Background Adults with congenital heart disease (CHD) are at high risk of premature death, making advance care planning (ACP) crucial for aligning care with individual values and goals. Previous ACP research has focused primarily on the United States and Canada, highlighting the need for a global perspective. We aimed to describe the ACP practices, needs and preferences of adults with CHD around the globe and to investigate associations with patient-related factors. Methods This cross-sectional study, part of the APPROACH-IS II project, assessed ACP preferences, needs and practices using patient-reported surveys. Overall, 8,281 patients with CHD (median age 32 years; 54% women; 15% mild, 58% moderate, 27% complex CHD) from 53 centers in 32 countries, spanning 6 continents, were included. Results Over half (55%) of participants reported speaking to their physician about how their health might be in the future and 9% had preferences being documented in a plan. According to 66% of patients, the best time to initiate ACP is early in the disease trajectory. Most patients indicated being relatively comfortable talking to their physician about their future health and about death. ACP varied widely across different countries, with United States and Canada top of the class for most variables. Conclusions When looking at global ACP practices, needs and preferences, much room for improvement of ACP provision could be noticed. Also, a notable variation in ACP was observed worldwide.

BACKGROUND:The global prevalence of congenital heart disease (CHD) is increasing. Research on patient-reported outcomes (PROs) predominantly originates from high-income countries, resulting in an incomplete understanding of the true global burden of CHD from the patient perspective. Therefore, we described perceived health, psychological distress and quality of life (QoL) in a large sample of adults with CHD from the globe and explored the relationship between PROs and the income level of the countries. METHODS:Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart Disease-International Study II (APPROACH-IS II) represents an international cross-sectional investigation of PROs in 8415 patients from 53 centres across 32 countries. Patients completed questionnaires to measure perceived health status (RAND-12 Health Survey; EuroQOL-5D Visual Analog Scale); depressive symptoms (Patient Health Questionnaire-8, PHQ-8); anxiety (Generalized Anxiety Disorder Scale-7) and QoL (Linear Analog Scale). Gross National Income per capita in US dollars was used for stratifying countries according to income levels. RESULTS:Large intercountry disparities in PROs were observed. Switzerland demonstrated the highest mean scores for physical functioning, self-rated health and QoL, while Senegal had the lowest scores. Patients from Malta demonstrated the highest mean scores for mental health, and Senegal had the lowest scores. With regard to depressive symptoms and anxiety, Pakistan had the lowest mean scores, while Turkey had the highest scores. Patients from high-income nations reported significantly better physical functioning, mental functioning and QoL. CONCLUSION:Large intercountry disparities in PROs were observed. APPROACH-IS II is a pioneering international endeavour that comprehensively evaluated PROs among adults with CHD, drawing participants from nations with different income levels. TRIAL REGISTRATION NUMBER:NCT04902768.

BACKGROUND:In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardiovascular 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.

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.

Background: Inter-country variation in patient-reported outcomes of adults with congenital heart disease has been observed. Country-specific characteristics may play a role. A previous study found an association between healthcare system performance and patient-reported outcomes. However, it remains unknown which specific components of the countries’ healthcare system performance are of importance for patient-reported outcomes. Aims: The aim of this study was to investigate the relationship between components of healthcare system performance and patient-reported outcomes in a large sample of adults with congenital heart disease. Methods: A total of 1591 adults with congenital heart disease (median age 34 years; 51% men; 32% simple, 48% moderate and 20% complex defects) from eight European countries were included in this cross-sectional study. The following patient-reported outcomes were measured: perceived physical and mental health, psychological distress, health behaviours and quality of life. The Euro Health Consumer Index 2015 and the Euro Heart Index 2016 were used as measures of healthcare system performance. General linear mixed models were conducted, adjusting for patient-specific variables and unmeasured country differences. Results: Health risk behaviours were associated with the Euro Health Consumer Index subdomains about patient rights and information, health outcomes and financing and access to pharmaceuticals. Perceived physical health was associated with the Euro Health Consumer Index subdomain about prevention of chronic diseases. Subscales of the Euro Heart Index were not associated with patient-reported outcomes. Conclusion: Several features of healthcare system performance are associated with perceived physical health and health risk behaviour in adults with congenital heart disease. Before recommendations for policy-makers and clinicians can be conducted, future research ought to investigate the impact of the healthcare system performance on outcomes further.

