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The relationship between smoking and illness perceptions among congenital heart disease (CHD) survivors is unknown. The primary aims of the present study were to compare the smoking prevalence among CHD survivors to a nationally representative U.S. sample and examine the relationship between smoking and illness perceptions. CHD survivors (N = 744) from six U.S. sites participated in the study. The smoking prevalence among CHD survivors (9.3%) was lower than the general population (15.3%). However, 23.3% of CHD survivors with severe functional limitations smoked. Smoking prevalence differed by U.S. region, with a greater proportion of those attending CHD care in the Midwest reporting smoking (11.8%). The illness perception dimensions of Concern and Emotional Response were independently associated with smoking. Differences in illness perceptions enhance our understanding of smoking among CHD survivors and may guide interventions promoting positive health behaviors. The protocol for the study from which the present analyses were conducted was recorded at ClinicalTrials.gov: NCT02150603. ispartof: JOURNAL OF BEHAVIORAL MEDICINE vol:44 issue:6 pages:772-783 ispartof: location:United States status: published

BACKGROUND: Atrial arrhythmias (ie, intra-atrial reentrant tachycardia and atrial fibrillation) are a leading cause of morbidity and hospitalization in adults with congenital heart disease (CHD). Little is known about their effect on quality of life and other patient-reported outcomes (PROs) in adults with CHD. OBJECTIVE: The purpose of this study was to assess the impact of atrial arrhythmias on PROs in adults with CHD and explore geographic variations. METHODS: Associations between atrial arrhythmias and PROs were assessed in a cross-sectional study of adults with CHD from 15 countries spanning 5 continents. A propensity-based matching weight analysis was performed to compare quality of life, perceived health status, psychological distress, sense of coherence, and illness perception in patients with and those without atrial arrhythmias. RESULTS: A total of 4028 adults with CHD were enrolled, 707 (17.6%) of whom had atrial arrhythmias. After applying matching weights, patients with and those without atrial arrhythmias were comparable with regard to age (mean 40.1 vs 40.2 years), demographic variables (52.5% vs 52.2% women), and complexity of CHD (15.9% simple, 44.8% moderate, and 39.2% complex in both groups). Patients with atrial arrhythmias had significantly worse PRO scores with respect to quality of life, perceived health status, psychological distress (ie, depression), and illness perception. A summary score that combines all PRO measures was significantly lower in patients with atrial arrhythmias (-3.3%; P = .0006). Differences in PROs were consistent across geographic regions. CONCLUSION: Atrial arrhythmias in adults with CHD are associated with an adverse impact on a broad range of PROs consistently across various geographic regions. ispartof: HEART RHYTHM vol:18 issue:5 pages:793-800 ispartof: location:United States status: published

In this international study, we (1) compared patient-reported outcomes (PROs) in adults with congenital heart disease (CHD) who had versus had not been hospitalized during the previous 12 month, (2) contrasted PROs in patients who had been hospitalized for cardiac surgery versus nonsurgical reasons, (3) assessed the magnitude of differences between the groups (i.e., effect sizes), and (4) explored differential effect sizes between countries. APPROACH-IS was a cross-sectional, observational study that enrolled 4,028 patients from 15 countries (median age 32 years; 53% females). Self-report questionnaires were administered to measure PROs: health status; anxiety and depression; and quality of life. Overall, 668 patients (17%) had been hospitalized in the previous 12 months. These patients reported poorer outcomes on all PROs, with the exception of anxiety. Patients who underwent cardiac surgery demonstrated a better quality of life compared with those who were hospitalized for nonsurgical reasons. For significant differences, the effect sizes were small, whereas they were negligible in nonsignificant comparisons. Substantial intercountry differences were observed. For various PROs, moderate to large effect sizes were found comparing different countries. In conclusion, adults with CHD who had undergone hospitalization in the previous year had poorer PROs than those who were medically stable. Researchers ought to account for the timing of recruitment when conducting PRO research as hospitalization can impact results. ispartof: AMERICAN JOURNAL OF CARDIOLOGY vol:145 pages:135-142 ispartof: location:United States status: published

The landscape of congenital heart disease has changed rapidly over the past few decades. The shift from pediatric to adult congenital heart disease care has stretched resources and the ability to provide high-quality access and delivery of care for the more than 1.5 million adults with congenital heart disease in the United States. Meeting the demand for delivering high-quality care requires a team-based approach, with each member highly specialized. This review describes the deficits and deficiencies in providing care for adults with congenital heart disease in the United States and a team-based approach to improving access and delivery of care.

Bicuspid aortic valve (BAV) is one of the most common congenital heart defects in children, adolescents and adults. BAV can occur as an isolated lesion or in association with other congenital heart defects, such as coarctation of the aorta or genetic syndromes like Turner syndrome. Although the majority of long term complications associated with BAV manifest later in life, children and adolescents may present with early onset valvular dysfunction or dilation of the aorta. BAV is a heterogeneous disease with a wide array of presentations at various ages, depending on the degree of aortic valve dysfunction, aortic dilation and presence of associated lesions. Aortic valve stenosis and/or regurgitation are the primary indications for intervention in children and adolescents with BAV. Although a majority of young patients with BAV also have some aortic dilation, interventions on the aorta are very rare during this time frame. Children and adolescents with BAV benefit from comprehensive assessment of their risk profile to determine follow-up surveillance intervals, sports recommendations, and timing of surgical intervention. The morphologic phenotype of BAV is important to identify, as it may predict future complications and prognosis.

James A. Arrighi, MD, FACC, Chair Lisa A. Mendes, MD, FACC, Co-Chair Jesse E. Adams iii, MD, FACC[∗][1] John E. Brush, Jr, MD, FACC[∗][1] G. William Dec, Jr, MD, FACC Ali Denktas, MD, FACC Susan M. Fernandes, LPD, PA-C Sanjeev A. Francis, MD, FACC Rosario Freeman, MD, MS, FACC[∗][1

BACKGROUND: Implantable cardioverter-defibrillators (ICDs) are increasingly being used to prevent sudden death in the growing population of adults with congenital heart disease (CHD). However, little is known about their impact on patient-reported outcomes (PROs). OBJECTIVE: The purpose of this study was to assess and compare PROs in adults with CHD with and without ICDs. METHODS: A propensity-based matching weight analysis was conducted to evaluate PROs in an international cross-sectional study of adults with CHD from 15 countries across 5 continents. RESULTS: A total of 3188 patients were included: 107 with ICDs and 3081 weight-matched controls without ICDs. ICD recipients were an average age of 40.1 ± 12.4 years, and >95% had moderate or complex CHD. Defibrillators were implanted for primary and secondary prevention in 38.3% and 61.7%, respectively. Perceived health status, psychological distress, sense of coherence, and health behaviors did not differ significantly among patients with and without ICDs. However, ICD recipients had a more threatening view of their illness (relative % difference 8.56; P = .011). Those with secondary compared to primary prevention indications had a significantly lower quality-of-life score (Linear Analogue Scale 72.0 ± 23.1 vs 79.2 ± 13.0; P = .047). Marked geographic variations were observed. Overall sense of well-being, assessed by a summary score that combines various PROs, was significantly lower in ICD recipients (vs controls) from Switzerland, Argentina, Taiwan, and the United States. CONCLUSION: In an international cohort of adults with CHD, ICDs were associated with a more threatening illness perception, with a lower quality of life in those with secondary compared to primary prevention indications. However, marked geographic variability in PROs was observed. ispartof: Heart Rhythm vol:17 issue:5 pages:1-9 ispartof: location:United States status: Published online

