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Background: A majority of adults with congenital heart disease (CHD) have reduced exercise capacity and do not reach the recommended level of physical activity. A physically active lifestyle is essential to maintain health and counteract acquired cardiovascular disease. This study illuminates aspects that may be relevant for performing physical activity.

Purpose: To describe facilitators and barriers for physical activity in adults with CHD.

Methods: Semi-structured interviews were performed individually with fourteen adults (age 19–68 years, women=7) with complex CHD. The interviews were analyzed using qualitative content analysis.

Results: Aspects that may enable or inhibit physical activity were found in two domains; Facilitators and Barriers, which both consisted of four categories physical, psychological, psychosocial and environmental aspects (Table 1).

This can be exemplified by the category physical aspects; where persons expressed being limited by the CHD to perform physical activity, but also that improved aerobic fitness allows for being more active, and in the category psychosocial aspects; the person's previous negative experiences and lack of support constituted barriers while encouragement from others and being active as a child facilitated an active lifestyle in adult age.

Conclusion: The present study identifies barriers and facilitators for being physically active in adults living with CHD. It is essential to identify prerequisites for supporting and promoting physical activity and thereby hopefully prevent long-term adverse outcomes. Barriers can potentially be transformed to facilitators through increased knowledge in both the adult with CHD and the healthcare provider.

Background: In general, adults with congenital heart disease (CHD) have impaired exercise capacity, and approximately 50% do not reach current recommendations on physical activity. Herein we analysed factors associated with physical activity level (PAL) in adults with CHD by using patient-reported outcomes (PRO). Methods: Patients with CHD (n = 471) were randomly selected from the national register on CHD and categorized according to complexity of lesions -simple (n = 172, 39.1 +/- 14.6 years), moderate (n = 212, 39 +/- 14.1 years), and severe (n = 87, 31.7 +/- 10.7 years). Participants completed a standardized questionnaire measuring PRO-domains including PAL. Variables associated with PAL were tested in multivariate logistic regression. Results: PAL was categorized into high (>= 3 METs = 2.5 h/week, n = 192) and low (>= 3 METs <2.5 h/week, n = 279). Patients with low PAL were older (42.6 vs. 35.8 years, p = 0.001), had more prescribed medications (51% vs. 39%, p = 0.009), more symptoms (25% vs. 16%, p = 0.02) and comorbidity (45% vs. 34% p= 0.02). Patients with low PAL rated a lower quality of life (76.6 vs. 83.4, p < 0.001), satisfaction with life (25.6 vs. 27.3, p = 0.003), a lower Physical Component Summary score (PCS) (78.1 vs. 90.5, p < 0.001) andMental Component Summary score (MCS) (73.5 vs. 79.5, p < 0.001). Complexity of heart lesion was not associated with PAL. The included PROs-separately tested in the model, together with age were associated with PAL. Conclusions: PCS and MCS are stronger associated with PAL than age and medical factors. The use of these PROs could therefore provide valuable information of benefit for individualized advice regarding physical activity to patients with CHD.

Background: In general, adults with congenital heart disease have reduced exercise capacity and many do not reach the recommended level of physical activity. A physically active lifestyle is essential to maintain health and to counteract acquired cardiovascular disease, therefore enablers and barriers for being physically active are important to identify.

Aim: To describe what adults with complex congenital heart diseases consider as physical activity, and what they experience as enablers and barriers for being physically active.

Methods: A qualitative study using semi-structured interviews in which 14 adults with complex congenital heart disease (seven women) participated. The interviews were analysed using qualitative content analysis.

Results: The analysis revealed four categories considered enablers and barriers – encouragement, energy level, approach and environment. The following is exemplified by the category encouragement as an enabler: if one had experienced support and encouragement to be physically active as a child, they were more positive to be physically active as an adult. In contrast, as a barrier, if the child lacked support and encouragement from others, they had never had the opportunity to learn to be physically active.

Conclusion: It is important for adults with congenital heart disease to have the opportunity to identify barriers and enablers for being physically active. They need knowledge about their own exercise capacity and need to feel safe that physical activity is not harmful. This knowledge can be used by healthcare professionals to promote, support and eliminate misconceptions about physical activity. Barriers can potentially be transformed into enablers through increased knowledge about attitudes and prerequisites.

Introduction: Several studies have shown that adults with congenital heart disease have reduced exercise capacity and do not reach the recommended daily level of physical activity. With this in view, it is of great importance to investigate how this population experiences physical activity. The aim of the study is to illuminate how adults with congenital heart disease describes themselves in relation to physical activity.