Patients with a systemic right ventricle are prone to develop heart failure. Beneficial effects of ACE-inhibition, angiotensin- or betareceptor blockade (advanced heart failure treatment, AHFT) are well established for patients with heart failure due to systemic left ventricular dysfunction, however

Background Secundum atrial septal defect (ASD2) is one of the most common cardiac malformations diagnosed in adult life. Catheter closure has made treatment possible even in patients of high age. However, published outcome data for elderly patients is limited. The aim of this study was to report, on a national basis, the long-term outcome of ASD2 catheter closure in the elderly. Material and results We report the clinical and echocardiographic outcome of catheter closure of ASD2 in 148 patients aged 65–87 years. Data was obtained from a national registry, medical records and a questionnaire. The proportion of patients in NYHA I increased from 34% to 61% (p < 0.001) one year after closure and remained stable at the latest follow-up 4,4 (SD 2,6) years post-closure. The proportion of patients with moderate/severe enlargement of the right ventricle and atrium fell from 77% and 76% to 25% and 40%, respectively, (p < 0.001) and right ventricular systolic pressure dropped significantly. Improvement of NYHA class was associated with reduced right ventricular systolic pressure but not with remodelling of the right heart. NYHA deteriorated in 9 patients, despite reduced right ventricular size. Overall, the prevalence of atrial fibrillation was unchanged after closure. Major complication rate was 2% and there was no procedure- or device-related mortality. Conclusion Catheter closure of ASD2 in the elderly is a worthwhile procedure since it improves symptoms and has a low complication rate. However, a subset of patients do not improve, in which we suggest that concealed left ventricular dysfunction may play a causative role.

Measuring quality of life (QOL) is fundamental to understanding the impact of disease and treatment on patients’ lives. This study aimed to explore QOL in an international sample of adults with congenital heart disease (CHD), the association between patient characteristics and QOL, and international variation in QOL and its relationship to country-specific characteristics. We enrolled 4,028 adults with CHD from 15 countries. QOL was assessed using a linear analog scale (LAS) (0 to 100) and the Satisfaction with Life Scale (SWLS) (5 to 35). Patient characteristics included sex, age, marital status, educational level, employment status, CHD complexity, and patient-reported New York Heart Association (NYHA) functional class. Country-specific characteristics included general happiness and 6 cultural dimensions. Linear mixed models were applied. Median QOL was 80 on the LAS and 27 on the SWLS. Older age, lack of employment, no marriage history, and worse NYHA functional class were associated with lower QOL (p < 0.001). Patients from Australia had the highest QOL (LAS: 82) and patients from Japan the lowest (LAS: 72). Happiness scores and cultural dimensions were not associated with variation in QOL after adjustment for patient characteristics and explained only an additional 0.1% of the variance above and beyond patient characteristics (p = 0.56). This large-scale, international study found that overall QOL in adults with CHD was generally good. Variation in QOL was related to patient characteristics but not country-specific characteristics. Hence, patients at risk for poorer QOL can be identified using uniform criteria. General principles for designing interventions to improve QOL can be developed.

Background: The last decade has seen a vast increase in the use of patient-reported outcomes. As patientreported outcomes are used in order to capture patients' perspectives of their health and illness, it is a prerequisite for accurate patient-reported outcome evaluations to use representative samples. In order to evaluate representativeness, the present study focussed on the comparison between participants and non-participants in the Swedish branch of the international study APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study), regarding demographic, clinical, and health status characteristics. Methods: Eligible patients for APPROACH-IS were identified and selected from SWEDCON, the Swedish registry for congenital heart disease (CHD). Overall, 912 eligible patients were identified, of whom 471 participated, 398 did not participate, and 43 were either unreachable or declined to participate in APPROACH-IS. The participants and nonparticipants were compared in terms of statistical significance and effect sizes. Results: Significant differences were observed between participants and non-participants for sex, age, primary diagnosis, number of cardiac operations, and fatigue; however, the effect sizes were in general small, except for the difference in primary diagnosis. No differences between the two groups were found in number of catheterisations, implanted device, the distribution of NYHA functional class, or health status and symptoms. Conclusions: This study shows that participants and non-participants are relatively comparable groups, which confirms the representativeness of the participants. The Swedish data from APPROACH-IS can therefore be reliably generalised to the population of adults with CHD in Sweden.