Background Health behaviours are essential to maintain optimal health and reduce the risk of cardiovascular complications in adults with congenital heart disease. This study aimed to describe health behaviours in adults with congenital heart disease in 15 countries and to identify patient characteristics associated with optimal health behaviours in the international sample. Design This was a cross-sectional observational study. Methods Adults with congenital heart disease ( n = 4028, median age = 32 years, interquartile range 25–42 years) completed self-report measures as part of the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS). Participants reported on seven health behaviours using the Health Behaviors Scale-Congenital Heart Disease. Demographic and medical characteristics were assessed via medical chart review and self-report. Multivariate path analyses with inverse sampling weights were used to investigate study aims. Results Health behaviour rates for the full sample were 10% binge drinking, 12% cigarette smoking, 6% recreational drug use, 72% annual dental visit, 69% twice daily tooth brushing, 27% daily dental flossing and 43% sport participation. Pairwise comparisons indicated that rates differed between countries. Rates of substance use behaviours were higher in younger, male participants. Optimal dental health behaviours were more common among older, female participants with higher educational attainment while sports participation was more frequent among participants who were younger, male, married, employed/students, with higher educational attainment, less complex anatomical defects and better functional status. Conclusions Health behaviour rates vary by country. Predictors of health behaviours may reflect larger geographic trends. Our findings have implications for the development and implementation of programmes for the assessment and promotion of optimal health behaviours in adults with congenital heart disease.

Thromboembolic events contribute greatly to morbidity and mortality following Fontan surgery for univentricular hearts.This study sought to evaluate the effect of type of Fontan surgery on thromboembolic risk.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.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).The EC Fontan was independently associated with a lower thromboembolic risk after controlling for time-varying effects of atrial arrhythmias and thromboprophylaxis.

Patients with a Fontan circulation are at risk of a sedentary lifestyle. Given the direct relationship between physical activity and health, promotion of physical activity has the potential to improve outcomes, including quality of life (QOL). This study aimed to describe self-reported physical activity levels in adult Fontan patients and examine associations between physical activity, perceived health status and QOL. The sample consisted of 177 Fontan patients (Mage = 27.5 ± 7.6 years, 52% male) who reported their physical activity, perceived health status, and QOL as part of the cross-sectional Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study. Descriptive statistics and univariate analyses of variance with planned contrasts were computed to describe physical activity characteristics. Mediation analyses tested whether perceived health status variables mediated the association between physical activity and QOL. Forty-six percent of patients were sedentary while only 40% met international physical activity guidelines. Higher physical activity was associated with younger age, lower NYHA class, higher perceived general health, and greater QOL. Patients who commuted by walking and engaged in sports reported better perceived health and QOL. Mediation analyses revealed that perceived general health but not NYHA functional class mediated the association between physical activity and QOL (αβ = 0.22, 95% confidence interval = 0.04 to 0.49). In conclusion, Fontan patients likely benefit from regular physical activity, having both higher perceived general health and functional capacity; greater perceived health status may contribute to enhanced QOL. In conclusion, these data support the pivotal role of regular physical activity for Fontan patients. ispartof: AMERICAN JOURNAL OF CARDIOLOGY vol:124 issue:1 pages:144-150 ispartof: location:United States status: published

Background: Although lower-complexity cardiac malformations constitute the majority of adult congenital heart disease (ACHD), the long-term risks of adverse cardiovascular events and relationship with conventional risk factors in this population are poorly understood. We aimed to quantify the risk of adverse cardiovascular events associated with lower-complexity ACHD that is unmeasured by conventional risk factors. Methods: A multitiered classification algorithm was used to select individuals with lower-complexity ACHD and individuals without ACHD for comparison among >500 000 British adults in the UK Biobank. ACHD diagnoses were subclassified as isolated aortic valve and noncomplex defects. Time-to-event analyses were conducted for the primary end points of fatal or nonfatal acute coronary syndrome, ischemic stroke, heart failure, and atrial fibrillation and a secondary combined end point for major adverse cardiovascular events. Maximum follow-up time for the study period was 22 years with retrospectively and prospectively collected data from the UK Biobank. Results: We identified 2006 individuals with lower-complexity ACHD and 497 983 unexposed individuals in the UK Biobank (median age at enrollment, 58 [interquartile range, 51–63] years). Of the ACHD-exposed group, 59% were male, 51% were current or former smokers, 30% were obese, and 69%, 41%, and 7% were diagnosed or treated for hypertension, hyperlipidemia, and diabetes mellitus, respectively. After adjustment for 12 measured cardiovascular risk factors, ACHD remained strongly associated with the primary end points, with hazard ratios ranging from 2.0 (95% CI, 1.5–2.8; P P Conclusions: Individuals with lower-complexity ACHD had a higher burden of adverse cardiovascular events relative to the general population that was unaccounted for by conventional cardiovascular risk factors. These findings highlight the need for closer surveillance of patients with mild to moderate ACHD and further investigation into management and mechanisms of cardiovascular risk unique to this growing population of high-risk adults.

Background: Physical activity is important to maintain and promote health. This is of particular interest in patients with congenital heart disease (CHD) where acquired heart disease should be prevented. The World Health Organization (WHO) recommends a minimum of 2.5 h/week of physical activity exceeding 3 metabolic equivalents (METS) to achieve positive health effects. It is unknown whether physical activity levels (PAL) in adult CHD patients differ by country of origin. Methods: 3896 adults with CHD recruited from 15 countries over 5 continents completed self-reported instruments, including the Health Behaviour Scale (HBS-CHD), within the APPROACH-IS project. For each patient, we calculated whether WHO recommendations were achieved or not. Associated factors were investigated using Generalized Linear Mixed Models. Results: On average, 31% reached the WHO recommendations but with a great variation between geographical areas (India: 10%–Norway: 53%). Predictors for physical activity level in line with the WHO recommendations, with country of residence as random effect, were male sex (OR 1.78, 95%CI 1.52–2.08), NYHA-class I (OR 3.10, 95%CI 1.71–5.62) and less complex disease (OR 1.46, 95%CI 1.16–1.83). In contrast, older age (OR 0.97, 95%CI 0.96–0.98), lower educational level (OR 0.41, 95%CI 0.26–0.64) and being unemployed (OR 0.57, 95%CI 0.42–0.77) were negatively associated with reaching WHO recommendations. Conclusions: A significant proportion of patients with CHD did not reach the WHO physical activity recommendations. There was a large variation in physical activity level by country of origin. Based on identified predictors, vulnerable patients may be identified and offered specific behavioral interventions. Keywords: Adult congenital heart disease, Physical activity level, Patient-reported outcome, Health-behaviour scale, Physical activity recommendation, Metabolic equivalent

Objective To derive cluster analysis-based groupings for adults with congenital heart disease (ACHD) when it comes to perceived health, psychological functioning, health behaviours and quality of life (QoL). Methods This study was part of a larger worldwide multicentre study called APPROACH-IS; a cross sectional study which recruited 4028 patients (2013–2015) from 15 participating countries. A hierarchical cluster analysis was performed using Ward's method in order to group patients with similar psychological characteristics, which were defined by taking into consideration the scores of the following tests: Sense Of Coherence, Health Behavior Scale (physical exercise score), Hospital Anxiety Depression Scale, Illness Perception Questionnaire, Satisfaction with Life Scale and the Visual Analogue Scale scores of the EQ-5D perceived health scale and a linear analogue scale (0–100) measuring QoL. Results 3768 patients with complete data were divided into 3 clusters. The first and second clusters represented 89.6% of patients in the analysis who reported a good health perception, QoL, psychological functioning and the greatest amount of exercise. Patients in the third cluster reported substantially lower scores in all PROs. This cluster was characterised by a significantly higher proportion of females, a higher average age the lowest education level, more complex forms of congenital heart disease and more medical comorbidities. Conclusions This study suggests that certain demographic and clinical characteristics may be linked to less favourable health perception, quality of life, psychological functioning, and health behaviours in ACHD. This information may be used to improve psychosocial screening and the timely provision of psychosocial care.