Methods: Semi-structured interviews with fourteen adults with complex congenital heart disease were performed. Patients were recruited from the clinic waiting list, based on their scheduled follow up and diagnosis. Interviews were analysed by qualitative content analysis.

Results: The overall theme It´s like balancing on a slackline illustrates how adults with congenital heart disease described themselves in relation to physical activity. The overall theme consists of four themes: Being an adventurer- enjoying the challenges of physical activity, Being a realist- adapting to physical ability, Beinga non-doer- lacking prerequisites for physical activity and Being an outsider- feeling excluded depending on physical ability.

Conclusions: The descriptions on themselves as a physically active were not constant or one-dimensional and the descriptions varied during the interviews, related to different time periods in life. It meant that they could described themselves as being an adventurer liking tough challenges, but at the same time describing themselves as being a non-doer with uncertainty over their physical strength. The findings point out specific factors for adults with CHD that might constitute as obstacles, but also possibilities for being physically active.

AIMS AND OBJECTIVES: To illuminate how adults with CHD describe themselves in relation to physical activity.

BACKGROUND: Several studies have shown that adults with congenital heart disease (CHD) have reduced exercise capacity and do not reach the recommended daily level of physical activity. With this in view, it is of immense importance to investigate how this population experiences physical activity.

DESIGN: Qualitative study with semi-structured interviews analysed with qualitative content analysis.

METHODS: Semi-structured interviews were individually performed with fourteen adults (women=7, age 19-68 years) with complex CHD. Patients were purposively recruited from the clinic waiting list, based on a scheduled follow-up and diagnosis.

RESULTS: The overall theme, It's like balancing on a slackline, illustrates how adults with CHD described themselves in relation to physical activity. This overall theme consisted of four subthemes: (1) Being an adventurer- enjoying the challenges of physical activity; (2) Being a realist- adapting to physical ability; (3) Being a non-doer- lacking prerequisites for physical activity; and (4) Being an outsider- feeling excluded depending on physical ability.

CONCLUSIONS: Adults with CHD seem to have a diverse relationship to physical activity and it involves various aspects throughout the lifespan. The findings point out factors that might constitute as obstacles for being physically active, specific for people with chronic conditions like CHD. This highlights the importance of further exploring the hindering and facilitating factors for being physically active in order to get a deeper understanding of how to support adults with CHD to be physically active.

RELEVANCE TO CLINICAL PRACTICE: Given the diverse relationship to physical activity, nurses have to further investigate the patients' relationship to physical activity, in order to support a healthy lifestyle. Nurses and allied health professionals should offer individualized exercise prescriptions and education about suitable physical activities in relation to physical ability. This article is protected by copyright. All rights reserved.

Background: Many adults with congenital heart disease (CHD) have reduced aerobic exercise capacity and impaired muscle function. However, it is largely unknown which factors have influence on the confidence to perform exercise training, i.e. Exercise Self-Efficacy (ESE).

Aims: To identify factors related to low ESE, and thus identify potential targets for rehabilitation and thereby enhance the potential for being physically active.

Methods: Seventy-nine adults with CHD; simple lesions n=38 (women n=16), complex lesions n=41 (women n=17) (mean age 36.7±14.6 years) and 42 age and sex matched controls were recruited. All participants completed questionnaires on ESE, quality of life (EQ-5D), and physical activity (international physical activity questionnaire, IPAQ), and performed muscle endurance tests.

Results: ESE was categorised into low (<26 points, n=24) and high (≥26 points, n=55). Patients with low ESE were older (45.2±15.4 vs. 32.6±12.5 years, p=0.002), more often had prescribed medication (67% vs. 44%, p=0.06), higher New York Heart Association functional class (NYHA) (≥ III) (25% vs. 7%, p=0.03) and performed fewer shoulder flexions (30.9±16.1 vs. 45.9±23.9, p=0.01) compared with those with high ESE. In the high ESE group, ESE did not differ from controls (33.8±3.9 vs. 33.4±6.1, p=0.74). In linear multivariate analysis age (B;-0.18, 95% CI -0.28- -0.08), smoking (B;-3.73, 95% CI -7.17- -0.28), EQ-5Dindex <1 (B;-3.33, 95% CI -6.08- -0.57) and number of shoulder flexions (B; 0.09, 95% CI 0.03–0.16) were independently associated with ESE.

Conclusion: Many adults with CHD have low ESE. Rehabilitation targeting quality of life, smoking cessation and muscle training may improve ESE, and thus enhance the potential for being physically active in this population.

