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Background: Patients with transposition of the great arteries (TGA) and systemic right ventricle often confront significant adverse cardiac events. The prognostic significance of invasive hemodynamic parameters in this context remains uncertain. Our hypothesis is that the aortic pulsatility index and hemodynamic profiling utilizing invasive measures provide prognostic insights for patients with TGA and a systemic right ventricle., Methods: This retrospective multicenter cohort study encompasses adults with TGA and a systemic right ventricle who underwent cardiac catheterization. Data collection, spanning from 1994 to 2020, encompasses clinical and hemodynamic parameters, including measured and calculated values such as pulmonary capillary wedge pressure, aortic pulsatility index, and cardiac index. Pulmonary capillary wedge pressure and cardiac index values were used to establish 4 distinct hemodynamic profiles. A pulmonary capillary wedge pressure of ≥15 mm Hg indicated congestion, termed wet, while a cardiac index <2.2 L/min per m 2 signified inadequate perfusion, labeled cold. The primary outcome comprised a composite of all-cause death, heart transplantation, or the requirement for mechanical circulatory support., Results: Of 1721 patients with TGA, 242 individuals with available invasive hemodynamic data were included. The median follow-up duration after cardiac catheterization was 11.4 (interquartile range, 7.5-15.9) years, with a mean age of 38.5±10.8 years at the time of cardiac catheterization. Among hemodynamic parameters, an aortic pulsatility index <1.5 emerged as a robust predictor of the primary outcome, with adjusted hazard ratios of 5.90 (95% CI, 3.01-11.62; P <0.001). Among the identified 4 hemodynamic profiles, the cold/wet profile was associated with the highest risk for the primary outcome, with an adjusted hazard ratio of 3.83 (95% CI, 1.63-9.02; P <0.001)., Conclusions: A low aortic pulsatility index (<1.5) and the cold/wet hemodynamic profile are linked with an elevated risk of adverse long-term cardiac outcomes in patients with TGA and systemic right ventricle., Competing Interests: None.

Background: Fenestrating a Fontan baffle has been associated with improved perioperative outcomes in patients with univentricular hearts. However, longer-term potential adverse effects remain debated. We sought to assess the impact of a fenestrated Fontan baffle on adverse cardiovascular events including all-cause mortality, cardiac transplantation, atrial arrhythmias, and thromboemboli., Methods: A multicentre North American retrospective cohort study was conducted on patients with total cavopulmonary connection Fontan baffle, with and without fenestration. All components of the composite outcome were independently adjudicated. Potential static and time-varying confounders were taken into consideration, along with competing risks., Results: A total of 407 patients were followed for 10.4 (7.1-14.4) years; 70.0% had fenestration of their Fontan baffle. The fenestration spontaneously closed or was deliberately sealed in 79.9% of patients a median of 2.0 years after Fontan completion. In multivariable analysis in which a persistent fenestration was modelled as a time-dependent variable, an open fenestration did not confer a higher risk of the composite outcome (hazard ratio, 1.18; 95% confidence interval, 0.71-1.97; P = 0.521). In secondary analyses, an open fenestration was not significantly associated with components of the primary outcome: that is, mortality or transplantation, atrial arrhythmias, or thromboemboli. However, sensitivity analyses to assess the possible range of error resulting from imprecise dates for spontaneous fenestration closures could not rule out significant associations between an open fenestration and atrial arrhythmias or thromboemboli., Conclusions: In this multicentre study, no significant association was identified between an open fenestration in the Fontan baffle and major adverse cardiovascular events., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

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., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: There is a paucity of data on long-term outcomes after Fontan palliation in patients with a dominant morphological univentricular right (uRV) vs left (uLV) ventricle., Objectives: The purpose of this study was to compare the incidence of atrial arrhythmias, thromboembolic events, cardiac transplantation, and death following Fontan palliation in patients with uRV vs uLV., Methods: The Alliance for Adult Research in Congenital Cardiology conducted a multicenter retrospective cohort study on patients with total cavopulmonary connection Fontan palliation across 12 centers in North America. All components of the composite outcome, that is, atrial arrhythmias, thromboembolic events, cardiac transplantation, and death, were reviewed and classified by a blinded adjudicating committee. Time-to-event analyses were performed that accounted for competing risks., Results: A total of 384 patients were followed for 10.5 ± 5.9 years. The composite outcome occurred in 3.7 vs 1.7 cases per 100 person-years for uRV (N = 171) vs uLV (N = 213), respectively ( P  < 0.001). In multivariable analyses, uRV conferred a >2-fold higher risk of the composite outcome (HR: 2.17, 95% CI: 1.45-3.45, P  < 0.001). In secondary analyses of components of the primary outcome, uRV was significantly associated with a greater risk of cardiac transplantation or death (HR: 9.09, 95% CI: 2.17-38.46, P  < 0.001) and atrial arrhythmias (HR: 2.17, 95% CI: 1.20-4.00, P  = 0.010) but not thromboembolic events (HR: 1.64, 95% CI: 0.86-3.16, P  = 0.131)., Conclusions: Fontan patients with uRV vs uLV morphology have a higher incidence of adverse cardiovascular events, including atrial arrhythmia, cardiac transplantation, and all-cause mortality., Competing Interests: The TACTIC study was funded by an investigator-initiated unrestricted grant from Boehringer Ingelheim. The sponsor had no role in the study design, data collection, analysis, interpretation, writing of the paper, and decision to submit the manuscript for publication. Dr Dib is supported by the 10.13039/501100003100French Federation of Cardiology (FFC) and 10.13039/501100009597ADETEC grants. Dr Khairy is supported by the André Chagnon research chair in electrophysiology and congenital heart disease. All other have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