Background: Advances in the management of patients with congenital heart disease (CHD) have made survival to adulthood an expectation. Although the literature is limited, existing evidence suggests that adult co-morbidities may be at least as prevalent in patients with CHD, if not more so, than in an age-matched general population. The presence of these co-morbidities in combination with underlying CHD likely places these patients at increased risk for early mortality. Thus, we sought to determine the predicted 10-year mortality risk in a cohort of adults with CHD. Methods: In this single center study, we administered a survey to patients ≥18 years of age with CHD to assess their 10-year mortality risk. Results: A total of 406 patients agreed to participate. The median age was 41.8 ± 15.3 years, and 58% were female. The average predicted 10-year mortality for the CHD cohort was 13.7 ± 15.8%. For CHD patients ≥50 years of age, the average 10-year mortality risk was 29.8 ± 3.0% compared to 26.1% ± 0.2% in the general population (p

Background We compared physical functioning, mental health, and quality of life (QoL) of patients with different subtypes of congenital heart disease (CHD) in a large international sample and investigated the role of functional class in explaining the variance in outcomes across heart defects. Methods In the cross-sectional Assessment of Patterns of Patient-Reported Outcome in Adults with Congenital Heart Disease-International Study (APPROACH-IS), we enrolled 4028 adult patients with CHD from 15 countries. Diagnostic groups with at least 50 patients were included in these analyses, yielding a sample of 3538 patients (median age: 32 years; 52% women). Physical functioning, mental health, and QoL were measured with the SF-12 health status survey, Hospital Anxiety and Depression Scale (HADS), linear analog scale (LAS) and Satisfaction with Life Scale, respectively. Functional class was assessed using the patient-reported New York Heart Association (NYHA) class. Multivariable general linear mixed models were applied to assess the relationship between the type of CHD and patient-reported outcomes, adjusted for patient characteristics, and with country as random effect. Results Patients with coarctation of the aorta and those with isolated aortic valve disease reported the best physical functioning, mental health, and QoL. Patients with cyanotic heart disease or Eisenmenger syndrome had worst outcomes. The differences were statistically significant, above and beyond other patient characteristics. However, the explained variances were small (0.6% to 4.1%) and decreased further when functional status was added to the models (0.4% to 0.9%). Conclusions Some types of CHD predict worse patient-reported outcomes. However, it appears that it is the functional status associated with the heart defect rather than the heart defect itself that shapes the outcomes.

Background Patients with adult congenital heart disease (ACHD) have many risk factors for chronic pain such as prior cardiac interventions and adult comorbidities. However, the prevalence of chronic pain has not been well described in this population. We sought to determine the prevalence of pain in a large international cohort of patients with ACHD. Methods Data from the APPROACH-IS dataset was utilized for this study which includes 4028 patients with ACHD from 15 different countries. The prevalence of pain was assessed under the health status patient reported outcome domain utilizing the EuroQol-5D 3 level version tool. Multivariable logistic regression was used to assess differences across countries in pain, taking into account country-level random effects for clustering across observations within each country. Results A total of 3832 patients with ACHD met the study criteria, median age 32 years [IQR 25, 42], 52.6% females. The prevalence of at least moderate pain was reported by 28.9% (95% CO 27.5 ​= ​30.3%) of participants. Pain was associated with country of origin, age, gender, background, education and marital status as well as several clinical variables including disease complexity, cardiac device presence, history of heart failure, psychiatric conditions and presence of other medical conditions. Those with pain had lower levels of perceived health and a lower quality of life score. Conclusion Pain in patients with ACHD is common, impacting nearly one-third of patients. Given the far reaching implications of pain in patients with ACHD, further study of pain characteristics and treatment management appear warranted.

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 CHA2DS2-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.

Background: Occasionally patients with congenitally corrected transposition of the great arteries (ccTGA) exhibit little clinical evidence of cardiovascular limitation even to their 8th decade. We aimed to assess survival prospects in a large cohort of ccTGA adults. Methods & Results: We included 555 ccTGA adults (median age 33.0 years, 48.3% female) under regular follow-up at 28 institutions between 2002 and 2019. The primary outcome was a composite of death, mechanical circulatory support (MCS) and heart transplant. During a median follow-up of 8.1 [IQR 4.4 - 13.3] years, 56 (10.1%) patients died, 10 (1.8%) patients underwent MCS and 14 (2.5%) had a heart transplant. Median age at time of primary outcome was 51.1 [IQR 37.5 - 63.2] years and cumulative incidence at 15 years from baseline was 21.5% [95% CI 16.1 - 26.5]. Leading causes of death were worsening of heart failure (43%) and sudden death (10%). Patients who died were more likely to use heart failure (HF) medications. In multivariable Cox analyses for baseline variables, age, prior atrial arrhythmia and HF admission were each associated with an increased risk of the primary outcome. Figure shows cumulative incidence according to history of atrial arrhythmia. During follow-up, 91 (16.4%) were admitted for HF, pacemaker implantation was performed in 68 (12.3%) patients, ICD in 82 (14.7%), and major cardiac surgery (mostly for systemic AV-valve) in 89 (15.8%) patients. Conclusion: In this large cohort of ccTGA adults, survival seemed to be primarily determined by heart failure-related complications. Prior atrial arrhythmia also seems to be a harbinger for adverse outcome. Few patients underwent advanced HF therapies. Figure: Cumulative incidence of the composite primary outcome (MCS, heart transplant or death) over a period of 14 years from first visit at an adult congenital heart disease clinic since 2002 stratified according to history of atrial arrhythmia. Shading represents upper and lower 95% confidence limits.