Background: Physical activity improves health, exercise tolerance and quality of life in adults with congenital heart disease (CHD), and exercise training is in most patients a high-benefit low risk intervention. However, factors that influence the confidence to perform exercise training, i.e. exercise self-efficacy (ESE), in CHD patients are virtually unknown. We aimed to identify factors related to low ESE in adults with CHD, and potential strategies for being physically active.

Methods: Seventy-nine adults with CHD; 38 with simple lesions (16 women) and 41 with complex lesions (17 women) with mean age 36.7 ± 14.6 years and 42 matched controls were recruited. All participants completed questionnaires on ESE and quality of life, carried an activity monitor (Actiheart) during four consecutive days and performed muscle endurance tests.

Results: ESE in patients was categorised into low, based on the lowest quartile within controls, (≤ 29 points, n = 34) and high (> 29 points, n = 45). Patients with low ESE were older (42.9 ± 15.1 vs. 32.0 ± 12.4 years, p = 0.001), had more complex lesions (65% vs. 42%, p = 0.05) more often had New York Heart Association functional class III (24% vs. 4%, p = 0.01) and performed fewer shoulder flexions (32.5 ± 15.5 vs. 47.7 ± 25.0, p = 0.001) compared with those with high ESE. In a logistic multivariate model age (OR; 1.06, 95% CI 1.02-1.10), and number of shoulder flexions (OR; 0.96, 95% CI 0.93-0.99) were associated with ESE.

Conclusion: In this study we show that many adults with CHD have low ESE. Age is an important predictor of low ESE and should, therefore, be considered in counselling patients with CHD. In addition, muscle endurance training may improve ESE, and thus enhance the potential for being physically active in this population.

Background: Most adult patients with congenital heart disease (CHD) have reduced aerobic exercise capacity. Their habitual physical activity (PA) level is, however, less well studied. In this study habitual PA level in a cohort of adults with CHD compared to healthy age and gender matched controls was investigated.

Methods: Eighty adults with CHD, classed as either “complex” (n=40) or “simple” (n=40), and 42 healthy controls were studied with a combined uniaxial accelerometer and heart rate monitor worn during 4 consecutive days. We analysed 1) the time spent during ≥ moderate/vigorous PA, 2) accelerometer counts/day and 3) to what extent the World Health Organization recommendations on PA were reached.

Results: Patients with simple lesions had higher total accelerometer counts/day compared to both patients with complex lesions and controls (simple lesions; median (IQR) 107.7(63.4) vs. complex lesions; 72.8(53.5) and controls; 78.3(49.6), p≤0.001 and p=0.002). Furthermore, no differences in time spent during ≥ moderate-to-vigorous PA was found between patients and controls. In addition 46% of the patients with simple lesions, 55% of the patients with complex lesions and 44% of the controls did not reach the W.H.O.-recommended level of daily PA, but no significant differences between groups were found. There were no differences in achieving recommended PA level between patients in NYHA I vs. NYHA II+III.

Conclusions: Patients with CHD follow the same PA-level pattern as the general population. Broad strategies promoting an active lifestyle are needed across the population and especially for patients with complex CHD and impaired NYHA class.

Background: Life expectancy of patients with congenital heart disease (CHD) has increased rapidly, resulting in a growing and aging population. Recent studies have shown that older people with CHD have higher morbidity, health care use, and mortality. To maintain longevity and quality of life, understanding their evolving medical and psychosocial challenges is essential.

Objectives: The authors describe the frailty and cognitive profile of middle-aged and older adults with CHD to identify predictor variables and to explore the relationship with hospital admissions and outpatient visits.

Methods: Using a cross-sectional, multicentric design, we included 814 patients aged ≥40 years from 11 countries. Frailty phenotype was determined using the Fried method. Cognitive function was assessed by the Montreal Cognitive Assessment.

Results: In this sample, 52.3% of patients were assessed as robust, 41.9% as prefrail, and 5.8% as frail; 38.8% had cognitive dysfunction. Multinomial regression showed that frailty was associated with older age, female sex, higher physiologic class, and comorbidities. Counterintuitively, patients with mild heart defects were more likely than those with complex lesions to be prefrail. Patients from middle-income countries displayed more prefrailty than those from higher-income countries. Logistic regression demonstrated that cognitive dysfunction was related to older age, comorbidities, and lower country-level income.