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.

Background: Altered coagulation is a striking feature of COVID-19. Adult patients with congenital heart disease (ACHD) are prone to thromboembolic (TE) and bleeding complications., Objectives: The purpose of this study was to investigate the prevalence and risk factors for COVID-19 TE/bleeding complications in ACHD patients., Methods: COVID-19-positive ACHD patients were included between May 2020 and November 2021. TE events included ischemic cerebrovascular accident, systemic and pulmonary embolism, deep venous thrombosis, myocardial infarction, and intracardiac thrombosis. Major bleeding included cases with hemoglobin drop >2 g/dl, involvement of critical sites, or fatal bleeding. Severe infection was defined as need for intensive care unit, endotracheal intubation, renal replacement therapy, extracorporeal membrane oxygenation, or death. Patients with TE/bleeding were compared to those without events. Factors associated with TE/bleeding were determined using logistic regression., Results: Of 1,988 patients (age 32 [IQR: 25-42] years, 47% male, 59 ACHD centers), 30 (1.5%) had significant TE/bleeding: 12 TE events, 12 major bleeds, and 6 with both TE and bleeding. Patients with TE/bleeding had higher in-hospital mortality compared to the remainder cohort (33% vs 1.7%; P  < 0.0001) and were in more advanced physiological stage ( P  = 0.032) and NYHA functional class ( P  = 0.01), had lower baseline oxygen saturation ( P  = 0.0001), and more frequently had a history of atrial arrhythmia ( P  < 0.0001), previous hospitalization for heart failure ( P  < 0.0007), and were more likely hospitalized for COVID-19 ( P  < 0.0001). By multivariable logistic regression, prior anticoagulation (OR: 4.92; 95% CI: 2-11.76; P  = 0.0003), cardiac injury (OR: 5.34; 95% CI: 1.98-14.76; P  = 0.0009), and severe COVID-19 (OR: 17.39; 95% CI: 6.67-45.32; P  < 0.0001) were independently associated with increased risk of TE/bleeding complications., Conclusions: ACHD patients with TE/bleeding during COVID-19 infection have a higher in-hospital mortality from the illness. Risk of coagulation disorders is related to severe COVID-19, cardiac injury during infection, and use of anticoagulants., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2023 The Authors.)

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., Competing Interests: Conflict of interest: All authors declare no conflict of interest for this contribution., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background and Aims: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear., Methods: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death., Results: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome., Conclusions: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

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., Competing Interests: Disclosures The authors have no conflicts of interest to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart disease is the most common cardiovascular condition in this group, present in up to 50% of people with Down syndrome and contributing to poor outcomes. Additional factors contributing to cardiovascular outcomes include pulmonary hypertension; coexistent pulmonary, endocrine, and metabolic diseases; and risk factors for atherosclerotic disease. Moreover, disparities in the cardiovascular care of people with Down syndrome compared with the general population, which vary across different geographies and health care systems, further contribute to cardiovascular mortality; this issue is often overlooked by the wider medical community. This review focuses on the diagnosis, prevalence, and management of cardiovascular disease encountered in people with Down syndrome and summarizes available evidence in 10 key areas relating to Down syndrome and cardiac disease, from prenatal diagnosis to disparities in care in areas of differing resource availability. All specialists and nonspecialist clinicians providing care for people with Down syndrome should be aware of best clinical practice in all aspects of care of this distinct population.

