

BACKGROUND: Adults with congenital heart disease face psychological challenges although an understanding of depression vs. anxiety symptoms is unclear. We analyzed the prevalence of elevated symptoms of anxiety and depression and explored associations with demographic and medical factors as well as quality of life. METHODS: Adults with congenital heart disease enrolled from an outpatient clinic completed the Hospital Anxiety and Depression Scale and two measures of quality of life: the Linear Analogue Scale and the Satisfaction with Life Scale. Medical data were obtained by chart review. RESULTS: Of 130 patients (median age = 32 years; 55% female), 55 (42%) had elevated anxiety symptoms and 16 (12%) had elevated depression symptoms on subscales of the Hospital Anxiety and Depression Scale. Most patients with elevated depression symptoms also had elevated anxiety symptoms (15/16; 94%). Of 56 patients with at least one elevated subscale, 37 (66%) were not receiving mental health treatment. Compared to patients with 0 or 1 elevated subscales, patients with elevations in both (n=15) were less likely to be studying or working (47% vs. 81%; p=0.016) and reported lower scores on the Linear Analogue Scale (60 vs. 81, p<0.001) and the Satisfaction with Life Scale (14 vs. 28, p<0.001). CONCLUSIONS: Among adults with congenital heart disease, elevated anxiety symptoms are common and typically accompany elevated depressive symptoms. The combination is associated with unemployment and lower quality of life. Improved strategies to provide psychosocial care and support appropriate engagement in employment are required.

Hospitalization Trends and Health Resource Use for Adult Congenital Heart Disease-Related Heart Failure.

Background This study assessed trends in heart failure ( HF) hospitalizations and health resource use in patients with adult congenital heart disease ( ACHD ). Methods and Results The Nationwide Inpatient Sample was used to compare ACHD with non- ACHD HF hospitalization and health resource trends. Health resource use was assessed using total hospital charges, hospital length of stay, and procedural burden. A total of 87 175±2676 ACHD -related HF hospitalizations occurred between 1998 and 2011. During this time, ACHD HF hospitalizations increased 91% (4620±438-8809±740, P<0.0001) versus a 21% increase in non- ACHD HF hospitalizations ( P=0.003). ACHD HF hospitalization was associated with longer length of stay ( ACHD HF versus non- ACHD HF, 7.2±0.09 versus 6.8±0.02 days; P<0.0001), greater procedural burden, and higher charges ($81 332±$1650 versus $52 050±$379; P<0.0001). ACHD HF hospitalization charges increased 258% during the study period ($26 533±$1816 in 1998 versus $94 887±$8310 in 2011; P=0.0002), more than double that for non- ACHD HF ( P=0.04). Patients with ACHD HF hospitalized in high-volume ACHD centers versus others were more likely to undergo invasive hemodynamic testing (30.2±0.6% versus 20.7±0.5%; P<0.0001) and to receive cardiac resynchronization/defibrillator devices (4.7±0.3% versus 1.8±0.2%; P<0.0001) and mechanical circulatory support (3.9±0.2% versus 2.4±0.2%; P<0.0001). Conclusions ACHD -related HF hospitalizations have increased dramatically in recent years and are associated with disproportionately higher costs, procedural burden, and health resource use.