Conclusions: Approximately one-half of included patients were (pre-)frail, and more than one-third experienced cognitive impairment. Frailty and cognitive dysfunction were identified in patients with mild CHD, indicating that these concerns extend beyond severe CHD. Assessing frailty and cognition routinely could offer valuable insights into this aging population.

Objectives: The aim included investigation of the associations between sedentary (SED), low-intensity physical activity (LIPA), moderate-to-vigorous intensity PA (MVPA) and the prevalence of subclinical atherosclerosis in both coronaries and carotids and the estimated difference in prevalence by theoretical reallocation of time in different PA behaviours.

Design: Cross-sectional.

Setting: Multisite study at university hospitals.

Participants: A total of 22 670 participants without cardiovascular disease (51% women, 57.4 years, SD 4.3) from the population-based Swedish CArdioPulmonary bioImage study were included. SED, LIPA and MVPA were assessed by hip-worn accelerometer.

Primary and secondary outcomes: Any and significant subclinical coronary atherosclerosis (CA), Coronary Artery Calcium Score (CACS) and carotid atherosclerosis (CarA) were derived from imaging data from coronary CT angiography and carotid ultrasound.

Results: High daily SED (>70% ≈10.5 hours/day) associated with a higher OR 1.44 (95% CI 1.09 to 1.91), for significant CA, and with lower OR 0.77 (95% CI 0.63 to 0.95), for significant CarA. High LIPA (>55% ≈8 hours/day) associated with lower OR for significant CA 0.70 (95% CI 0.51 to 0.96), and CACS, 0.71 (95% CI 0.51 to 0.97), but with higher OR for CarA 1.41 (95% CI 1.12 to 1.76). MVPA above reference level, >2% ≈20 min/day, associated with lower OR for significant CA (OR range 0.61-0.67), CACS (OR range 0.71-0.75) and CarA (OR range 0.72-0.79). Theoretical replacement of 30 min of SED into an equal amount of MVPA associated with lower OR for significant CA, especially in participants with high SED 0.84 (95% CI 0.76 to 0.96) or low MVPA 0.51 (0.36 to 0.73).

Conclusions: MVPA was associated with a lower risk for significant atherosclerosis in both coronaries and carotids, while the association varied in strength and direction for SED and LIPA, respectively. If causal, clinical implications include avoiding high levels of daily SED and low levels of MVPA to reduce the risk of developing significant subclinical atherosclerosis.

Background: Cardiac rehabilitation (CR) programs addressing risk factor management, educational interventions, and exercise contribute to reduce mortality after myocardial infarction (MI). However, the fulfillment of guideline-recommended CR targets is currently unsatisfactory. eHealth, i.e., the use of electronic communication for healthcare, including the use of mobile smartphone applications combined with different sensors and interactive computerized programs, offers a new array of possibilities to provide clinical care. The present study aims to assess the efficacy of a web-based application (app) designed to support persons in adhering to lifestyle advice and medication as a complement to traditional CR programs for improvement of risk factors and clinical outcomes in patients with MI compared with usual care.

Methods/design: An open-label multi-center randomized controlled trial is being conducted at different CR centers from three Swedish University Hospitals. The aim is to include 150 patients with MI < 75 years of age who are confident smartphone and/or Internet users. In addition to participation in CR programs according to the usual routine at each center, patients randomized to the intervention arm will receive access to the web-based app. A CR nurse reviews the patients’ self-reported data twice weekly through a medical interface at the clinic. The primary outcome of the study will be change in submaximal exercise capacity (in watts) between 2 and 4 weeks after discharge and when the patient has completed his/her exercise program at the CR center, usually around 3–6 months post-discharge. Secondary outcomes include changes in self-reported physical activity, objectively assessed physical activity by accelerometry, self-rated health, dietary, and smoking habits, body mass index, blood pressure, blood lipids, and glucose/HbA1c levels between inclusion and follow-up visits during the first year post-MI. Additionally, we will assess uptake and adherence to the application, the number of CR staff contacts, and the incidence of cardiovascular events at 1 and 3 years after the MI. Patient recruitment started in 2016, and the first study results are expected in the beginning of 2019.

Discussion: The present study will add evidence to whether electronic communication can be used to improve traditional CR programs for patients after MI.

Introduction/aim: Young patients with Fontan circulation may have low serum 25-hydroxyvitamin D levels, an affected liver, and unhealthy body compositions. This study aimed to explore the association between vitamin D intake/levels, liver biomarkers, and body composition in young Fontan patients.