Background: Vitamin K antagonists (VKAs) are frequently prescribed to patients with congenital heart disease (CHD) for atrial arrhythmias or Fontan palliation, but there is a paucity of data regarding time spent in the therapeutic range (TTR). We sought to determine the TTR in patients with CHD and atrial arrhythmias or Fontan palliation prescribed VKAs and explore associations with thromboembolic and bleeding events., Methods: A multicentre North American cohort study was conducted on patients with CHD who received VKAs for sustained atrial arrhythmia or Fontan palliation. TTR was calculated using the Rosendaal linear interpolation method. Generalized estimating equations were used to explore factors associated with time outside the therapeutic range., Results: A total of 567 patients, aged 33 ± 17 years, 56% female, received VKAs for 11.5 ± 8.4 years for atrial arrhythmias (63.0%) or Fontan palliation (58.0%). CHD was simple, moderate, and complex in 10.8%, 20.3%, and 69.0%, respectively. Site investigators perceived good control over international normalized ratio (INR) levels in most patients (75.3%), with no or minor compliance or adherence issues (85.6%). The mean TTR was 41.9% (95% confidence interval [CI], 39.0%-44.8%). Forty-seven (8.3%) and 34 (6.0%) patients had thromboembolic and bleeding events, respectively. Thromboembolic events were associated with a higher proportion of time below the therapeutic range (31.3% vs 19.1%, P = 0.003) and bleeding complications with a higher proportion of time above the therapeutic range (32.5% vs 19.5%, P = 0.006)., Conclusions: Patients with CHD who receive VKAs spend < 42% of their time with INR levels in the therapeutic range, with repercussions regarding thromboembolic and bleeding complications., (Copyright © 2022 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes., Objectives: The authors aimed to determine factors associated with survival in a large cohort of such individuals., Methods: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS)., Results: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography., Conclusions: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk., Competing Interests: Funding Support and Author Disclosures This study was funded by a joint grant from the Children’s Heart Foundation and the American Heart Association (17GRNT33670334). Dr Opotowsky was supported by the Heart Institute Research Core at Cincinnati Children’s Hospital and the Dunlevie Family Fund. The authors used the Research Electronic Data Capture (REDCap) online database, which is funded by the National Institutes of Health (UL1TR002369). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

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., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Cardiovascular disease is the leading cause of death during pregnancy with thoracic aortic dissection being one of the main causes. Thoracic aortic disease is commonly related to hereditary disorders and congenital heart malformations such as bicuspid aortic valve (BAV). Pregnancy is considered a high risk period in women with underlying aortopathy., Methods: The ESC EORP Registry Of Pregnancy And Cardiac disease (ROPAC) is a prospective global registry that enrolled 5739 women with pre-existing cardiac disease. With this analysis, we aim to study the maternal and fetal outcome of pregnancy in women with thoracic aortic disease., Results: Thoracic aortic disease was reported in 189 women (3.3%). Half of them were patients with Marfan syndrome (MFS), 26% had a BAV, 8% Turner syndrome, 2% vascular Ehlers-Danlos syndrome and 11% had no underlying genetic defect or associated congenital heart defect. Aortic dilatation was reported in 58% of patients and 6% had a history of aortic dissection. Four patients, of whom three were patients with MFS, had an acute aortic dissection (three type A and one type B aortic dissection) without maternal or fetal mortality. No complications occurred in women with a history of aortic dissection. There was no significant difference in median fetal birth weight if treated with a beta-blocker or not (2960 g (2358-3390 g) vs 3270 g (2750-3570 g), p value 0.25)., Conclusion: This ancillary analysis provides the largest prospective data review on pregnancy risk for patients with thoracic aortic disease. Overall pregnancy outcomes in women with thoracic aortic disease followed according to current guidelines are good., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

We are witnessing an unparalleled pandemic caused by the novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) associated with coronavirus disease 2019 (COVID-19). Current data show that SARS-CoV-2 results in mild flu-like symptoms in the majority of healthy and young patients affected. Nevertheless, the severity of COVID-19 respiratory syndrome and the risk of adverse or catastrophic outcomes are increased in patients with pre-existing cardiovascular disease. Patients with adult congenital heart disease (ACHD)-by definition-have underlying cardiovascular disease. Many patients with ACHD are also afflicted with residual haemodynamic lesions such as valve dysfunction, diminished ventricular function, arrhythmias or cyanosis, have extracardiac comorbidities, and face additional challenges regarding pregnancy. Currently, there are emerging data of the effect of COVID-19 on ACHD patients, but many aspects, especially risk stratification and treatment considerations, remain unclear. In this article, we aim to discuss the broad impact of COVID-19 on ACHD patients, focusing specifically on pathophysiology, risk stratification for work, self-isolation, hospitalization, impact on pregnancy, psychosocial health, and longer-term implications for the provision of ACHD care., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2020. For permissions, please email: journals.permissions@oup.com.)

