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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

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

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.

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 ​

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.

Patient-reported outcomes (PROs) are “any outcome based on data provided by patients or patient proxy as opposed to data provided from other sources”. Together with clinician-reported, physiological, and caregiver-reported outcomes, PROs are important sources of data to assess health and well-being. Examples of PROs include quality of life, psychosocial well-being, functional status, symptoms, health behaviours, and adherence to treatment. PRO research requires specific methodologies, for which dedicated expertise is needed. In congenital heart disease (CHD), PRO research has received increasing attention over the past decade. This paper summarizes knowledge advancements achieved by the ‘Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study’ (APPROACH-IS). This was an international project conducted in 15 countries situated on 5 continents, and enrolled more than 4000 patients. Using a uniform research methodology, a broad set of correlates of PROs in CHD was investigated, both at the individual and contextual level. APPROACH-IS confirmed intercountry variation in PROs. Furthermore, several country-level characteristics were associated with PROs in CHD, although they explained little of the variance. Indeed, most of the variance in PROs was explained by individual medical, demographic, behavioural, psychological, and social factors. Although much has been learned about PROs in CHD as a result of APPROACH-IS, this project also generated new questions. The assessment of PROs must thus remain a research priority.

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.

Purpose To understand the effectiveness of a nurse-led transition intervention by analyzing qualitative data generated in the context of a clinical trial. Design & methods Qualitative study of a two-session transition intervention conducted by registered nurses at two sites. Adolescents aged 16–17 years with moderate or complex congenital heart disease (CHD) had been randomized to a two-session transition intervention or usual care. Session 1 emphasized patient education including creation of a health passport and goal setting. Session 2, two months later, emphasized self-management. Qualitative data extracted from intervention logs, field notes and audio recordings of the sessions were analyzed for content and themes. Results Data from 111 transition intervention sessions with 57 adolescents were analyzed. Creating a health passport, goal setting, and role-plays were the elements of the intervention most valued by participants. A typology of transition readiness was identified: 1) the independent adolescent (5%), already managing their own care; 2) the ready adolescent who was prepared for transition after completing the intervention (46%); 3) the follow-up needed adolescent who was still in need of extra coaching (26%), and 4) the at-risk adolescent who warranted immediate follow-up (14%). Baseline knowledge and transition surveys scores validated the typology. Conclusions A two-session nursing intervention met the transition needs of approximately half of adolescents with CHD. However, additional transition-focused care was needed by 40% of participants (groups 3 and 4). Practice implications These findings will guide pediatric nurses and other healthcare professionals to optimize an individualized approach for ensuring transition readiness for adolescents with CHD.

Introduction: Adverse childhood experiences (ACEs), such as witnessing domestic abuse or experiencing physical/sexual abuse, are associated with poorer long-term health outcomes, including acquired cardiovascular disease. This study offers the first investigation of ACEs in adults with congenital heart disease (CHD). Methods: In this cross-sectional study, adult CHD outpatients completed the ACE Questionnaire, the Relationship Scales Questionnaire, the Perceived Stress Scale, and a 0 - 100 linear analogue scale of quality of life. Total scores on the ACE Questionnaire range from 0 - 10 (a point for each ACE reported) and scores ≥ 4 are associated with poorest health outcomes. We performed correlations, t-tests, and analyses of variance to explore relationships between ACE Questionnaire scores and demographics, medical variables, and other survey scores. Results: A total of 100 patients were enrolled in the study (40 ± 13 years; 60% female). Ninety percent had defects of moderate or great complexity and 79% were categorized as American Heart Association anatomy + physiology stage B, C or D (i.e., some degree of symptoms and/or functional impairment). Total scores on the ACE Questionnaire ranged from 0 - 9, with a mean of 2.6 ± 2.5; 30% reported ≥ 4 ACEs. The most frequently reported ACEs were parental divorce (46%), emotional abuse (42%), and parental substance abuse problem (32%). ACE Questionnaire scores did not differ as a function of age, sex, defect complexity, or anatomy + physiology stage. However, total ACE score was significantly correlated with all 4 relationship styles (secure, preoccupied, dismissive and fearful; p-values < 0.05). Quality of life scores were lower among those with elevated ACEs, although this did not reach statistical significance (69 vs. 76, p = 0.07). Conclusion: It is well accepted that adults with CHD face many health-related challenges throughout their lives. This study serves as an important reminder to providers that many patients will also experience other significant (and potentially traumatic) stressors in childhood. Further research is needed to determine whether the impact of ACEs on adults with CHD extends beyond relationship and stress outcomes and also includes cardiac morbidity and mortality.