Introduction: Current research endorses regionalizing the care of infants with critical congenital heart disease (CCHD) to facilities with high levels of obstetrical, neonatal, and surgical volumes. California has a regionalization model that designates these facilities as accredited pediatric cardiac centers (PCCs). The impact of this system on the location of birth and cardiac surgeries for CCHD and for different social groups remains unexplored. Hypothesis: We hypothesize, there has been a trend toward increased accredited PCC utilization for birth and surgical intervention for CCHD in California with accompanying widening in social inequality. Methods: A retrospective, population-based analysis of CCHD hospitalizations between 1984-2018 using the California Office of Statewide Health Planning and Development’s discharge database was conducted. Demographic data and trends in location of birth and surgical procedures for CCHD from delivery to age 1 were evaluated in relation to hospital designation as an accredited PCC. Results: There were 15,091 live births with CCHD and 29,466 surgical admissions meeting inclusion criteria. Births at accredited PCCs increased from 17% in 1984 to 41% in 2018 while surgical admissions were largely above 80%. From 2015-2018, infants with CCHD, born outside an accredited PCC were more likely to have private HMO insurance and live 6-20 miles away. Patients with surgical admission to an accredited PCC were more likely to have private HMO insurance and reside in a zip code where the median income is >2x the federal poverty level. Conclusions: Significant gains have been made in the regionalization of care for infants with CCHD in California over 30 years. However, a substantial number of infants with CCHD do not receive care in accredited PCCs and social disparities in utilization remain. Increased efforts to reduce barriers to access, particularly those related to payer type and resident distance from PCCs are needed.

Background: Although several factors have been cited for risk stratification in patients with simple transposition of the great arteries (dTGA), no single predictor emerges consistently. We aimed to assess survival and determine factors associated with survival in a large cohort of dTGA adults with atrial switch. Methods and Results: We included 1,169 dTGA adults (median age 28.1 years, 38.7% female) under regular follow-up at 28 institutions between 2002 and 2019. The primary outcome was a composite of death, mechanical circulatory support (MCS) and heart transplant. During a median follow-up of 9.2 [IQR 5.5-14.2] years, 67 (5.7%) patients died, six (0.5%) patients underwent MCS and 21 (1.8%) had a heart transplant. Cumulative incidence of composite endpoint at 15 years was 12.8% [95% CI 9.8 - 15.7], see Figure). Median age at time of primary outcome was 39.5 [IQR 33.9 - 45.1] years. Leading causes of death were worsening of heart failure (34%), non-cardiac (21%) and sudden unexplained death (12%). In multivariable Cox analyses for baseline variables, age, VSD, ventricular arrhythmia and heart failure admission were each associated with increased risk of the outcome, whereas prior pacemaker (26% of patients) was not. New pacemaker implantation was performed in 107 (9.1%), ICD in 109 (9.3%), and cardiac surgery in 35 (3%) patients. Patients who died were more likely to develop arrhythmias, be admitted for heart failure or require surgery during follow-up. Conclusion: In this large contemporary cohort of dTGA adults after atrial switch, late survival was excellent and seemed to be determined by arrhythmia and heart failure-related complications. Few patients underwent advanced heart failure therapies. Figure. Cumulative incidence of the composite primary outcome (MCS, heart transplant or death) over a period of 15 years from first visit at an adult congenital heart disease clinic since 2002. Shading represents upper and lower 95% confidence limits.

Background The relationship between healthcare system inputs (e.g., human resources and infrastructure) and mortality has been extensively studied. However, the association between healthcare system inputs and patient-reported outcomes remains unclear. Hence, we explored the predictive value of human resources and infrastructures of the countries’ healthcare system on patient-reported outcomes in adults with congenital heart disease. Methods This cross-sectional study included 3588 patients with congenital heart disease (median age = 31y; IQR = 16.0; 52% women; 26% simple, 49% moderate, and 25% complex defects) from 15 countries. The following patient-reported outcomes were measured: perceived physical and mental health, psychological distress, health behaviors, and quality of life. The assessed inputs of the healthcare system were: (i) human resources (i.e., density of physicians and nurses, both per 1000 people) and (ii) infrastructure (i.e., density of hospital beds per 10,000 people). Univariable, multivariable, and sensitivity analyses using general linear mixed models were conducted, adjusting for patient-specific variables and unmeasured country differences. Results Sensitivity analyses showed that higher density of physicians was significantly associated with better self-reported physical and mental health, less psychological distress, and better quality of life. A greater number of nurses was significantly associated with better self-reported physical health, less psychological distress, and less risky health behavior. No associations between a higher density of hospital beds and patient-reported outcomes were observed. Conclusions This explorative study suggests that density of human resources for health, measured on country level, are associated with patient-reported outcomes in adults with congenital heart disease. More research needs to be conducted before firm conclusions about the relationships observed can be drawn. Trial registration ClinicalTrials.gov: NCT02150603. Registered 30 May 2014

Background Previous studies have found that sense of coherence (SOC) is positively related to quality of life (QoL) in persons with chronic conditions. In congenital heart disease (CHD), the evidence is scant. Aims We investigated (i) intercountry variation in SOC in a large international sample of adults with CHD; (ii) the relationship between demographic and clinical characteristics and SOC; (iii) the relationship between cultural dimensions of countries and SOC; and (iv) variation in relative importance of SOC in explaining QoL across the countries. Methods APPROACH-IS was a cross-sectional, observational study, with 4028 patients from 15 countries enrolled. SOC was measured using the 13-item SOC scale (range 13–91) and QoL was assessed by a linear analog scale (range 0–100). Results The mean SOC score was 65.5±13.2. Large intercountry variation was observed with the strongest SOC in Switzerland (68.8±11.1) and the lowest SOC in Japan (59.9±14.5). A lower SOC was associated with a younger age; lower educational level; with job seeking, being unemployed or disabled; unmarried, divorced or widowed; from a worse functional class; and simple CHD. Power distance index and individualism vs collectivism were cultural dimensions significantly related to SOC. SOC was positively associated with QoL in all participating countries and in the total sample, with an explained variance ranging from 5.8% in Argentina to 30.4% in Japan. Conclusion In adults with CHD, SOC is positively associated with QoL. The implementation of SOC-enhancing interventions might improve QoL, but strategies would likely differ across countries given the substantial variation in explained variance.

Objective: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL. Design: Cross-sectional observational study. Setting: Twenty-four cardiology centers from 15 countries across five continents. Patients: Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years. Outcome Measures: QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions Questionnaire, respectively. Results: Patients with a Fontan circulation reported lower QOL (Wald Z = −3.59, p =

Aims: Geographical differences in patient-reported outcomes (PROs) of adults with congenital heart disease (ConHD) have been observed, but are poorly understood. We aimed to: (1) investigate inter-country variation in PROs in adults with ConHD; (2) identify patient-related predictors of PROs; and (3) explore standard of living and healthcare system characteristics as predictors of PROs. Methods and results: Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS) was a cross-sectional, observational study, in which 4028 patients from 15 countries in 5 continents were enrolled. Self-report questionnaires were administered: patient-reported health (12-item Short Form Health Survey; EuroQOL-5D Visual Analog Scale); psychological functioning (Hospital Anxiety and Depression Scale); health behaviors (Health Behavior Scale-Congenital Heart Disease) and quality of life (Linear Analog Scale for quality of life; Satisfaction With Life Scale). A composite PRO score was calculated. Standard of living was expressed as Gross Domestic Product per capita and Human Development Index. Healthcare systems were operationalized as the total health expenditure per capita and the overall health system performance. Substantial inter-country variation in PROs was observed, with Switzerland having the highest composite PRO score (81.0) and India the lowest (71.3). Functional class, age, and unemployment status were patient-related factors that independently and consistently predicted PROs. Standard of living and healthcare system characteristics predicted PROs above and beyond patient characteristics. Conclusions: This international collaboration allowed us to determine that PROs in ConHD vary as a function of patient-related factors as well as the countries in which patients live. (C) 2017 Elsevier B.V. All rights reserved