Background: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications., Objectives: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes., Methods: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined., Results: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not., Conclusions: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

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., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to disclose., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Patients with systemic right ventricle (sRV), including transposition of great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of great arteries (ccTGA), may require anticoagulation for thromboembolism (TE) prevention. In the absence of data on non-vitamin K antagonist oral anticoagulants (NOACs), vitamin K antagonists (VKAs) remain the agent of choice. We investigated the safety, efficacy and feasibility of NOACs treatment in adults with sRV in a worldwide study., Methods: This is an international multicentre prospective study, using data from the NOTE registry on adults with sRV taking NOACs between 2014 and 2019. The primary endpoints were TE and major bleeding (MB). The secondary endpoint was minor bleeding., Results: A total of 76 patients (42.5 ± 10.0 years, 76% male) with sRV (74% TGA, 26% ccTGA) on NOACs were included in the study. During a median follow-up of 2.5 years (IQR1.5-3.9), TE events occurred in 3 patients (4%), while no MB episodes were reported. Minor bleeding occurred in 9 patients (12%). NOAC treatment cessation rate was 1.4% (95%CI:0.3-4%) during the first year of follow-up. All the patients with TE events had a CHA 2 DS 2 -VASc score ≥ 2 and impaired sRV systolic function at baseline. The total incidence of major events during follow-up was significantly lower compared to historical use of VKAs or aspirin before study inclusion (1.4% (95%CI:0.29-4%) vs 6,9% (95%CI:2.5-15.2%); p = .01)., Conclusions: In this prospective study, NOACs appear to be well-tolerated, with excellent efficacy and safety at mid-term in patients with sRV., Competing Interests: Declaration of Competing Interest Dr. B.J. Bouma has received restricted research grant from Bristol-Myers Squibb and Pfizer. Prof. B.J.M. Mulder has received restricted research grants from Ingelheim-Boehringer, Bayer, and Daiichi Sankyo. The other Authors report no relationships that could be construed as a conflict of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objectives: Women with Turner syndrome (TS) are frequently counselled against pregnancy due to lack of data and unclear aortic dissection risk. However, with advances in fertility therapy, more women with TS are contemplating pregnancy. This study compared rates of adverse cardiovascular (CV) outcomes among: (1) pregnant and non-pregnant women with TS and (2) pregnant women with TS with/without structural heart disease., Methods: Retrospective analysis of pregnant and age-matched non-pregnant controls with TS (2005-2017) across 10 CV centres was done. Data were collected at initial evaluation in pregnancy and outcomes were assessed to 6 months postpartum. Adverse CV events were defined as CV death, aortic dissection/rupture and/or aortic intervention. Non-pregnant age-matched controls were followed over the same time period., Results: Sixty-eight pregnancies were included (60 women, mean age 33 years, 48% primigravid, 49% fertility therapy, 80% structurally normal heart, 25% XO karyotype). Based on American Society of Reproductive Medicine criteria, 10 pregnancies occurred in women stratified to high-risk category. There were no CV events in the pregnant women or in the non-pregnant women with TS. Obstetric events complicated 12 (18%) pregnancies with 9 (13%) attributed to hypertensive disorder of pregnancy. Fetal events included small for gestational age neonates (18%), preterm delivery (15%) and fetal death (3%)., Conclusions: This study helps to refine the approach to pregnancy in women with TS. Among women with TS without structural heart disease, pregnancy does not impose an increased risk of CV outcomes. Among women with TS with structural heart disease, the risk of pregnancy is not as prohibitive as previously described but does require ongoing evaluation., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Venous stenosis is a late complication of the atrial switch (Mustard/Senning) procedure seen in patients with transposition of the great arteries ( d-TGA). Many atrial switch patients require cardiac implantable electronic devices (CIEDs) which further increases the incidence of venous stenosis. Stenosis of the superior limb of the systemic venous pathway (SLSVP) in the presence of CIED leads presents a management challenge. We propose a method for navigating SLSVP stenosis in atrial switch patients with CIEDs., Methods: The pulse generator and leads were removed using standard extraction techniques. Axillary access was retained via existing leads or new access was obtained. The interventional cardiology team, via groin access, performed stent-angioplasty of the stenotic SLSVP. After stent deployment, the axillary access wire was snared from below, guided through the stent, and pulled into a long groin sheath. A sheath was then advanced over the axillary wire and into the groin sheath creating a path for passage of leads through the stent. New leads were advanced through the axillary sheath into the heart. Leads were secured using standard techniques., Results: All patients had a history of d-TGA and prior atrial switch procedures. In each case, there was stenosis of the SLSVP in the setting of a CIED lead. There were no immediate complications and there was no restenosis on follow-up., Conclusion: Post-atrial switch patients with CIEDs can develop stenosis of the SLSVP. A collaboration between electrophysiology and interventional cardiology can allow for device extraction, stent-angioplasty, and lead reimplantation to avoid "jailing" the leads., (© 2020 Wiley Periodicals LLC.)