Background: Ventricular arrhythmias (VAs) and their treatment have been associated with psychological distress and poorer quality of life (QOL) in research studies. Patient-reported outcome (PRO) measures are beginning to be introduced in clinical settings and early experiences with their clinical utility warrant attention. We wanted to describe PROs of patients attending a multidisciplinary VA clinic in order to elucidate roles that PRO measures might have in clinical settings. Methods: In this retrospective study, we enrolled patients with history of sustained VA and/or ICD shock at their initial visit to a clinic staffed by an electrophysiologist and a psychologist. Patients completed several PRO measures including the following: anxiety and depression symptoms, visual analog scales for health status and QOL, cardiac device acceptance, ICD shock anxiety, and general cardiac anxiety. Here we report total scores for general PROs and item-level analysis of the three cardiac-specific measures. Results: A total of 57 patients (56 ±15 years; 84% male) were included; 39% had a history of sudden cardiac arrest, 70% had prior ICD shock and 44% had previously undergone VA ablation. Symptoms that exceeded clinical thresholds were common for anxiety (49%) and depression (20%). On 0-100 visual analogue scales, the mean rating for health status was 57 ± 16 and for QOL was 66 ± 18. Item-level analysis of cardiac-specific PROs revealed that between 40% and 50% of patients endorsed specific concerns regarding return to work, resumption of physical activities, and sexual relations (Table 1). Conclusion: Among patients attending a VA clinic, elevated symptoms of anxiety and depression were common and self-reported health status and QOL were low. Although PRO total scores provided general information, review at the individual item level provided critical information about potential sources of anxiety that can guide cardiologists during discussions with patients.

Introduction: Adult congenital heart disease (ACHD) patients face a unique set of medical, psychological, and social challenges, and access to specialized ACHD care has been shown to improve their outcomes. Rural ACHD patients may represent a uniquely disadvantaged group given their complex health care needs and the distance they must travel to access care. The aim of this study was to investigate the challenges faced by ACHD patients in accessing outpatient cardiac care, with a specific focus on understanding barriers related to distance from care. Methods: This cross-sectional, survey-based study took place in the ACHD clinic at an academic medical center in a metropolitan area. Additional medical information was abstracted in a retrospective manner from the electronic health record. T-tests and Chi-square tests were used to investigate the relationship between variables. Results: A total of 100 patients participated in the study (mean age 40 ± 13 years, 60% female, 18% rural dwelling). The median driving distance to clinic was 20 miles (interquartile range 12 to 77 miles). The most commonly identified barriers to cardiac clinic visits were the financial losses related to taking time off from work (39%), distance of clinic from home (33%), and the weather (33%), whereas finding a ride to clinic, finding childcare, or the time of available clinic appointments were not perceived as significant barriers. As compared to urban dwelling patients, those who were rural dwelling had a lower level of education (p=0.04), more difficulty paying insurance premiums (p Conclusions: Loss of compensation from taking time off work to attend a visit and distance to clinic were the most commonly identified barriers to outpatient cardiac care in this cohort of ACHD patients. 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 ACHD care and better serve this growing patient population.

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 To evaluate the impact of a novel nurse-led transition intervention program designed for young adolescents (age 13-14 years) with congenital heart disease (CHD). We hypothesized that the intervention would result in improved self-management skills and CHD knowledge. STUDY DESIGN Single-center cluster randomized controlled trial of a nurse-led transition intervention vs usual care. The intervention group received a 1-hour individualized session with a cardiology nurse, focusing on CHD education and self-management. The primary end point was change in TRANSITION-Q (transition readiness) score between baseline and 6 months. The secondary end point was change in MyHeart score (CHD knowledge). RESULTS We randomized 60 participants to intervention (n = 30) or usual care (n = 30). TRANSITION-Q score (range 0-100) increased from 49 ± 10 at baseline to 54 ± 9.0 at 6 months (intervention) vs 47 ± 14 to 44 ± 14 (usual care). Adjusted for baseline score, TRANSITION-Q scores at 1 and 6 months were greater in the intervention group (mean difference 5.9, 95% CI 1.3-10.5, P = .01). MyHeart score (range 0-100) increased from 48 ± 24 at baseline to 71 ± 16 at 6 months (intervention) vs 54 ± 24 to 57 ± 22 (usual care). Adjusted for baseline score, MyHeart scores at 1 and 6 months were greater in the intervention group (mean difference 19, 95% CI 12-26, P

