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The congenital heart disease (CHD) population now comprises an increasing number of older persons in their 6th decade of life and beyond. We cross-sectionally evaluated patient-reported outcomes (PROs) in persons with CHD aged 60 years or older, and contrasted these with PROs of younger patients aged 40-59 years and 18-39 years. Adjusted for demographic and medical characteristics, patients ≥60 years had a lower Physical Component Summary, higher Mental Component Summary, and lower anxiety (Hospital Anxiety and Depression Scale-Anxiety) scores than patients in the two younger categories. For satisfaction with life, older persons had a higher score than patients aged 40-59 years. Registration: ClinicalTrials.gov NCT02150603. ispartof: EUROPEAN JOURNAL OF CARDIOVASCULAR NURSING vol:22 issue:4 pages:339-344 ispartof: location:England status: published

As promising therapeutic targets for several types of cancers, Aurora kinases A and B share remarkably high sequence and structure similarity, which render the design of subtype selective small-molecule inhibitors challenging. Since Aurora A acts as both an oncogene and a haploinsufficient tumor suppressor, inhibiting Aurora B with highly selective inhibitors represents a less toxic anti-cancer strategy. However, the molecular mechanism governing the ligand selectivity for Aurora A over B reminds elusive. In this study, an experimentally validated ligand, Barasertib which displays 1000-fold selectivity for Aurora B over A was used as a template molecule, and the selectivity mechanism was investigated by molecular dynamics simulation and binding free energy analyses. The computed binding free energies are consistent with the experimental bioactivity of Barasertib. The major residues that contribute to ligand binding were also identified. The hinge residue Arg159, only found in Aurora B was found to contribute significantly to the binding of Aurora B, while no such binding was found in Aurora A. A highly unfavorable polar interaction with Glu181 in Aurora A hinders the ligands binding ability. These 2 interactions play a key role in the observed selectivity of Barasertib for Aurora B over A. Interestingly, the binding of partner proteins forces the aC helix and the beta sheets in the N-lobe to move inward towards the ATP binding pocket, leading to a smaller hydrophobic back pocket and unfavorable binding. Taken together, our results show how differential behavior of the ligand arises from a complex interplay of small but relevant structural differences upon binding to different kinase subtypes. The insights gained into the structural determinants of subtype selectivity will facilitate the further development of highly selective and potent inhibitors as drug candidates for cancer therapy.

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

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.

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

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., Central Illustration

Although resilience and high quality of life are demonstrated by many individuals with congenital heart disease, a range of significant psychological challenges exists across the life span for this growing patient population. Psychiatric disorders represent the most common comorbidity among people with congenital heart disease. Clinicians are becoming increasingly aware of the magnitude of this problem and its interplay with patients' physical health, and many seek guidance and resources to improve emotional, behavioral' and social outcomes. This American Heart Association scientific statement summarizes the psychological outcomes of patients with congenital heart disease across the life span and reviews age-appropriate mental health interventions, including psychotherapy and pharmacotherapy. Data from studies on psychotherapeutic, educational' and pharmacological interventions for this population are scarce but promising. Models for the integration of mental health professionals within both pediatric and adult congenital heart disease care teams exist and have shown benefit. Despite strong advocacy by patients, families' and health care professionals, however, initiatives have been slow to move forward in the clinical setting. It is the goal of this scientific statement to serve as a catalyst to spur efforts for large-scale research studies examining psychological experiences, outcomes, and interventions tailored to this population and for integrating mental health professionals within congenital heart disease interdisciplinary teams to implement a care model that offers patients the best possible quality of life.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