Background: Adult congenital heart disease (ACHD) patients can benefit from a subcutaneous implantable cardioverter-defibrillator (S-ICD)., Objective: The purpose of this study was to assess left- and right-sided S-ICD eligibility in ACHD patients, use machine learning to predict S-ICD eligibility in ACHD patients, and transform 12-lead electrocardiogram (ECG) to S-ICD 3-lead ECG, and vice versa., Methods: ACHD outpatients (n = 101; age 42 ± 14 years; 52% female; 85% white; left ventricular ejection fraction [LVEF] 56% ± 9%) were enrolled in a prospective study. Supine and standing 12-lead ECG were recorded simultaneously with a right- and left-sided S-ICD 3-lead ECG. Peak-to-peak QRS and T amplitudes; RR, PR, QT, QTc, and QRS intervals; Tmax, and R/Tmax (31 predictor variables) were tested. Model selection, training, and testing were performed using supine ECG datasets. Validation was performed using standing ECG datasets and an out-of-sample non-ACHD population (n = 68; age 54 ± 16 years; 54% female; 94% white; LVEF 61% ± 8%)., Results: Forty percent of participants were ineligible for S-ICD. Tetralogy of Fallot patients passed right-sided screening (57%) more often than left-sided screening (21%; McNemar χ 2 P = .025). Female participants had greater odds of eligibility (adjusted odds ratio [OR] 5.9; 95% confidence interval [CI] 1.6-21.7; P = .008). Validation of the ridge models was satisfactory for standing left-sided (receiver operating characteristic area under the curve [ROC AUC] 0.687; 95% CI 0.582-0.791) and right-sided (ROC AUC 0.655; 95% CI 0.549-0.762) S-ICD eligibility prediction. Validation of transformation matrices showed satisfactory agreement (<0.1 mV difference)., Conclusion: Nearly half of the contemporary ACHD population is ineligible for S-ICD. The odds of S-ICD eligibility are greater for female than for male ACHD patients. Machine learning prediction of S-ICD eligibility can be used for screening of S-ICD candidates., (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Advance care planning and palliative care are gaining recognition as critical care components for adults with CHD, yet these often do not occur. Study objectives were to evaluate ACHD providers' 1) comfort managing patients' physical symptoms and psychosocial needs and 2) perspectives on the decision/timing of advance care planning initiation and palliative care referral., Methods: Cross-sectional study of ACHD providers. Six hypothetical patients were described in case format, followed by questions regarding provider comfort managing symptoms, initiating advance care planning, and palliative care referral., Results: Fifty providers (72% physicians) completed surveys. Participants reported low levels of personal palliative care knowledge, without variation by gender, years in practice, or prior palliative care training. Providers appeared more comfortable managing physical symptoms and discussing prognosis than addressing psychosocial needs. Providers recognised advance directives as important, although the percentage who would initiate advance care planning ranged from 18 to 67% and referral to palliative care from 14 to 32%. Barriers and facilitators to discussing advance care planning with patients were identified. Over 20% indicated that advance care planning and end-of-life discussions are best initiated with the development of at least one life-threatening complication/hospitalisation., Conclusions: Providers noted high value in advance directives yet were themselves less likely to initiate advance care planning or refer to palliative care. This raises the critical questions of when, how, and by whom discussion of these important matters should be initiated and how best to support ACHD providers in these endeavours.

Background: Current guidelines consider vitamin K antagonists (VKA) the oral anticoagulant agents of choice in adults with atrial arrhythmias (AA) and moderate or complex forms of congenital heart disease, significant valvular lesions, or bioprosthetic valves, pending safety data on non-VKA oral anticoagulants (NOACs). Therefore, the international NOTE registry was initiated to assess safety, change in adherence and quality of life (QoL) associated with NOACs in adults with congenital heart disease (ACHD)., Methods: An international multicenter prospective study of NOACs in ACHD was established. Follow-up occurred at 6 months and yearly thereafter. Primary endpoints were thromboembolism and major bleeding. Secondary endpoints included minor bleeding, change in therapy adherence (≥80% medication refill rate, ≥6 out of 8 on Morisky-8 questionnaire) and QoL (SF-36 questionnaire)., Results: In total, 530 ACHD patients (mean age 47 SD 15 years; 55% male) with predominantly moderate or complex defects (85%), significant valvular lesions (46%) and/or bioprosthetic valves (11%) using NOACs (rivaroxaban 43%; apixaban 39%; dabigatran 12%; edoxaban 7%) were enrolled. The most common indication was AA (91%). Over a median follow-up of 1.0 [IQR 0.0-2.0] year, thromboembolic event rate was 1.0% [95%CI 0.4-2.0] (n = 6) per year, with 1.1% [95%CI 0.5-2.2] (n = 7) annualized rate of major bleeding and 6.3% [95%CI 4.5-8.5] (n = 37) annualized rate of minor bleeding. Adherence was sufficient during 2 years follow-up in 80-93% of patients. At 1-year follow-up, among the subset of previous VKA-users who completed the survey (n = 33), QoL improved in 6 out of 8 domains (p ≪ 0.05)., Conclusions: Initial results from our worldwide prospective study suggest that NOACs are safe and may be effective for thromboembolic prevention in adults with heterogeneous forms of congenital heart disease., (Copyright © 2019 The Authors. Published by Elsevier B.V. All rights reserved.)