Method: We collected prospective data in 2017 to 2018, obtained with food-frequency questionnaires, biochemical analyses of liver biomarkers, and dual-energy X-ray absorptiometry scans in 44 children with Fontan circulation. Body compositions were compared to matched controls (n = 38). Linear regression analyses were used to investigate associations of biomarkers, leg pain, and lean mass on serum levels of 25-hydroxyvitamin D. Biomarkers were converted to z scores and differences were evaluated within the Fontan patients.

Results: Our Fontan patients had a daily mean vitamin D intake of 9.9 µg and a mean serum 25-hydroxyvitamin D of 56 nmol/L. These factors were not associated with fat or lean mass, leg pain, or biomarkers of liver status. The Fontan patients had significantly less lean mass, but higher fat mass than controls. Male adolescents with Fontan circulation had a greater mean abdominal fat mass than male controls and higher cholesterol levels than females with Fontan circulation.

Conclusion: Vitamin D intake and serum levels were not associated with body composition or liver biomarkers in the Fontan group, but the Fontan group had lower lean mass and higher fat mass than controls. The more pronounced abdominal fat mass in male adolescents with Fontan circulation might increase metabolic risks later in life.

Aim: To describe and compare self‐reported health‐related quality of life between younger and older patients with severe heart failure eligible for treatment with sacubitril–valsartan and to explore the association between health‐related quality of life and age, NYHA classification, systolic blood pressure and NT‐proBNP level.

Design: Cross‐sectional study.

Methods: A total of 59 patients, eligible for treatment with sacubitril–valsartan were consecutively included and divided into a younger (≤75 years) and older group (>75 years). Health‐related quality of life was assessed using the Kansas City Cardiomyopathy Questionnaire and the EuroQol 5‐dimensions. Data were collected between June 2016 and January 2018. The STROBE checklist was used.

Results: There were no differences in overall health‐related quality of life between the age groups. The older patients reported lower scores in two domains measured with the Kansas City Cardiomyopathy Questionnaire, namely self‐efficacy (67.0 SD 22.1 vs. 78.8 SD 19.7) and physical limitation (75.6 SD 19.0 vs. 86.3 SD 14.4). Higher NYHA class was independently associated with lower Kansas City Cardiomyopathy Questionnaire Overall Summary Score.

Objective: To investigate associations between physical activity and risk factors for cardiovascular disease (CVD), subclinical atherosclerosis, and disease activity in patients with early and long-standing rheumatoid arthritis (RA).

Method: This cross-sectional study included 84 patients with early and 37 with long-standing RA (disease duration, mean ± sd: 1.4 ± 0.4 and 16.3 ± 2.3 years, respectively). Physical activity was measured using a combined accelerometer and heart-rate monitor. Further assessments were disease activity (erythrocyte sedimentation rate, Disease Activity Score in 28 joints), functional ability (Health Assessment Questionnaire), risk factors for CVD (blood lipids, i.e. triglycerides, high-density lipoprotein, low-density lipoprotein; blood glucose, blood pressure, sleeping heart rate, waist circumference, body mass index, and body fat), and subclinical atherosclerosis (pulse-wave velocity, augmentation index, and carotid intima–media thickness).

Results: Physical activity variables did not differ between patients with early and long-standing RA. However, 37% of the patients with early and 43% of those with long-standing RA did not reach the World Health Organization’s recommended levels of moderate to vigorous physical activity (MVPA). In a final multiple regression model, adjusted for age, gender, disease duration, and activity monitor wear time, higher total physical activity was associated with lower body fat and higher functional ability. With the same adjustments, more time spent in MVPA was associated with lower high-density lipoprotein and lower sleeping heart rate.

Conclusions: Physical activity was associated with more favourable risk factors for CVD. However, many patients were physically inactive, stressing the importance of promoting physical activity in RA.

Objective: We aimed to determine relationships between objectively measured nightly sleep, sedentary behavior (SB), light physical activity (LPA), and moderate to vigorous physical activity (MVPA) with risk factors for cardiovascular disease (CVD) in patients with early rheumatoid arthritis (RA). Furthermore, we aimed to estimate consequences for these risk factors of theoretical displacements of 30 minutes per day in one behavior with the same duration of time in another.

Methods: This cross-sectional study included 78 patients with early RA. Nightly sleep, SB, LPA, and MVPA were assessed by a combined heart rate and accelerometer monitor. Associations with risk factors for CVD were analyzed using linear regression models and consequences of reallocating time between the behaviors by isotemporal substitution modeling.