Background Atrial arrhythmias are the most common complication encountered in the growing and aging population with congenital heart disease. Objectives This study sought to assess the types and patterns of atrial arrhythmias, associated factors, and age-related trends. Methods A multicenter cohort study enrolled 482 patients with congenital heart disease and atrial arrhythmias, age 32.0 ± 18.0 years, 45.2% female, from 12 North American centers. Qualifying arrhythmias were classified by a blinded adjudicating committee. Results The most common presenting arrhythmia was intra-atrial re-entrant tachycardia (IART) (61.6%), followed by atrial fibrillation (28.8%), and focal atrial tachycardia (9.5%). The proportion of arrhythmias due to IART increased with congenital heart disease complexity from 47.2% to 62.1% to 67.0% in patients with simple, moderate, and complex defects, respectively (p = 0.0013). Atrial fibrillation increased with age to surpass IART as the most common arrhythmia in those ≥50 years of age (51.2% vs. 44.2%; p Conclusions IART is the most common presenting atrial arrhythmia in patients with congenital heart disease, with a predominantly paroxysmal pattern. However, atrial fibrillation increases in prevalence and atrial arrhythmias progressively become permanent as the population ages.

Understanding the contraceptive practices of women with complex congenital heart disease (CHD) and providing them individualized contraception counseling may prevent adverse events and unplanned high-risk pregnancies. Given this, we sought to examine the contraceptive practices in women with CHD, describe adverse events associated with contraceptive use, and describe the provision of contraception counseling. Women >18 years were recruited from 2011 to 2014 from 9 adult CHD (ACHD) centers throughout North America. Subjects completed a 48-item questionnaire regarding contraceptive use and perceptions of contraception counseling, and a medical record review was performed. Of 505 subjects, median age was 33 (interquartile range 26 to 44) and 81% had CHD of moderate or great complexity. The majority (86%, 435 of 505) of the cohort had used contraception. The types included barrier methods (87%), oral contraception (OC) 84%, intrauterine device (18%), Depo-Provera (15%), vaginal ring (7%), patch (6%), hormonal implant (2%), Plan B (19%), and sterilization (16%). Overall OC use was not significantly different by CHD complexity. Women with CHD of great complexity were more likely to report a thrombotic event while taking OC than those with less complex CHD (9% vs 1%, p = 0.003). Contraception counseling by the ACHD team was noted by 43% of subjects. Unplanned pregnancy was reported by 25% with no statistical difference by CHD complexity. In conclusion, contraceptive practices of women with complex CHD are highly variable, and the prevalence of blood clots while taking OC is not insignificant while provision of contraception counseling by ACHD providers appears lacking. publisher: Elsevier articletitle: Contraceptive Practices of Women With Complex Congenital Heart Disease journaltitle: The American Journal of Cardiology articlelink: http://dx.doi.org/10.1016/j.amjcard.2016.11.047 content_type: article copyright: © 2016 Elsevier Inc. All rights reserved. ispartof: American Journal of Cardiology vol:119 issue:6 pages:911-915 ispartof: location:United States status: published

BACKGROUND: The prevalence and effects of cigarette smoking and cannabis use in persons with congenital heart disease (CHD) are poorly understood. We (1) described the prevalence of cigarette smoking, cannabis consumption, and co-use in adults with CHD; (2) investigated intercountry differences; (3) tested the relative effects on physical functioning, mental health, and quality of life (QOL); and (4) quantified the differential effect of cigarette smoking, cannabis use, or co-use on those outcomes. METHODS: APPROACH-IS was a cross-sectional study, including 4028 adults with CHD from 15 countries. Patients completed questionnaires to measure physical functioning, mental health, and QOL. Smoking status and cannabis use were assessed by means of the Health Behaviour Scale-Congenital Heart Disease. Linear models with doubly robust estimations were computed after groups were balanced with the use of propensity weighting. RESULTS: Overall, 14% of men and 11% of women smoked cigarettes only; 8% of men and 4% of women consumed cannabis only; and 4% of men and 1% of women used both substances. Large intercountry variations were observed, with Switzerland having the highest prevalence for smoking cigarettes (24% of men, 19% of women) and Canada the highest for cannabis use (19% of men, 4% of women). Cigarette smoking had a small negative effect on patient-reported outcomes, and the effect of cannabis was negligible. The effect of co-use was more prominent, with a moderate negative effect on mental health. CONCLUSIONS: We found significant intercountry variability in cigarette and cannabis use in adults with CHD. Co-use has the most detrimental effects on patient-reported outcomes. ispartof: CANADIAN JOURNAL OF CARDIOLOGY vol:35 issue:12 pages:1842-1850 ispartof: location:England status: published

BACKGROUND: Conflicting results have been reported regarding employment status and work ability in adults with congenital heart disease (CHD). Since this is an important determinant for quality of life, we assessed this in a large international adult CHD cohort. METHODS: Data from 4028 adults with CHD (53% women) from 15 different countries were collected by a uniform survey in the cross-sectional APPROACH International Study. Predictors for employment and work limitations were studied using general linear mixed models. RESULTS: Median age was 32 years (IQR 25-42) and 94% of patients had at least a high school degree. Overall employment rate was 69%, but varied substantially among countries. Higher education (OR 1.99-3.69) and having a partner (OR 1.72) were associated with more employment; female sex (OR 0.66, worse NYHA functional class (OR 0.67-0.13), and a history of congestive heart failure (OR 0.74) were associated with less employment. Limitations at work were reported in 34% and were associated with female sex (OR 1.36), increasing age (OR 1.03 per year), more severe CHD (OR 1.31-2.10), and a history of congestive heart failure (OR 1.57) or mental disorders (OR 2.26). Only a university degree was associated with fewer limitations at work (OR 0.62). CONCLUSIONS: There are genuine differences in the impact of CHD on employment status in different countries. Although the majority of adult CHD patients are employed, limitations at work are common. Education appears to be the main predictor for successful employment and should therefore be encouraged in patients with CHD. ispartof: CONGENITAL HEART DISEASE vol:14 issue:3 pages:362-371 ispartof: location:United States status: published

National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13–20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC.

Ensuring a successful transition from the cardiovascular intensive care unit (CVICU) to the ward for the adult congenital heart disease (ACHD) patient is a vital step in the critical care admission and can have a significant impact on outcomes. The transition process may require unique protocols given the various hospital settings and models of care for ACHD patients. Successful transition involves proper assessment for readiness to transfer, formalized provider handoff procedures, and proactive preparation for hospital discharge. Although various roles within the health-care team will be involved in care transition, the physician, advanced practice provider, and nursing roles have the most direct participation in the transition process. Defining the unique focus of these providers in handoffs and discharge planning will improve the formalized processes for inpatient transitions for ACHD patients. A multidisciplinary and comprehensive approach to transition of the ACHD patient from the CVICU to the ward may decrease critical care morbidity and mortality while promoting patient safety and satisfaction.