The initial "Frontiers in Fontan Failure" conference in 2015 in Atlanta, GA, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with "Failing Fontan" physiology. Four types of "Fontan Failure" were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure "imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation." The purpose of the second "Frontiers in Fontan Failure" was to further the discussion regarding new data and technologies as well as novel interventions. The 2017 "Frontiers in Fontan Failure: Innovation and Improving Outcomes" was sponsored by Children's Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complications, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic-related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to.

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

Purpose To assess a motivational interviewing (MI) intervention to improve moderate-to-vigorous physical activity (MVPA) in adolescents with congenital heart disease. Methods Intervention participants received one-on-one telephone-based adapted MI sessions over 3 months. Outcomes were acceptability, change mechanisms (stage of change and self-efficacy), and limited efficacy (physical activity, fitness, and quality of life). Results Thirty-six participants were randomized. Intervention participants completed 4.2 ± 1.2/6 MI sessions, with no improvements in the high self-efficacy or stage of change observed. Participants accumulated 47.24 ± 16.36 minutes of MVPA/day, and had comparable outcomes to peers without heart disease (except for functional capacity). There was no significant difference in change in any outcome by group. Conclusions The intervention was acceptable, but effectiveness could not be determined due to the nature and size of sample. Clinical relevance Pediatric cardiac rehabilitation remains the sole effective intervention to increase MVPA in this population.

Background North American adults with congenital heart disease (CHD) are known to be at elevated risk of mood and anxiety disorders. This was the first trial of a group psychosocial intervention targeting this patient population. Methods Within this feasibility study, we conducted a 2-arm pilot randomized controlled trial (RCT) in which patients were randomized to Usual Care or an 8-session group psychosocial intervention ( A dult C ongenital H eart D isease- C oping a nd Re silience [ACHD-CARE]). Here, we report feasibility outcomes in accordance with published recommendations: (1) process, (2) resources, (3) management, (4) acceptability of the intervention, and (5) scientific outcomes (for which the primary outcome measures were anxiety and depression symptoms). Results Forty-two patients were randomized in the pilot RCT. The study was executable within a realistic timeline and revealed no significant human and data-management problems. The intervention was determined to be acceptable and highly valued by participants who participated in the ACHD-CARE program. The main challenges were practical barriers (eg, transportation, scheduling group sessions in-person given competing schedules) and retention. With regard to scientific outcomes, there were no adverse outcomes, and treatment fidelity was confirmed. Although not powered to test efficacy, there was a medium effect size (in favour of the intervention group) for depression symptoms. Conclusions We determined it would be feasible to conduct a full-scale trial of a psychosocial intervention targeting adults with CHD, although with modifications to address practical barriers to participation. Should this intervention prove effective, a manualized intervention could be made be available.

In the current era, most individuals born with congenital heart disease will reach adulthood. There are now more adults than children living with congenital heart disease in developed countries, and the population continues to expand. Although the life-saving accomplishments achieved in pediatric cardiology are to be extolled, longer-term sequelae also warrant close attention. Adults with moderate or complex disease continue to face cardiac and noncardiac comorbidities as well as a shorted life expectancy. This article begins with a summary of common comorbidities and patterns of mortality faced by adults with congenital heart disease. This is followed by an overview of the broader impact of these issues on the lives of patients, as well as a call for interdisciplinary and comprehensive care to optimize patient outcomes.