Background As patients with congenital heart disease (CHD) are living longer, understanding the comorbidities they develop as they age is increasingly important. However, there are no published population-based estimates of the comorbidity burden among the US adult patients with CHD. Methods and Results Using the IBM MarketScan commercial claims database from 2010 to 2016, we identified adults aged ≥18 years with CHD and 2 full years of continuous enrollment. These were frequency matched with adults without CHD within categories jointly defined by age, sex, and dates of enrollment in the database. A total of 40 127 patients with CHD met the inclusion criteria (mean [SD] age, 36.8 [14.6] years; and 48.2% were women). Adults with CHD were nearly twice as likely to have any comorbidity than those without CHD ( P <0.001). After adjusting for covariates, patients with CHD had a higher prevalence risk ratio for "previously recognized to be common in CHD" (risk ratio, 9.41; 95% CI, 7.99-11.1), "other cardiovascular" (risk ratio, 1.73; 95% CI, 1.66-1.80), and "noncardiovascular" (risk ratio, 1.47; 95% CI, 1.41-1.52) comorbidities. After adjusting for covariates and considering interaction with age, patients with severe CHD had higher risks of previously recognized to be common in CHD and lower risks of other cardiovascular comorbidities than age-stratified patients with nonsevere CHD. For noncardiovascular comorbidities, the risk was higher among patients with severe than nonsevere CHD before, but not after, the age of 40 years. Conclusions Our data underscore the unique clinical needs of adults with CHD compared with their peers. Clinicians caring for CHD may want to use a multidisciplinary approach, including building close collaborations with internists and specialists, to help provide appropriate care for the highly prevalent noncardiovascular comorbidities.

Background: Early detection of right ventricular dysfunction after transannular patch for tetralogy of Fallot (TOF-TAP) is essential for management., Objectives: To evaluate echocardiographic metrics of ventricular function correlate with functional MRI measurements, in patients with TOF-TAP., Methods: A retrospective review of patients with TOF-TAP between 2007 and 2017 who had an echocardiogram and MRI within six months were analyzed. Systolic to diastolic ratio (SD ratio) was measured from the tricuspid regurgitation and adjusted for heart rate. Tricuspid Annular Plane Systolic Excursion (TAPSE), Fractional Area Change (FAC), and shortening fraction (SF) were additionally measured. Echocardiographic measurements were correlated with MRI assessment of right ventricular ejection fraction (RVEF), left ventricular ejection fraction (LVEF), right ventricular end-diastolic volume index (RVEDi), and right to left ventricle volume ratio (RV/LV)., Results: Of the 53 patients (mean age 12.8 years) that met inclusion criteria, 45 (85%) had available TR jets for SD ratio analysis. The HR adjusted SD ratio negatively correlated with RVEF (r = -.359, P = .016), LVEF (r = -.317, P = .038) and positively with RV/LV EDV ratio (r = .347, P = .024). TAPSE, FAC, and SF measurements did not show significant correlation., Conclusion: In patients with TOF-TAP, there is a moderate negative correlation between heart rate adjusted SD ratio and MRI metrics of ventricular function, suggesting that decreased filling time is a marker for reduced right ventricular function. The SD ratio may be a useful echocardiographic tool for serial evaluation of in this population., (© 2019 Wiley Periodicals, Inc.)