Objectives Transition and transfer to adult-oriented health care is an important yet challenging task for adolescents and young adults with chronic medical conditions. Transition practices vary widely, but a paucity of data makes determination of best practices difficult. We described North American pediatric gastroenterologists' preferences and present transition practice patterns and explored whether experience affected providers' perspectives. Methods An online survey was distributed via e-mail to members of the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition. Participation was voluntary and answers were anonymous. Quantitative and qualitative analysis was performed. Results Almost three quarters of the 175 respondents describe providing transition or self-care management education, but only 23% use structured readiness assessments. Most respondents (88%) report having age cutoffs above which they no longer accept new referrals, with the most common age being 18 years (57%). One third report the ability to provide age-appropriate care to patients older than 21 years. Only 6% indicate that their practice or institution should provide care for individuals older than 25 years. Many (63%) indicate that their practice or institution has a policy regarding age of transfer, but most (79%) are flexible. Provider preferences for triggers to transfer to adult care diverge widely between age, milestones, and comorbidities. Overall, parent (81%) and patient (74%) attachment to pediatric health care providers are cited as the most common barriers to transition. Conclusions Preferences and practices surrounding transition preparation and transfer to adult care vary widely, reflecting continued uncertainty regarding optimal transition strategies.

Background There is a paucity of data to guide decisions regarding thromboprophylaxis for atrial arrhythmias in congenital heart disease. Methods A retrospective multicenter cohort study enrolled patients with documented sustained atrial arrhythmias and congenital heart disease from 12 North American centers to quantify thromboembolic and bleeding rates associated with antiplatelet and anticoagulation therapy, and explore associated factors. A blinded committee adjudicated all qualifying arrhythmias and outcomes. Results A total of 482 patients, 45.2% female, age 32.0±18.0years, were followed for 11.3±9.4years since the qualifying arrhythmia. Antiplatelet therapy was administered to 37.8%, anticoagulation to 54.4%, and neither to 7.9%. Congenital heart disease complexity was simple, moderate, and severe in 18.5%, 34.4%, and 47.1%, respectively. Freedom from thromboembolic events was 84.7±2.7% at 15years, with no difference between anticoagulation versus antiplatelet therapy (P=0.97). Congenital heart disease complexity was independently associated with thromboembolic events, with rates of 0.00%, 0.93%, and 1.95%/year in those with simple, moderate, and severe forms (P 2 and CHA 2 DS 2 -VASc scores were not predictive of thromboembolic risk. Annualized bleeding rates with antiplatelet and anticoagulation therapy were 0.66% and 1.82% (P=0.039). In multivariable analyses, anticoagulation [hazard ratio (HR) 4.76, 95% CI (1.05–21.58), P=0.043] and HAS-BLED score [HR 3.15, 95% CI (1.02, 9.78), P=0.047] were independently associated with major bleeds. Conclusion Current management of atrial arrhythmias in congenital heart disease is associated with a modest rate of thromboembolic events, which is predicted by disease complexity but not CHADS 2 /CHA 2 DS 2 -VASc scores. HAS-BLED score is applicable to the congenital population in predicting major bleeds.

Background: The physician associate/assistant (PA) role was introduced into the Irish healthcare system in 2015 when four PAs from North America were recruited to work in Ireland as part of a two-year pilot program. In parallel, the Royal College Surgeons in Ireland (RCSI) began its own PA program in January of 2016 and has graduated 25 PAs. To date, there is little published on the few Irish-trained PAs. This study seeks to share initial experiences with the PA profession in Ireland.Methods: This descriptive phenomenological study explores the newly implemented PA role in Ireland through semi-structured, one-to-one interviews with five PAs and their supervising consultants in Dublin, Ireland. Questions centered on the PA scope of clinical practice, satisfaction with the role, acceptance by the medical team, and integration into the Irish healthcare system.Results: Six themes were identified from the interviews: continuity of patient care, flexible scope of practice, lack of role clarity, complementary to the medical team, regulation and prescribing challenges, and the emerging role of the PA.Conclusions: The development of the PA role is in its infancy in Ireland, but this study suggests that they fill an important role by providing continuity of care to both patients and the medical team. Currently, there are significant challenges imposed by a lack of regulation of the PA profession and the inability to prescribe. However, PAs in Ireland appear to enjoy their work and have great potential to expand throughout Irish healthcare and are valued by their team members.

Data on the differential impact of physical activity on perceived health status (PHS) in a large adult congenital heart disease (ACHD) patient population are lacking. We conducted a cross-sectional assessment of 4,028 ACHD patients recruited from 24 ACHD-specialized centers in 15 countries across 5 continents to examine the association between physical activity and PHS in a large international cohort of ACHD patients. A linear analog scale of the EuroQol-5D 3 level version and the 12-item Short Form Health Survey-version 2 were used to assess self-reported health status and the Health-Behavior Scale-Congenital Heart Disease was used as a subjective measurement of physical activity type, participation, and level. Correlation analyses and Wilcoxon Rank Sum tests examined bivariate relations between sample characteristics and PHS scores. Then, multivariable models were constructed to understand the impact of physical activity on PHS. Only 30% of our sample achieved recommended physical activity levels. Physically active patients reported better PHS than sedentary patients; however, the amount of physical activity was not associated with PHS. Further statistical analyses demonstrated that specifically sport participation regardless of physical activity level was a predictor of PHS. In conclusion, the majority of ACHD patients across the world are physically inactive. Sport participation appears to be the primary physical activity-related driver of PHS. By promoting sport-related exercise ACHD specialists thus may improve PHS in ACHD patients. ispartof: AMERICAN JOURNAL OF CARDIOLOGY vol:122 issue:8 pages:1437-1442 ispartof: location:United States status: published

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

Purpose of review Originally conceived by pediatricians as a model for the care of children with special healthcare needs, the 'patient-centered medical home' (PCMH) has been identified by the Affordable Care Act as a model for all future outpatient care delivery. Although few studies have demonstrated its efficacy in improving care for children with congenital heart disease (CHD), access to a PCMH is likely to improve CHD-patient outcomes, including global function and quality of life, while reducing healthcare costs. Recent findings To date, most patients with complex CHD have their care anchored in cardiology subspecialty-care clinics, which lack many of the attributes of a PCMH. Given that many of these patients have noncardiac morbidities, including neurocognitive impairment and multiorgan dysfunction, we believe such patients will benefit from a PCMH model of care based on primary care. Summary The PCMH based on primary care for patients with complex CHD could result in improved clinical outcomes, improved patient satisfaction and quality of life as well as decreased healthcare costs. Policy and practice reform are required to increase CHD-patient access to a PCMH, based on primary-care settings.

Background American Academy of Pediatrics guidelines emphasize regionalized systems of care for pediatric chronic illness. There remains a paucity of information on the status of regionalized systems of care for pediatric congenital heart disease (CHD). Objectives This study evaluated variations in use of pediatric cardiology specialty care centers (PCSCC) for pediatric patients with CHD in California between 1983 and 2011. Methods We performed a retrospective, total population analysis of pediatric CHD patients using the California Office of Statewide Health Planning and Development unmasked database. PCSCCs were identified by California's Title V program. Results There were 164,310 discharges meeting inclusion criterion. Discharges from PCSCCs grew from 58% to 88% between 1983 and 2011. Regionalized care was highest for surgical (96%) versus nonsurgical (71%) admissions. Admissions with a public payer increased from 42% (1983) to 61% (2011). Total bed days nearly doubled, and median length of stay increased from 2 to 3 days (nonspecialty care) and from 4 to 5 days (specialty care). There was a decrease in the pediatric CHD in-hospital death rate from 5.1 to 2.3 per 100,000 between 1983 and 2011, and a shift toward a larger percent of deaths occurring in the newborn period. Conclusions California’s inpatient regionalized specialty care of pediatric CHD has increased substantially since 1983, especially for surgical CHD discharges. The death rate has decreased, the number of bed days has increased, and a large proportion of these discharges now have public payers. Health care reform efforts must consider these shifts while protecting advances in regionalization of pediatric CHD care.