Objective Although heart failure (HF) is the leading cause of premature death in adult congenital heart disease (ACHD), little population-specific data exist. This study reports early experience from a dedicated, sub-specialty adult congenital heart disease-heart failure (ACHD-HF) clinic, aiming to identify risk factors for adverse outcome. Methods Between 2012 and 2015, 126 patients (57% male) attended the ACHD-HF clinic. Baseline and follow-up data were analysed and compared across 4 anatomical/physiological subgroups: cyanotic ACHD, Fontan circulation (1V), biventricular circulation with a subaortic right ventricle (2V-RV) and biventricular circulation with a subaortic left ventricle (2V-LV). Predictors of the composite primary outcome: death, transplant or ventricular assist device (VAD) were identified using multivariable Cox proportional hazard models. Results Mean age at first visit was 38±13years. Patients were grouped as follows: cyanotic ACHD 10%, 1V 24%, 2V-RV 29% and 2V-LV 37%. During a median follow-up of 1.7 (IQR 0.8–2.9) years, 38 patients (30%) reached the primary outcome. Event-free survival was 89%, 78% and 63% at 1, 2 and 3years. Forty (31.7%) patients experienced 69 HF hospitalisations. Between-group differences were noted for systolic function, valvular regurgitation, pacing prevalence and invasive hemodynamics. Multivariable analysis revealed 2V-RV subgroup ( p =0.001), NYHA class ( p =0.002) B-type natriuretic peptide >164pg/ml ( p =0.003) and sodium p =0.036) as independently associated with death, transplant or VAD. Conclusions Our young ACHD-HF patients experienced high adverse event rates during a short period of follow-up. The prognostic markers identified will aid clinicians to stratify short-term risk and thereby guide advanced HF management decisions in ACHD.

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

Objective A physically active lifestyle can help maintain positive physical and psychosocial health outcomes among adults with congenital heart disease (CHD). This study explored the physical activity perceptions and behaviors among young adults with CHD. Design This was a cross-sectional, mixed-methods study that included objectively measured physical activity assessment (accelerometer), individual semistructured interviews, and psychosocial questionnaires. Results Fifteen participants (67% male; 21 ± 3 years old) with moderate (n = 10) or complex (n = 5) CHD were recruited from an outpatient adult CHD clinic. Participants accumulated 26 ± 16 minutes of moderate-to-vigorous physical activity per day, and reported a high quality of life, moderate self-efficacy for exercise, and low cardiac-focused anxiety. Qualitative data indicated that participants reported more positive perceptions toward activity if their family members encouraged physical activity participation, including siblings that engaged in physical activity alongside participants. Participants described parents as supportive rather than overprotective. Activity precautions were perceived by participants as being instructions from cardiologists rather than restrictions by parents. Participants described some physical limitations compared to peers, but managed challenges by either working within their limitations or choosing activities that met their expectations and/or in which they could fully participate. Participants often described childhood physical activity in the context of school, physical education, and organized sports. Whereas physical activity in childhood was viewed as recreational, the cardiac health-promoting aspects became more prominent in adulthood. Activities performed during one's employment were considered sufficient to meet physical activity recommendation levels, and participants reported limited time and/or energy to participate in activity outside of work. Conclusions The influence of family appeared to help participants adopt a positive perception toward activity participation in childhood that was carried forward to young adulthood. Future clinical work should target adolescents with CHD with less social supports and/or negative perceptions toward physical activity.

Background Patients diagnosed with inflammatory bowel disease (IBD) during childhood require transfer to an adult gastroenterologist, in Ontario usually just before their 18th birthday. Pediatric onset IBD is a complex phenotype with demonstrated noncompliance risk that may require targeted measures to optimize health care outcomes in the adult care setting. Purpose The purpose of this study was to determine the impact of posttransfer health care setting (academic versus community gastroenterologist) on emergent health resource utilization. Methods This was a population-based retrospective cohort study using health care administrative data from Ontario, Canada. A cohort of patients with Pediatric onset IBD was identified and health resource utilization during a 2-year pretransfer period, transfer of care period and 2-year posttransfer period was analyzed. Posttransfer health care setting was defined as academic (i.e., gastroenterologists providing care in a university affiliated tertiary care center) versus community. A third comparator group, loss to follow-up, was also identified. The primary outcome of this study comprised emergency department utilization. Secondary outcomes included hospitalizations, surgeries, ambulatory visits, endoscopic investigations, and radiological investigations. Results Overall, there were no significant differences found in emergency department use, ambulatory care visits (aside from the expected drop in the lost to follow-up group), hospitalizations, endoscopic procedures, or radiological procedures between exposure groups. Conclusions Posttransfer health care setting does not seem to significantly impact emergent health resource utilization in the posttransfer period.