Purpose of review Adults with congenital heart disease (CHD) may experience anxiety specifically related to their medical condition. This review introduces the concepts of health anxiety and heart-focused anxiety, summarizes what is currently known about heart-focused anxiety among adults with CHD and offers suggestions to help adult CHD providers address heart-focused anxiety in their patients. Recent findings Although minimal research has been conducted specific to this outcome, health anxiety may occur at any point across the lifespan of individuals with CHD. A recent study found that children and adolescents with CHD reported greater health anxiety than community peers. Health anxiety was commonly reported among adults with CHD presenting for psychological assessment. It was linked with older age, trait anxiety, perceived parental overprotection and greater CHD complexity in one study. Summary Adults with CHD face many potential health-related stressors, including cardiac symptoms, treatments and interventions throughout the lifespan (including surgeries and other invasive procedures), the impact of CHD on daily lives and longer-term health expectations. Providers should be aware that heart-focused anxiety among patients is understandable and perhaps common. Patient-centred education and psychological intervention should be integrated within a comprehensive approach to long-term disease management.

Learning objectives In 1964, Glaser recognised that, ‘Despite benefits derived from these remarkable therapeutic gains, children with congenital heart disease face many difficulties in their efforts toward social and emotional adjustment.’1 In 1981, Doucet wrote, ‘The impact of congenital heart disease on the individual would not be limited to the physiological alteration in the heart’s functioning but would affect the whole individual.’2 Recognition of the broader lifestyle implications of congenital heart disease (CHD) for both children and adults is thus not a modern concept. However, what is unique to the current era of CHD care in developed countries is that over 90% of infants born with CHD survive to adulthood, with the number of adults living with CHD now far surpassing the number of children.3 This demands that we expand our definition of successful adult CHD (ACHD) outcomes to include good quality of life, including the best possible neurocognitive and psychosocial functioning, and target our clinical efforts accordingly. Although neurocognitive and psychosocial outcomes among adults with CHD are often considered independently, this silo-oriented approach ignores their reciprocal interplay. For example, neurocognitive, academic and occupational functioning can be adversely affected if a person is struggling with anxiety or depression. Similarly, neuropsychological deficits can adversely affect social development, impairing one’s ability to establish and sustain satisfying relationships in adulthood. In addition, one cannot fully appreciate adult outcomes without considering their origins in childhood and adolescence. In this review, we thus apply a developmental lifespan perspective to better understand neurocognitive and psychosocial outcomes of …

Although the majority of congenital heart disease survivors are thriving, many are at risk for declining emotional well-being as they age. Emotional distress is a risk factor for poorer health outcomes and must be addressed. Primary care and cardiology teams may be the first line of defense in identifying and providing referral resources for symptoms of depression, anxiety, and medical trauma. The current review provides information about commonly used self-report measures of emotional distress to identify symptoms that warrant referral and describes multiple options for addressing these symptoms.

The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

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: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.

Traditional congenital heart disease (CHD) outcomes include mortality (survival to adulthood and life expectancy) as well as cardiac and noncardiac morbidity. Strategies to identify and manage sequelae have primarily focused on objective data obtained though invasive and noninvasive diagnostic approaches. In contrast, patient-reported outcomes (PROs) provide subjective information, using standardized measures, about patients' health and well-being as reported directly by patients, without interpretation, interference, or assumptions made by clinicians or others. Selection of PRO measures entails thoughtful consideration of who the individuals being surveyed are, why assessment is occurring (eg, what are the domains of interest; clinical vs research), and what processes are in place for acquisition, administration, interpretation, and response. In this review, we focus on 3 domains of PROs for pediatric and adult patients with Fontan physiology: physical health status, psychological functioning, and quality of life. Infants, children, adolescents, and adults with CHD face a spectrum of challenges that might influence PROs across the life span. In general, patients with Fontan palliation tend to have lower physical health status, experience more psychological distress, and have equivalent or reduced quality of life compared with healthy peers. Herein, we provide an overview of PROs among people with Fontan circulation as a group, yet simultaneously emphasize that the optimal way to understand the experiences of any individual patient is to ask and listen. We also offer clinical and research initiatives to improve the adoption and utility of PROs in CHD settings, which show commitment to capturing, understanding, and responding to the patient voice.