The American College of Cardiology and American Heart Association guidelines recommend that management of adult congenital heart disease (ACHD) be coordinated by specialty ACHD centers and that ACHD surgery for patients with moderate or complex congenital heart disease (CHD) be performed by surgeons with expertise and training in CHD. Given this, the aim of this study was to determine the proportion of ACHD surgery performed at specialty ACHD centers and to identify factors associated with ACHD surgery being performed outside of specialty centers. This retrospective population analysis used California's Office of Statewide Health Planning and Development's discharge database to analyze ACHD cardiac surgery (in patients 21 to 65 years of age) in California from 2000 to 2011. Designation as a "specialty ACHD center" was defined on the basis of a national ACHD directory. A total of 4,611 ACHD procedures were identified. The proportion of procedures in patients with moderate and complex CHD delivered at specialty centers increased from 46% to 71% from 2000 to 2011. In multivariate analysis among those discharges for ACHD surgery in patients with moderate or complex CHD, performance of surgery outside a specialty center was more likely to be associated with patients who were older, Hispanic, insured by health maintenance organizations, and living farther from a specialty center. In conclusion, although the proportion of ACHD surgery for moderate or complex CHD being performed at specialty ACHD centers has been increasing, 1 in 4 patients undergo surgery at nonspecialty centers. Increased awareness of ACHD care guidelines and of the patient characteristics associated with differential access to ACHD centers may help improve the delivery of appropriate care for all adults with CHD.

Background Impaired quality of life (QOL) is associated with congenital heart disease (CHD) and country of residence; however, few studies have compared QOL in patients with differing complexities of CHD across regional populations. The current study examined regional variation in QOL outcomes in a large multinational sample of patients with a Fontan relative to patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs). Methods From the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease—International Study (APPROACH-IS), 405 patients (163 Fontan and 242 ASD/VSD) across Asia, Europe, and North America provided consent for access to their medical records and completed a survey evaluating QOL (0 to 100 linear analog scale). Primary CHD diagnosis, disease complexity, surgical history, and documented history of mood and anxiety disorders were recorded. Differences in QOL, medical complications, and mood and anxiety disorders between Fontan and ASD/VSD patients, and across geographic regions, were examined using analysis of covariance. Hierarchical regression analyses were conducted to identify variables associated with the QOL ratings. Results Patients with a Fontan reported significantly lower QOL, and greater medical complications and mood and anxiety disorders relative to patients with ASD/VSD. Inpatient cardiac admissions, mood disorders, and anxiety disorders were associated with lower QOL among patients with a Fontan, and mood disorders were associated with lower QOL among patients with ASD/VSD. Regional differences for QOL were not observed in patients with a Fontan; however, significant differences were identified in patients with ASD/VSD. Conclusions Regional variation of QOL is commonplace in adults with CHD; however, it appears affected by greater disease burden. Among patients with a Fontan, regional variation of QOL is lost. Specific attempts to screen for QOL and mood and anxiety disorders among CHD patients may improve the care of patients with the greatest disease burden.

Since the 1995 publication of its Core Cardiovascular Training Statement (COCATS),1 the American College of Cardiology (ACC) has played a central role in defining the knowledge, experiences, skills, and behaviors expected of all clinical cardiologists upon completion of training. Subsequent updates have incorporated major advances and revisions—both in content and structure—including, most recently, …

Background. Illness perceptions are cognitive frameworks that patients construct to make sense of their illness. Although the importance of these perceptions has been demonstrated in other chronic illness populations, few studies have focused on the illness perceptions of adults with congenital heart disease (CHD). This study examined (1) inter-country variation in illness perceptions, (2) associations between patient characteristics and illness perceptions, and (3) associations between illness perceptions and patient-reported outcomes. Methods. Our sample, taken from APPROACH-IS, consisted of 3,258 adults with CHD from 15 different countries. Patients completed questionnaires on illness perceptions and patient-reported outcomes (i.e., quality of life, perceived health status, and symptoms of depression and anxiety). Patient characteristics included sex, age, marital status, educational level, employment status, CHD complexity, functional class, and ethnicity. Linear mixed models were applied. Results. The inter-country variation in illness perceptions was generally small, yet patients from different countries differed in the extent to which they perceived their illness as chronic and worried about their illness. Patient characteristics that were linked to illness perceptions were sex, age, employment status, CHD complexity, functional class, and ethnicity. Higher scores on consequences, identity, and emotional representation, as well as lower scores on illness coherence and personal and treatment control, were associated with poorer patient-reported outcomes. Conclusions. This study emphasizes that, in order to gain a deeper understanding of patients’ functioning, health-care providers should focus not only on objective indicators of illness severity such as the complexity of the heart defect, but also on subjective illness experiences. ispartof: International Journal of Cardiology vol:244 pages:130-138 ispartof: location:Netherlands status: published

Objective: Adolescent patients with chronic health conditions must gradually assume responsibility for their health. Self-management skills are needed for a successful transfer from adolescent to adult health care, but the development of these skills could be resource intensive. Pediatric providers are already instrumental in teaching patients about their health and may improve these skills. The aim of the study was to evaluate whether informal education of pediatric providers regarding transition improves inflammatory bowel disease (IBD) patient self-management skills. Methods: Consecutive patients with IBD older than 10 years who presented to the outpatient setting were administered a survey regarding self-management behaviors in 2008 and 2011. During this time, several conferences on transition were presented to the providers. Results: In 2008, 294 patients completed the survey (82%) compared with 121 patients (89%) in 2011. The patient groups were comparable with respect to sex (boys 50% vs 42%), mean age (16.7 vs 16.2 years), and type of IBD (Crohn 68% vs 66%). The 13- to 15-year-olds reported calling in refills (11%, 8%, respectively), scheduling clinic appointment (0, 1%), preparing questions (13%, 5%), and taking the main role in talking during clinic visits (15%, 24%). The 16- to 18-year-olds reported calling in refills (13%, 27%), scheduling clinic appointments (9%, 6%), preparing questions (9%, 16%), and taking the main role in talking in clinic visits (36%, 45%). Responsibility for behaviors gradually increases with age, but did not differ significantly between 2008 and 2011. Conclusions: Increasing awareness around transition readiness for pediatric providers had an insignificant effect on the self-management skills of patients with IBD. A more formal or structured approach is likely required to improve transition skills in adolescent patients.