Background Adult congenital heart disease (ACHD) patients with moderate or great defect complexity are at risk for premature death. Although early engagement in advance care planning (ACP) is recommended, previous research suggests that it seldom occurs. Methods This study investigated ACHD patient preferences for ACP and factors that impact preferences. ACHD patients completed an ACP preferences questionnaire, the Hospital Anxiety and Depression Scale and a measure of attachment styles. Results Of 152 ACHD patients (median age 33years, 50% female), 13% reported previous ACP discussions with providers and 21% had completed advance directives. On a 0–10 scale, the median rating for the importance of discussing ACP with providers was 7; 18years was identified as the most appropriate age to initiate this dialogue. Higher ratings for the importance of discussing ACP with providers was observed in patients who were female ( p =0.03), had lower disease complexity ( p =0.03), and had elevated anxiety symptoms ( p =0.001); elevated anxiety remained significant in a multivariable model. Interest in receiving information about life expectancy (61% overall) was greater among patients with lower disease complexity ( p =0.04) and a history of ≥2 cardiac surgeries ( p =0.01); disease complexity remained significant in a multivariable model. Conclusions As a group, ACHD patients value the opportunity for ACP discussions and prefer earlier communication. Although some clinicians might avoid ACP discussions in patients who are generally more anxious or have less complex CHD, such avoidance does not appear to be warranted.

Survival prospects in adults with congenital heart disease (CHD), although improved in recent decades, still remain below expectations for the general population. Patients and their loved ones benefit from preparation for both unexpected and predictable deaths, sometimes preceded by a prolonged period of declining health. Hence, advance care planning (ACP) is an integral part of comprehensive care for adults with CHD. This position paper summarizes evidence regarding benefits of and patients' preferences for ACP and provides practical advice regarding the implementation of ACP processes within clinical adult CHD practice. We suggest that ACP be delivered as a structured process across different stages, with content dependent upon the anticipated disease progression. We acknowledge potential barriers to initiate ACP discussions and emphasize the importance of a sensitive and situation-specific communication style. Conclusions presented in this article reflect agreed expert opinions and include both patient and provider perspectives. ispartof: EUROPEAN HEART JOURNAL vol:41 issue:43 pages:4200-4210 ispartof: location:England status: published

ispartof: European Journal Of Cardiovascular Nursing vol:19 issue:6 pages:465-468 ispartof: location:England 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

Adults with congenital heart disease face many potential psychosocial challenges. This review focuses on their experiences of clinically significant anxiety and depression and provides recommendations for appropriately timed referrals to mental health professionals. Adults with congenital heart disease have a higher probability of experiencing symptoms consistent with mood and anxiety disorders, yet risk factors are not well understood. Although patients as a group are often interested in psychological treatment, there is currently under-recognition and under-treatment of mental health concerns. As provider and patient awareness of the biopsychosocial implications of living with congenital heart disease continues to grow, the focus of comprehensive care is broadening to include attention to psychosocial well-being. Care teams are encouraged to create a culture that fosters open and ongoing dialog about emotional well-being, including depression and anxiety, and includes reliable processes for mental health referrals.

Objective:Subspecialisation is increasingly a fundamental part of the contemporary practice of medicine. However, little is known about how medical trainees learn in the modern era, and particularly in growing and relatively new subspecialties, such as adult CHD. The purpose of this study was to assess institutional-led and self-directed learning strategies of adult CHD fellows.Methods:This international, cross-sectional online survey was conducted by the International Society for Adult Congenital Heart Disease and consisted primarily of categorical questions and Likert rating scales. All current or recent (i.e., those within 2 years of training) fellows who reported training in adult CHD (within adult/paediatric cardiology training or within subspecialty fellowships) were eligible.Results:A total of 75 fellows participated in the survey: mean age: 34 ± 5; 35 (47%) female. Most adult CHD subspecialty fellows considered case-based teaching (58%) as “very helpful”, while topic-based teaching was considered “helpful” (67%); p = 0.003 (favouring case-based). When facing a non-urgent clinical dilemma, fellows reported that they were more likely to search for information online (58%) than consult a faculty member (29%) or textbook (3%). Many (69%) fellows use their smartphones at least once daily to search for information during regular clinical work.Conclusions:Fellows receiving adult CHD training reported a preference for case-based learning and frequent use of online material and smartphones. These findings may be incorporated into the design and enhancement of fellowships and development of online training resources.