Results: Median (Q1-Q3) nightly sleep duration was 4.6 (3.6-5.8) hours. Adjusted for monitor wear time, age, and sex, 30-minutes-longer sleep duration was associated with favorable changes in the values β (95% confidence interval [CI]) for waist circumference by -2.2 (-3.5, -0.9) cm, body mass index (BMI) by -0.9 (-1.4, -0.4) kg/m2 , body fat by -1.5 (-2.3, -0.8)%, fat-free mass by 1.6 (0.8, 2.3)%, sleeping heart rate by -0.8 (-1.5, -0.1) beats per minute, and systolic blood pressure by -2.5 (-4.0, -1.0) mm Hg. Thirty-minute decreases in SB, LPA, or MVPA replaced with increased sleep was associated with decreased android fat and lower systolic blood pressure levels. Replacement of SB or LPA with MVPA yielded lower BMIs.

Conclusion: Shorter sleep during the night is common among patients with early RA and is associated with adverse risk factors for CVD.

Background: Adults with congenital heart disease (CHD) have a low muscle mass (measured with Dual energy x-ray absorptiometry). Our aims were to confirm these results using peripheral quantitative computed tomography (pQCT), and to study the relationship between muscle cross sectional area (CSA) and isometric muscle strength. 

Methods: In this cross sectional study, we performed pQCT and tested isometric muscle strength in the forearm and calf of 49 adults with complex CHD and 49 age and sex matched controls (for each group: mean age 36±15.5 years; n=23 females, 47%). 

Results: In the forearm, after adjusting for height, patients had lower muscle CSA and lower isometric strength compared to controls (women: 24.1±6.7 vs. 26.2±6.7 cm2, p=0.05 and 30.0±1.0 vs. 33.4±1.0 kg, p=0.03; men: 36.0±1.2 vs. 42.5±1.2 cm2, p=0.001 and 47.2±1.9 vs. 53.4±1.9 kg, p=0.03). In addition, both patients and controls had strong correlations between muscle CSA and isometric strength. In the calf, female patients did not have lower muscle CSA than controls after correcting for height (60.5±1.4 vs. 62.7±1.4cm2, p=0.3), whereas men did (69.9±1.7 vs. 80.3±1.7 cm2, p<0.001). Isometric strength in the calf was lower in female patients than controls (487±35 vs. 614±35 N, p=0.02) but not in males (588±44 vs. 697±44 N, p=0.08), and there was no correlation with muscle CSA. 

Conclusion: In conclusion, adults with complex CHD have low forearm muscle CSA as assessed with pQCT. Grip strength correlates well with forearm muscle CSA and may serve as an indirect measurement of muscle mass.

Objective 

To study bone mineral density (BMD) and skeletal strength in adults with complex congenital heart disease (CHD) in comparison to a matched control group.

Methods

Peripheral quantitative computed tomography (pQCT) was performed on standardized sites of the radius and tibia in 49 adults with complex CHD, and in 49 age and sex matched controls (n = 23 [47 %] female, mean age 36 ± 15.5 years.) Strength for lateral and anterior acting forces in the radius and tibia were presented, respectively, in terms of x- and y-Strength-Strain Index (SSI).

Results 

Patients had similar total BMD as controls in both the radius (807 ± 82 vs. 792 ± 75 mg/cm3, p = 0.3) and tibia (663 ± 86 vs.689 ± 67 mg/cm3, p = 0.1). In the radius, patients had a lower xSSI than controls (154 ± 46 vs. 175 ± 54 mm3, p = 0.04) but a similar ySSI (178 ± 58 vs. 195 ± 55 mm3, p = 0.1). In the tibia, patients had a lower xSSI (1492 ± 399 vs. 1780 ± 372 mm3, p < 0.001) as well as ySSI (1066 ± 304 vs. 1250 ± 281, p = 0.002). In a multiple linear regression model, only height was independently associated with decreased skeletal strength in patients tibia in terms of xSSI [F(2,46) = 40.002, p < 0.001, R2 = 0.635]. Being a patient, with single ventricle physiology or other diagnose of complex CHD, was independently associated with decreased tibia xSSI [F(2,95) = 7.085, p = 0.001, η2 = 0.13].

Conclusion 

Adults with complex CHD had reduced strength in the radius and tibia, despite a normal BMD as assessed with pQCT. Whether their reduced bone strength entails increased risk of future fractures is currently unknown.

Background: We aimed to study bone mineral density (BMD) and skeletal strength in the growing population of adults with moderate or complex congenital heart disease (CHD).