Background: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardio-vascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II.Methods/design: APPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments.Discussion: APPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD., KU Leuven - University of Leuven; Research Foundation Flanders [1159522N, 12E9819N]; Swedish Heart and Lung Foundation [20190525]; Research Council of Aga Khan University in Karachi in Pakistan; Maltese Cardiac Society; Children's Heart Unit Fund; UnIC (Unidade de Investigacao Cardiovascular da FMUP); Italian Ministry of Health, This work is supported by KU Leuven - University of Leuven, Research Foundation Flanders through grants 1159522N and 12E9819N, Swedish Heart and Lung Foundation through grant 20190525, the University Research Council of the Aga Khan University in Karachi in Pakistan, Maltese Cardiac Society, UnIC (Unidade de Investigacao Cardiovascular da FMUP) supported the costs of the translations into Portuguese used in Portugal, Children's Heart Unit Fund ( www.chuf.org.uk), Newcastle upon Tyne through their funding of the CHUF Fontan nurse specialist. This study was partially supported by Ricerca Corrente funding from the Italian Ministry of Health to IRCCS Policlinico San Donato.

Background: Adverse childhood experiences (ACEs) are associated with poorer health outcomes, including a higher risk of acquired cardiovascular disease. This was the first investigation of ACEs in adults with congenital heart disease (CHD). Methods: Adults with CHD completed the following surveys in the outpatient setting: ACE questionnaire, measure of attachment style, and linear analogue measure of quality of life. We performed Chi-square tests and t-tests to explore relationships between ACE scores with other surveys as well as demographic and clinical characteristics. Results: A total of 100 patients participated in the study (age 40 ​± ​13 years; 60% female); 90% had CHD of moderate or great complexity. The total number of reported ACEs ranged from 0 to 9 out of a possible total of 10, with a mean of 2.6 ​± ​2.5.30% of participants reported ≥ 4 ACEs, which is higher than the reported prevalence in the general population. The most frequently reported ACEs were parental divorce (46%), emotional abuse (42%), and parental substance abuse (32%). ACE scores did not differ as a function of age, sex or CHD defect complexity. Total ACE scores were significantly associated with two attachment styles (preoccupied: p ​

Формування та використання місцевих бюджетів є одним з невирішених питань у взаємовідносинах між Державним і місцевими бюджетами. Для забезпечення життєдіяльності місцевих бюджетів необхідно не делегувати їм відповідні повноваження, а в законодавчому порядку розмежувати компетенцію , функції та обов’язки держави і органів самоврядування та законодавчо це визначити, що Україна в даний період здійснює, але повільно і не завжди використовує досвід європейських країн. Фінансовою основою розвитку територіальних громад є місцеві бюджети. В процесі реформування місцевого самоврядування та територіальної організації влади функціонування місцевих бюджетів повинно бути спрямоване на активізацію внутрішнього потенціалу, застосування механізмів нарощування доходної бази, забезпечення оптимального використання ресурсів громад та її стабільного економічного розвитку. Особливої актуальності зазначені питання набувають у зв’язку з реалізацією першого етапу адміністративно-територіальної реформи, внесенням змін до Конституції, бюджетного і податкового законодавства України та ряду інших законодавчих актів пов’язаних з цими змінами. Україна розпочала новий етап розвитку шляхом здійснення реформ у всіх її сферах, серед яких вирізняється реформа управ¬ління державою, в основі якої лежись реформа бюджетно-податкової системи як децентра¬лізація місцевих бюджетів. Фінансова децентралізація є тією рушійною силою, яка дала змогу місцевим громадам почати вирішувати проблеми, що накопичувались десятиліттями, зокрема — це передача значних повноважень і бюджетних ресурсів від державних органів влади до органів місцевого самоврядування, тому що місцева влада краще знається на проблемах своїх територій, ніж центральна і має змогу ефективніше їх вирішувати.

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.

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: 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

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

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 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.