It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals. ispartof: CIRCULATION vol:140 issue:6 pages:E234-E284 ispartof: location:United States status: published

Dramatic increases in survival to adulthood for persons born with congenital heart disease (CHD) have led rise to a corresponding need to provide age-appropriate and developmentally appropriate care across the lifespan. Health care transition is a multidimensional process that ideally begins in early adolescence in the pediatric setting and continues through young adulthood with input from both pediatric and adult CHD providers. Preparation for transition includes the fostering of adolescents' knowledge of their CHD and of self-management and self-advocacy skills needed for lifelong management of chronic disease. Transfer is the event in time when a patient's care and ownership of health records is taken over by the adult health care team; this is just one element of the broader transition process. Transfer typically occurs by age 18 throughout much of Canada. Successful transition is a shared responsibility, requiring engaged pediatric and adult providers and partnership with both young adults and their parents, all of whom may struggle with this process. An interdisciplinary approach to transition is recommended, given that health care transition is a complex process that occurs within the broader context of young adults' lives. This review summarizes existing evidence regarding transition and transfer, offers perspectives from multiple stakeholders, and proposes a transition curriculum of development of CHD education and self-management and self-advocacy skills. Specific recommendations to improve implementation of transition and transfer care within the Canadian context are provided. This review sheds light on the current capacity and challenges of adult CHD providers and proposes directions to move this field forward.

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

Children born with congenital heart disease (CHD) are now living to adulthood in unprecedented numbers and many will eventually live to become senior citizens. As care goals shift from surviving to thriving, a new focus on quality of life has emerged. Neurocognition and the ability to participate fully in society, form meaningful relationships, and collaborate effectively with the health care system are important considerations. As adults with CHD age, research regarding their cognitive function becomes prescient. The focus is now shifting from defining neurocognitive deficits in children with CHD to preventing neurocognitive decline in adults living with CHD. In this review, we describe the possible etiologies and predictors of neurocognitive decline in adults with CHD. We performed a comprehensive literature review to identify all of the current data available on neurocognitive function in adults with CHD. We summarize the available evidence by describing common deficits in this patient population and the potential effects of these deficits on adult functioning, health care decision-making, and long-term relationships with care providers. We review potential modifiable etiologies for progressive neurocognitive decline and suggest strategies for surveillance and prevention of the potential decline. We conclude that the current information available regarding the aging brain of adults with CHD and the effect of neurocognitive decline on morbidity and mortality is woefully insufficient. This review, therefore, provides a roadmap for future research endeavours to study neurocognition in older adults with CHD.

Introduction: Physical activity (PA) has well-known benefits for mental health, which is closely related to clinical outcomes in patients with heart disease; however, little is known about the relationship between PA and anxiety. In this study, we examined their association in a large international cohort of adult congenital heart disease (ACHD) patients. Hypothesis: We hypothesize that different PA types would have differential impact on anxiety symptoms. Methods: We conducted a cross-sectional assessment of data derived from the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart Disease - International Study . This study enrolled 4,028 ACHD patients from 24 centers in 15 countries. Anxiety symptoms were assessed with the Hospital Anxiety and Depression Scale (HADS). Participants reported whether they were regular active commuters (either walk or cycle to work/school) and/or regular sport participants (either recreational or competitive). To assess the relationship between PA and anxiety, we constructed ordinal logistic mixed models, treating country as a random effect. We built an optimally-adjusted model via stepwise selection, which considered all variables having significant relations with anxiety in bivariate analyses and missing at a rate of < 5%. Results: Of 3,893 patients (mean age of 35 ± 13) who completed the HADS, 53% were female; 27% reported elevated anxiety symptoms (i.e., HADS ≥ 8). No significant relationship was found between active commute and anxiety symptoms in bivariate analysis while sport participation was associated with a 40% lower risk of elevated anxiety. The benefit of sport participation on anxiety remained, even after accounting for relevant demographic and clinical variables (adjusted OR=0.769; 95% CI 0.654 - 0.906; p = 0.002) (Table 1). Conclusion: Sport participation is associated with fewer symptoms of anxiety. In conclusion, individualized sport-related exercise prescriptions may reduce anxiety symptoms in cardiac patients.