BACKGROUND Measuring quality of life (QOL) is fundamental to understanding the impact of disease and treatment on patients' lives.OBJECTIVES This study aimed to explore QOL in an international sample of adults with congenital heart disease (CHD), the association between patient characteristics and QOL, and international variation in QOL and its relationship to country-specific characteristics.METHODS We enrolled 4,028 adults with CHD from 15 countries. QOL was assessed using a linear analog scale (LAS) (0 to 100) and the Satisfaction with Life Scale (SWLS) (5 to 35). Patient characteristics included sex, age, marital status, educational level, employment status, CHD complexity, and patient-reported New York Heart Association (NYHA) functional class. Country-specific characteristics included general happiness and 6 cultural dimensions. Linear mixed models were applied.RESULTS Median QOL was 80 on the LAS and 27 on the SWLS. Older age, lack of employment, no marriage history, and worse NYHA functional class were associated with lower QOL (p < 0.001). Patients from Australia had the highest QOL (LAS: 82) and patients from Japan the lowest (LAS: 72). Happiness scores and cultural dimensions were not associated with variation in QOL after adjustment for patient characteristics and explained only an additional 0.1% of the variance above and beyond patient characteristics (p = 0.56).CONCLUSIONS This large-scale, international study found that overall QOL in adults with CHD was generally good. Variation in QOL was related to patient characteristics but not country-specific characteristics. Hence, patients at risk for poorer QOL can be identified using uniform criteria. General principles for designing interventions to improve QOL can be developed. (C) 2016 by the American College of Cardiology Foundation.

Background— The atrial switch operation, the Mustard or Senning operation, for the transposition of the great arteries (TGA) was introduced in the late 1950s and was the preferred surgery for TGA until the early 1990s. The Mustard and Senning operation involves extensive surgery in the atria and leaves the right ventricle as the systemic ventricle. The Mustard and Senning cohort is now well into adulthood and we begin to see the long-term outcome. Methods and Results— All the 6 surgical centers that performed Mustard and Senning operations in Sweden and Denmark identified all operated TGA patients. Information about death was obtained in late 2007 and early 2008 from the Danish and Swedish Centralised Civil Register by using the patients’ unique national Civil Registration Numbers. Four hundred sixty-eight patients undergoing the atrial switch operation were identified. Perioperative 30-day mortality was 20%, and 60% were alive after 30 years of follow-up. Perioperative mortality was significantly increased by the presence of a ventricular septal defect, left ventricular outflow obstruction, surgery early in the Mustard and Senning era. However, only pacemaker implantation is predictive of long-term outcome (hazard ratio, 1.90; 95% confidence interval, 1.05–3.46, P =0.04), once the TGA patient has survived the perioperative period. The risk of reoperation was correlated to the presence of associated defects and where the first Mustard/Senning operation was performed. Conclusions— The long-term survival of patients with Mustard and Senning correction for TGA appears to be primarily determined by factors in the right ventricle and tricuspid valve and not the timing of or the type of surgery in childhood. Cardiac function necessitating the implantation of a pacemaker is associated with an increase in mortality.