Methods: Peripheral quantitative computed tomography (pQCT) was performed on the radius and tibia in 49 adults with moderate or complex CHD, and in 49 age and sex matched controls (n = 23 [47%] female, mean age 36 +/- 15.5 years.) Strength in the radius and tibia were presented, respectively, in terms of Strength-Strain Index (SSI).

Results: Patients had similar total BMD as controls in both the radius (807 +/- 82 vs. 792 +/- 75 mg/cm(3), p = 0.3) and tibia (663 +/- 86 vs. 689 +/- 67 mg/cm(3), p = 0.1). In the radius, patients had a lower xSSI than controls (154 +/- 46 vs. 175 +/- 54 mm(3), p = 0.04) but a similar ySSI (178 +/- 58 vs. 195 +/- 55 mm(3), p = 0.1). In the tibia, patients had a lower xSSI (1492 +/- 399 vs. 1780 +/- 372 mm(3), p < 0.001) as well as ySSI (1066 +/- 304 vs. 1250 +/- 281, p = 0.002). In a multiple linear regression model, only height was independently associated with decreased skeletal strength in patients tibia in terms of xSSI [F(2,46) = 40.002, p < 0.001, R-2 = 0.635].

Conclusion: Adults with moderate or complex CHD had reduced strength in the radius and tibia, despite a normal BMD as assessed with pQCT. Their reduced bone strength could increase the risk of future fractures.

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.

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 hours/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: 4028 adults with CHD recruited from 15 countries over 5 continents completed self-reported instruments, including the Health Behaviour Scale (HBS-CHD), within a multicenter 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, 27% reached the WHO recommendations but with a great variation between geographical areas (Japan: 9% - Norway: 49%) (Figure). Predictors for PAL in line with the WHO recommendations, with country of residence as random effect, were male sex (OR 1.68, 95% CI 1.43–1.97), NYHA-class I (OR 2.79, 95% CI 1.54–5.06) and less complex disease (OR 1.28, 95% CI 1.02–1.62). In contrast, older age (OR 0.97, 95% CI 0.97–0.98), lower educational level (OR 0.41, 95% CI 0.26–0.65) and being unemployed (OR 0.58, 95% CI 042–0.78) 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 PAL by country of origin. Based on identified predictors, vulnerable patients may be identified and offered specific behavioural interventions.

Background: Physical activity reduces the risk of acquired cardiovascular disease, which is of great importance in patients with congenital heart disease (CHD). There are diverging data whether physical activity level (PAL) differs between patients with CHD and controls. Furthermore, it is unknown if PAL can be reliably assessed in patients with CHD using self-reported instruments.

Methods: Seventy-five patients with CHD (mean age 37.5 ± 15.5 years, women n = 29 [38.7%]) and 42 age and sex matched controls completed the International Physical Activity Questionnaire (IPAQ) and carried the activity monitor Actiheart over 4 days. Time spent at ≥3 METS ≥21.4 min/day, i.e. reaching the WHO recommendation for PAL to promote health, was used as the outcome measure. Data on PAL obtained from IPAQ were compared with Actiheart.

Results: The proportion of individuals reaching target PAL according to IPAQ was similar in patients with CHD and controls (70.7%vs.76.2%, p = 0.52) as well as between patients with simple and complex lesions. There was an overall difference between IPAQ and Actiheart in detecting recommended PAL (72.6%vs.51.3%, p b 0.001). In a subgroup analysis, this difference was also detected in patients but was borderline for controls. The negative predictive value for IPAQ in detecting insufficient PAL was higher in patients than in controls (73%vs.40%).

Conclusions: The proportion of persons reaching sufficient PAL to promote health was similar in patients and controls. The self-reported instrument overestimated PAL in relation to objective measurements. However, with a high negative predictive value, IPAQ is a potentially useful tool for detecting patients with insufficient PAL.

Background: Atrial septal defect (ASD) is a congenital heart lesion that often remains undiagnosed until adulthood. The reasons for this may be multifactorial. It is, however, known that closure of a hemodynamically significant ASD improves exercise capacity. This study aimed to explore whether the aerobic capacity in late adolescence is associated with time to diagnosis and intervention in adult men with late diagnosis of an atrial shunt.

Methods: The Swedish Military Conscription Service Register contains data on exercise tests performed in late adolescence. By linking these data with the National Patient Register, 254 men with a later intervention for an ASD were identified.

Results: Interventions were performed at a mean of 26.5 +/- 7.9 years after the initial exercise tests. The mean absolute workload among those with a later diagnosed ASD was similar to those without a later diagnosed ASD (274 +/- 51W vs. 276 +/- 52 W, p = 0.49). Men with a higher exercise capacity (>= 1 SD) had their intervention earlier (21.9 +/- 8.6 years vs. 27.5 +/- 7.4 years, p < 0.001).