Background: Thromboembolic events contribute greatly to morbidity and mortality following Fontan surgery for univentricular hearts., Objectives: This study sought to evaluate the effect of type of Fontan surgery on thromboembolic risk., Methods: A North American multicenter retrospective cohort study enrolled 522 patients with Fontan palliation consisting of an atriopulmonary connection (APC) (21.4%), lateral tunnel (LT) (41.8%), or extracardiac conduit (EC) (36.8%). Thromboembolic complications and new-onset atrial arrhythmia were reviewed and classified by a blinded adjudicating committee. Thromboembolic risk across surgical techniques was assessed by multivariable competing-risk survival regression., Results: Over a median follow-up of 11.6 years, 10- and 20-year freedom from Fontan conversion, transplantation, or death was 94.7% and 78.9%, respectively. New-onset atrial arrhythmias occurred in 4.4, 1.2, and 1.0 cases per 100 person-years with APC, LT, and EC, respectively. APC was associated with a 2.82-fold higher risk of developing atrial arrhythmias (p < 0.001), with no difference between LT and EC (p = 0.95). A total of 71 thromboembolic events, 32 systemic and 39 venous, occurred in 12.8% of subjects, for an overall incidence of 1.1%/year. In multivariable analyses, EC was independently associated with a lower risk of systemic (hazard ratio [HR]: 0.20 vs. LT; 95% confidence interval [CI]: 0.04 to 0.97) and combined (HR: 0.34 vs. LT; 95% CI: 0.13 to 0.91) thromboembolic events. A lower incidence of combined thromboembolic events was also observed with antiplatelet agents (HR: 0.54; 95% CI: 0.32 to 0.92) but not anticoagulation (p = 0.53)., Conclusions: The EC Fontan was independently associated with a lower thromboembolic risk after controlling for time-varying effects of atrial arrhythmias and thromboprophylaxis., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Patients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular magnetic resonance (CMR) and T1 relaxometry, reflect fibrotic remodeling and/or congestion in the liver. The aim of this study was to investigate whether pediatric patients with single ventricle physiology experience increased native T1 and ECV as markers of liver fibrosis/congestion., Methods: Hepatic native T1 times and ECV, using a cardiac short axis modified Look-Locker inversion recovery sequence displaying the liver, were measured retrospectively in children with BCPC- and Fontan circulations and compared to pediatric controls., Results: Hepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64 ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43 ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54 ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan., Conclusion: Fontan and BCPC patients have elevated CMR markers suggestive of hepatic fibrosis and/or congestion, even at a young age. The tissue changes do not appear to be related to central venous pressures., Trial Registration: Retrospectively registered data.

Background: No established reference-standard technique is available for ascending aortic diameter measurements. The aim of this study was to determine agreement between modalities and techniques., Methods: In patients with aortic pathology transthoracic echocardiography, computed tomography angiography (CTA) and magnetic resonance angiography (MRA) were performed. Aortic diameters were measured at the sinus of Valsalva (SoV), sinotubular junction (STJ) and tubular ascending aorta (TAA) during mid-systole and end-diastole. In echocardiography both the inner edge-to-inner edge (I-I edge) and leading edge-to‑leading edge (L-L edge) methods were applied, and the length of the aortic annulus to the most cranial visible part of the ascending aorta was measured. In CTA and MRA the I-I method was used., Results: Fifty patients with bicuspid aortic valve (36 ± 13 years, 26% female) and 50 Turner patients (35 ± 13 years) were included. Comparison of all aortic measurements showed a mean difference of 5.4 ± 2.7 mm for the SoV, 5.1 ± 2.0 mm for the STJ and 4.8 ± 2.1 mm for the TAA. The maximum difference was 18 mm. The best agreement was found between echocardiography L-L edge and CTA during mid-systole. CTA and MRA showed good agreement. A mean difference of 1.5 ± 1.3 mm and 1.8 ± 1.5 mm was demonstrated at the level of the STJ and TAA comparing mid-systolic with end-diastolic diameters. The visible length of the aorta increased on average 5.3 ± 5.1 mmW during mid-systole., Conclusions: MRA and CTA showed best agreement with L-L edge method by echocardiography. In individual patients large differences in ascending aortic diameter were demonstrated, warranting measurement standardization. The use of CTA or MRA is advised at least once., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Myocardial dysfunction has been implicated in gradual heart failure in transposition of the great arteries (TGA) with a systemic right ventricle (RV). Fibrosis can be assessed using the extracellular volume fraction (ECV). Our aim was to measure ECV and determine its associations with clinical findings and outcomes., Methods: We prospectively measured ECV in systemic RV subjects (either D-loop after atrial switch or L-loop) and healthy controls. T 1 measurements for a single mid-ventricular short-axis plane before and 3, 7, and 15 min after gadolinium contrast were used to quantify systemic ventricular ECV. Individuals with elevated ECV were compared to those without., Results: In 53 TGA subjects (age 34.6 ± 10.3 years, 41% female) the mean ECV for the systemic RV (28.7 ± 4.4%) was significantly higher than the left ventricle in 22 controls (26.1 ± 2.8%, P = 0.0104). Those with an elevated ECV (n = 15, 28.3%) had a higher b-type natriuretic peptide (BNP) (P < 0.011) and a longer 6-min walk distance (P = 0.021), but did not differ by age, arrhythmia history, ventricular volume, function, or circulating collagen byproducts. At follow-up (median 4.4 years), those experiencing major cardiovascular endpoints (new arrhythmia, arrhythmia device, heart failure hospitalization, listing for transplantation, mechanical support, or cardiovascular death, n = 14) had a higher ECV. ECV, age, and BNP were independent predictors of cardiac events in Cox-proportional hazard models., Conclusions: Myocardial fibrosis is common in the systemic RV and associated with a higher BNP. Elevated CMR-derived ECV was associated with adverse clinical outcome. The findings suggest a role of diffuse myocardial fibrosis in clinical deterioration of the systemic RV., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Owing to significant medical advances, it is now estimated that more than 90% of persons born with congenital heart disease (CHD) will reach adulthood. Medically appropriate physical activity represents an opportunity to improve physical functioning as well as quality of life and psychosocial outcomes. By reviewing published CHD research and clinical recommendations, herein we first summarize how adults with CHD are known to be less physically active and have reduced exercise capacity compared with healthy peers. Cardiopulmonary exercise testing is important for routine clinical management and before the onset of an exercise program. Physiological anomalies are common in adults with CHD, although very few necessitate activity restrictions, and positive results from exercise training have been demonstrated. In recent decades, the focus has thus shifted from restriction of exercise to promotion of exercise. Adults with CHD also face unique psychosocial challenges associated with living with a chronic cardiac condition, many of which may influence exercise behaviours. However, much less is known about participation of adults with CHD in cardiac rehabilitation (CR) programs, which differ from exercise training in their comprehensive, interdisciplinary approach to management of chronic disease and that might be uniquely poised to meet the physical and psychosocial needs of adults with CHD. Initial CR outcomes have been positive and with no reported adverse events. This review summarizes the unique physical and psychosocial considerations that may guide the provision of CR to adults with CHD., (Copyright © 2018 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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.