Patients with mechanical heart valves (MHV) undergoing invasive procedures often receive periprocedural bridging with low-molecular-weight heparin (LMWH). The bridging strategies used in real-life and the predictors for bleeding and thrombosis are not well studied. We retrospectively assessed patients with MHV that underwent invasive procedures requiring vitamin K antagonist interruption and LMWH bridging. Thromboembolic and bleeding events occurring up to 30 days after the procedures were recorded. Predictors of major bleeding events (MBEs) were analyzed with logistic regression. We evaluated 547 patients with MHV who underwent 275 procedures during a 6.5-year period. Bridging with LMWH was used in 185 procedures in a total of 117 patients. Combined pre- and post-operative bridging was the most frequently employed (63 %). Doses of LMWH were prophylactic in 96 (52 %) of the procedures and therapeutic in 89 (48 %). The procedure-related bleeding risk was evaluated as high in 70 (38 %) and low in 115 (62 %) of the procedures. There was a trend to more frequent use of prophylactic doses (61 %) in high-risk surgery, and more therapeutic doses (53 %) in low-risk ones. There were 36 bleeding episodes, 21 (11 % of procedures) of which were classified as MBEs, but there were no thromboembolic events. Most MBEs (n = 14; 67 %) occurred in surgeries with high bleeding risk. In the multivariate analysis, the bleeding risk of the surgery itself was the only independent predictor for MBEs. For patients with MHV receiving perioperative bridging with LMWH, the major predictor for MBE is the bleeding risk of the surgery.

Population-based study of long-term outcome of mustard/senning correction for transposition of the great arteries (TGA)

Purpose: Despite the relative high prevalence of congenital aortic valve disease, the outcome in contemporary cohorts of adults is not well established. In the present study, we have analysed data in the National Registry on Adult Congenital Heart Disease in order to elucidate the long-term outcome regarding functional class, symptoms, quality of life, medications and need for arrhythmia devices in this cohort. Methods: Six hundred fifty-one adult patients with isolated congenital aortic valve disease met the criteria and were grouped according to: if their first aortic valve intervention was < 18 years (group 1) (n=152), first aortic valve intervention > 18 years (group 2) (n=129) or no aortic valve intervention (group 0) (n=370). Results: 92% of the patients were in NYHA I. Symptoms were reported in 12.7% but more commonly in group 2 compared with group 1 (20.7% vs. 9.6%, p = 0.039). The overall quality of life assessed with EQ-VAS was 90% and equal between groups. The use of cardiovascular medications, anticoagulation excluded, was higher in group 2 than in groups 0 and 1 (29.1% vs. 9.1% and 11.6%, p = 0.001, p < 0.001). Warfarin was prescribed in 55.3% of the patients in group 2, in 34.5% in group 1 and 1.7% in group 0 (p < 0.001 for all comparisons) which indicates that non-mechanical valve prostheses or other alternatives are common in group 1 and 2. Implanted arrhythmia devices were more common in group 2 compared with group 0 (5.1 vs. 0.6%, p = 0.01). Conclusion: Functional status and quality of life is generally good and not obviously related to previous interventions. Symptoms, cardiovascular medications, including warfarin, and anti-arrhythmia devices were more common in patients with their initial valve intervention in adult age. Many patients with a previous intervention have alternatives to mechanical heart valve prostheses and may thus need future re-interventions.

Introduction: Treatment strategy of patients with acute coronary syndrome presenting with STEMI/LBBB has changed over time from thrombolysis to primary PCI (pPCI). Methods: Patients (n=116982) treated in Sweden 1995-2011 in SWEDEHEART, a national registry in Sweden, were compared in terms of reperfusion strategy over time, by gender and age and for bleeding complications. Results: The proportion of patients undergoing acute reperfusion increased from 64% to 83% among patients Corresponding numbers including all ages were for thrombolysis 52% vs 3.6% pPCI in 1995 and 4.1% vs 61.8% in 2011. The proportion of women undergoing reperfusion increased from 52% to 61% and among men from 59% to 71% in 2011 and in lower frequency among patients >80 years for both genders. Acute angiography but without reperfusion occurred on average in 2-3%. Acute CABG occurred only in around 0.1% per year. During the same period, in-hospital fatal or intracerebral bleeding complications decreased from 0.7% to 0.15% (all ages). Bleedings requiring surgery or transfusion has remained stable over time. Conclusions: Reperfusion strategy has changed dramatically over time from mainly thrombolysis in 1995 to primary PCI in 2011, with the only exception of remote areas with long transportation times. During the same period, fatal/intracerebral bleeding complications have decreased. The change is consistent in both men and women and for all ages.