OBJECTIVE: This study aimed to examine the association between physical activity (PA) and depression in a large international cohort of adults with congenital heart disease (ACHD) as data about the differential impact of PA type on depression in this population are lacking. METHODS: In 2018, we conducted a cross-sectional assessment of 3908 ACHD recruited from 24 ACHD-specialized centers in 15 countries between April 2013 to March 2015. The Hospital Anxiety and Depression Scale was used to assess self-reported depressive symptoms and the Health-Behavior Scale-Congenital Heart Disease was used to collect PA information. Cochran-Armitage tests were performed to assess trends between depressive symptom levels and PA participation. Chi-Square and Wilcoxon Rank Sum tests were utilized to examine relations between depressive symptom levels and patient characteristics. Stepwise multivariable models were then constructed to understand the independent impact of PA on depressive symptoms. RESULTS: The overall prevalence of elevated depressive symptoms in this sample was 12% with significant differences in rates between countries (p

Exercise capacity following Fontan surgery is often depressed. An inability to reduce pulmonary vascular resistance appropriately during exercise may contribute to this phenomenon. The aim of this study was to determine whether administration of iloprost, a selective pulmonary vasodilator, would improve exercise function after Fontan procedure.Double-blind, randomized, placebo controlled, crossover trial. Patients performed two cardiopulmonary exercise tests (CPX) separated by1 month. A single nebulizer treatment (iloprost or placebo) was administered before each CPX.18 patients aged 12-49 (median 17) years were recruited. Mild throat discomfort developed in 10/18 patients during iloprost administration; all but 1 were able to complete treatment. No symptoms developed during placebo treatments (p0.001). Two additional patients did not complete CPX: one with atrial flutter; another with developmental issues that precluded adequate CPX. In the 15 remaining subjects oxygen pulse (a surrogate for forward stroke volume) at peak exercise was higher following iloprost (median increase 1.2 ml/beat; p0.001). Peak VO2 also rose (median increase 1.3 ml/kg/min; p0.04). Nine patients had peak VO230 ml/kg/min; each of these patients had higher peak VO2 following iloprost. Only 3/6 patients with peak VO230 ml/kg/min had higher peak VO2 following iloprost (p0.04).Iloprost improves the peak oxygen pulse and peak VO2 of patients with Fontan physiology and appears to be particularly beneficial among patients with impaired exercise function. Treatment is associated with minor side effects. These findings support the concept of pulmonary vasodilator therapy in Fontan patients with limited functional capacity.

Objectif: Determiner la prevalence de la dysfonction de la valve aortique, de la dilatation de l'aorte proximale et des interventions au niveau de la valve aortique et de l'aorte ascendante chez les adultes avec une coarctation de l'aorte. Contexte: La dysfonction de la valve aortique et la dilatation de l'aorte proximale sont rares chez les enfants et les adolescents avec une coarctation de l'aorte. A long terme, les adultes pourraient etre plus a risque de developper ce type de pathologie. Methode: Nous avons retrospectivement passe en revue tous les adultes avec une coarctation de l'aorte corrigee ou pas suivis au « Boston Children's Hospital » entre 2004 et 2010. Resultats: 216 adultes (56 % d'hommes) avec une coarctation ont ete identifies. L'âge median a la derniere evaluation etait de 28 (de 18 a 75) ans. Une bicuspidie aortique etait presente dans 66% des cas. Au dernier controle, 3% avaient une stenose aortique moderee ou severe et 4% avaient une insuffisance aortique moderee a severe. Une dilatation de la racine de l'aorte ou de l'aorte ascendante etait presente dans 28%, respectivement 42% des patients. Une dilatation au moins moderee de la racine de l'aorte ou de l'aorte ascendante (score Z > 4) etait presente dans 8%, respectivement 14%. Les patients avec une bicuspidie aortique etaient plus sujets a avoir une dilatation au moins moderee de la racine de l'aorte ou de l'aorte ascendante compares a ceux sans bicuspidie (20% contre 0%; p

Background Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with congenital heart disease (CHD) worldwide. Among ISACHD's objectives is to "promote a holistic team-based approach to the care of the adult with CHD that is comprehensive, patient-centered, and interdisciplinary" (http://www.isachd.org). This emphasis on team-based care reflects the fact that adults with CHD constitute a heterogeneous population with a wide spectrum of disease complexity, frequent association with other organ involvement, and varied co-morbidities and psychosocial issues. Methods Recognizing the vital role of the adult CHD (ACHD) nurse coordinator (ACHD-NC) in optimizing team-based care, ISACHD established a task force to elucidate and provide guidance on the roles and responsibilities of the ACHD-NC. Acknowledging that nursing roles can vary widely from region to region based on factors such as credentials, scopes of practice, regulations, and local culture and tradition, an international panel was assembled with experts from North America, Europe, East Asia, and Oceania. The writing committee was tasked with reviewing key aspects of the ACHD-NC's role in team-based ACHD care. Results/conclusion The resulting ISACHD position statement addresses the ACHD-NC's role and skills required in organizing, coordinating, and facilitating the care of adults with CHD, holistic assessment of the ACHD patient, patient education and counseling, and support for self-care management and self-advocacy.

The adult with congenital heart disease (CHD) is at risk of developing atherosclerotic cardiovascular disease (ASCVD). We performed a cross-sectional study to describe established ASCVD risk factors and estimate 10-year and lifetime risk of ASCVD in adults over age 18 with CHD of moderate or great complexity using three validated risk assessment tools—the Framingham Study Cardiovascular Disease Risk Assessment, the Reynolds Risk Score, and the Atherosclerotic Cardiovascular Disease (ASCVD) Risk Estimator. We obtained extensive clinical and survey data on 178 enrolled patients, with average age 37.1±12.6 years, 51% men. At least one modifiable ASCVD risk factor was present in 70%; the two most common were overweight/obesity (53%) and systemic hypertension (24%). Laboratory data was available in 103 of the 178 patients. Abnormal levels of glycated hemoglobin, high-sensitivity C-reactive protein, and high-density lipoprotein (HDL) were each found in around 30% of patients. The 10-year ASCVD predicted risk using all three tools was relatively low (i.e., at least 90% of patients

Background Pediatric oncologists are responsible for ensuring that adolescent and young adult (AYA) childhood cancer survivors have the knowledge and skills necessary to manage their follow-up care in adult healthcare systems. Procedures To describe transition practices and barriers to transfer, we electronically surveyed U.S. Children's Oncology Group members: 507/1449 responded (35%) and 347/507 (68%) met eligibility criteria. Results Of 347 respondents, 50% are male, median years in practice 10 (range 5–22), 37% practice in freestanding children's hospitals. Almost all care for survivors up to age 21 years (96%), 42% care for survivors over age 25 years, and only 16% over age 30 years. While 66% of oncologists reported providing transition education to their patients, very few (8%) reported using standardized transition assessments. The most frequent barriers to transfer were perceived attachment to provider (91%), lack of adult providers with cancer survivor expertise (86%), patient's cognitive delay (81%), or unstable social situation (80%). Oncologists who continue to care for patients older than 25 years are more likely to perceive parents’ attachment to provider (P = 0.037) and patients’ social situation as barriers to transfer (P = 0.044). Four themes emerged from a content analysis of 75 respondents to the open-ended question inviting comments on transition/transfer practices: desire for flexible transfer criteria; providers as barriers; provider lack of transition knowledge, skills, and resources; and desire for collaboration. Conclusions Although most pediatric oncologists reported transferring AYA cancer survivors to adult care and providing some transition education, they endorse deficits in transition skills, emotional readiness, and institutional resources.