Background: Administrative data sets utilize billing codes for research and quality assessment. Previous data suggest that such codes can accurately identify adults with congenital heart disease (CHD) in the cardiology clinic, but their use has yet to be validated in a larger population., Methods and Results: All administrative codes from an entire health system were queried for a single year. Adults with a CHD diagnosis code ( International Classification of Diseases, Ninth Revision , ( ICD-9 ) codes 745-747) defined the cohort. A previously validated hierarchical algorithm was used to identify diagnoses and classify patients. All charts were reviewed to determine a gold standard diagnosis, and comparisons were made to determine accuracy. Of 2399 individuals identified, 206 had no CHD by the algorithm or were deemed to have an uncertain diagnosis after provider review. Of the remaining 2193, only 1069 had a confirmed CHD diagnosis, yielding overall accuracy of 48.7% (95% confidence interval, 47-51%). When limited to those with moderate or complex disease (n=484), accuracy was 77% (95% confidence interval, 74-81%). Among those with CHD, misclassification occurred in 23%. The discriminative ability of the hierarchical algorithm (C statistic: 0.79; 95% confidence interval, 0.77-0.80) improved further with the addition of age, encounter type, and provider (C statistic: 0.89; 95% confidence interval, 0.88-0.90)., Conclusions: ICD codes from an entire healthcare system were frequently erroneous in detecting and classifying CHD patients. Accuracy was higher for those with moderate or complex disease or when coupled with other data. These findings should be taken into account in future studies utilizing administrative data sets in CHD., (© 2018 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.)

Background: In roughly half of patients with coarctation of the aorta (CoA), the aorta may be enlarged. It is uncertain whether enlargement is independent of aortic valve morphology. We sought to compare aortic size in CoA with a tricuspid valve (TAV) to those with bicuspid aortic valve (BAV)., Methods: Sixty-eight CoA patients and 20 healthy controls with prior cardiac magnetic resonance (CMR) imaging were included. CMR was retrospectively reanalyzed to measure aortic root and mid-ascending aorta. The maximum aortic diameter was compared between CoA with TAV, CoA with BAV, and control groups., Results: CoA with TAV patients (n=27) had smaller aortic root diameters than CoA with BAV (n=41) (32±4.9 vs. 37±5.8mm, p=0.001), despite being older (40 vs. 32years, p=0.01). Similarly, TAV CoA patients had a smaller mid-ascending aortic diameter (28±4.5 vs. 33±6.9mm, p=0.019) than BAV patients. TAV CoA was similar to controls in all metrics. Twenty-four patients (35%) with CoA had dilated aortas (>37mm), of which 79% had BAV. A history of hypertension did not predict larger aortic root or mid-ascending aortic dimensions., Conclusions: In patients with CoA, TAV is associated with smaller aortic size compared to those with BAV, and similar to healthy controls. Aortic size in CoA is independent of hypertension. Therefore, aortopathy associated with BAV is likely a reflection of the BAV phenotype rather than CoA or its physiologic effects. This distinction may have implications for the frequency and types of monitoring and treatment of CoA patients., (Copyright © 2017 Elsevier B.V. All rights reserved.)