The aim was to evaluate effects on bone mineral content (BMC) in children with severe cerebral palsy (CP) standing on a self-controlled dynamic platform (vibrations, jumps and rotation), assess reactions expressed and record negative effects. An experimental design was used. Four children with severe CP participated. Two children used the platform for 8-9 months while two children were controls (period I). After 1 year, the former users were controls (period II). Dual-energy X-ray absorptiometry was performed. Children in period I (Child 1/Child 2) were exposed to whole body vibration for 330/394 min on 28/25 occasions and showed a percentage change in BMC values at the lumbar spine of + 35/+ 23% (versus controls, Child 3/Child 4, -9/+ 7%), left legs-9/-12% (vs. -2/-12%) and right legs + 61/+ 34% (vs.-18/+ 10%). Children in period II (Child 3/Child 4) were exposed for 524/635 min on 57/64 occasions. The corresponding percentage change in BMC values at the lumbar spine was + 10/+ 10% (+ 21/+ 5%), left legs +26/+ 22% (0/+5%) and right legs +26/+17% (+15/-1%). The children's reactions were perceived positive. No negative effects were recorded. Standing on a self-controlled dynamic platform may be an enjoyable method to increase BMC in children with severe CP.

PURPOSE The aim of this study was to describe physical activity, quality of and satisfaction with life, pain, joint mobility and muscle function in adults with mild-to-moderate osteogenesis imperfecta (OI) to form the basis of improved clinical care and physical therapy treatment. METHOD A total of 40 men and women aged between 21 and 71 years were identified and a prospective, cross-sectional study was performed on 29 (18 women) included participants. The participants had to be able to walk and to have a diagnosis of mild-to-moderate OI. Self-administered questionnaires and clinical examinations were used. RESULTS Difficulties were found in all domains of the International Classification of Functioning, Activity and Health. Pain was reported in 25 of 29 participants and scoliosis was found in 23 participants. Difficulty to run was estimated in 18 participants. A total of 19 of 27 participants reported reaching the recommendations of 30 min of moderate-intensity activity preferably every day. Life satisfaction was high even though health-related quality of life, assessed with the Short Form 36, was significantly lower than the Swedish norm. CONCLUSION Impairments and activity limitations involved pain, scoliosis, contractures as well as trouble with running, heavy lifting, heavy work and sports. This study show that individuals with mild-to-moderate OI perceive themselves as having decreased health-related quality of life and this seems to depend on decreased physical functioning. Despite that, as a group, they estimated high life satisfaction and 19 participants reported adhering to the general recommendation of 30 min of moderate-intensity activity preferably every day.

Method. aEuro integral A nominated sample of 18 obese adolescents (12 girls, 14--16 years, body mass index (BMI) 25--47.4 kg center dot center dot m<SU--2</SU), recruited from a paediatric obesity clinic, participated in semi-structured interviews. These were analysed using a phenomenographic research approach. Purposeful sampling reflected variations in age, gender, degree of obesity, weight loss achievement, ethnicity, time of registration and socioeconomic status.Results. aEuro integral The adolescents expressed numerous physical, psychological and social disabilities as a consequence of their obesity. Qualitatively different ways of perceiving and responding to obesity treatment were identified and could be described in six categories of descriptions and a two-dimensional construct for interpreting these categories; (a) personal empowerment, (b) despair and disappointment, (c) safety and relief, (d) ambivalence and uncertainty, (e) acceptance and realisation and (f) shame and guilt. The distinguishing structures to be found between the categories were as follows: focus on the individual and focus on body weight.Conclusions. aEuro integral Treatment strategies must consider the large impact obesity has on adolescents'' lives. It is necessary to engage the obese adolescent personally in the treatment process and to focus on the adolescents'' personal needs, goals and motive for weight reduction.