Conclusions: The aerobic exercise capacity was similar in adolescent men with later interventions for ASD compared to the reference population. Furthermore, those with high exercise capacity appeared to be diagnosed earlier. Thus, low exercise capacity may not be a feature of ASD during adolescence, but rather develop later in life as a natural progression of the disease.

Background: Cardiac rehabilitation is central in reducing mortality and morbidity after myocardial infarction. However, the fulfillment of guideline-recommended cardiac rehabilitation targets is unsatisfactory. eHealth offers new possibilities to improve clinical care.

Objective: This study aims to assess the effect of a web-based application designed to support adherence to lifestyle advice and self-control of risk factors (intervention) in addition to center-based cardiac rehabilitation, compared with cardiac rehabilitation only (usual care).

Methods: All 150 patients participated in cardiac rehabilitation. Patients randomized to the intervention group (n=101) received access to the application for 25 weeks where information about lifestyle (eg, diet and physical activity), risk factors (eg, weight and blood pressure [BP]), and symptoms could be registered. The software provided feedback and lifestyle advice. The primary outcome was a change in submaximal exercise capacity (Watts [W]) between follow-up visits. Secondary outcomes included changes in modifiable risk factors between baseline and follow-up visits and uptake and adherence to the application. Regression analysis was used, adjusting for relevant baseline variables.

Results: There was a nonsignificant trend toward a larger change in exercise capacity in the intervention group (n=66) compared with the usual care group (n=40; +14.4, SD 19.0 W, vs +10.3, SD 16.1 W; P=.22). Patients in the intervention group achieved significantly larger BP reduction compared with usual care patients at 2 weeks (systolic −27.7 vs −16.4 mm Hg; P=.006) and at 6 to 10 weeks (systolic −25.3 vs −16.4 mm Hg; P=.02, and diastolic −13.4 vs −9.1 mm Hg; P=.05). A healthy diet index score improved significantly more between baseline and the 2-week follow-up in the intervention group (+2.3 vs +1.4 points; P=.05), mostly owing to an increase in the consumption of fish and fruit. At 6 to 10 weeks, 64% (14/22) versus 46% (5/11) of smokers in the intervention versus usual care groups had quit smoking, and at 12 to 14 months, the respective percentages were 55% (12/22) versus 36% (4/11). However, the number of smokers in the study was low (33/149, 21.9%), and the differences were nonsignificant. Attendance in cardiac rehabilitation was high, with 96% (96/100) of patients in the intervention group and 98% (48/49) of patients receiving usual care only attending 12- to 14-month follow-up. Uptake (logging data in the application at least once) was 86.1% (87/101). Adherence (logging data at least twice weekly) was 91% (79/87) in week 1 and 56% (49/87) in week 25.

Conclusions: Complementing cardiac rehabilitation with a web-based application improved BP and dietary habits during the first months after myocardial infarction. A nonsignificant tendency toward better exercise capacity and higher smoking cessation rates was observed. Although the study group was small, these positive trends support further development of eHealth in cardiac rehabilitation.

Purpose: The purpose of this study was to examine the concurrent validity of a fixated hand-held dynamometer (HHD) in comparison to a load cell in measurement of maximal isometric knee extension force in adults with congenital heart disease (CHD) and healthy adults.

Methods: Fourteen adults with CHD and fourteen healthy adults were included. Each participant was tested three times with each method and the mean of the top two results for each participant and method was used in analysis.

Results: The agreement between the two methods was excellent in both groups (intraclass correlation coefficient [ICC], 95% confidence interval [CI]) 0.98 (0.92–1.00) in the CHD group and ICC 0.99 (0.96–1.00) in the healthy group). There was a small difference of 19.5 Newton or 4.8% (p<.05) between the two methods in the CHD group. No significant difference was seen between the two methods in the healthy group (p>.05).

Conclusions: The fixated HHD demonstrated excellent concurrent validity when compared to a load cell among adults with CHD as well as in healthy adults. Thus, in a healthy population the methods can be used interchangeably, however, a small difference between the methods is seen in the CHD group.

Background Adults with congenital heart disease (CHD) is a growing population and related to advances in surgical and medical treatment, they now outnumber the children with corresponding lesions. Since a congenital heart lesion often results in reduced exercise capacity, this population is a potential target for physiotherapy. To what extent this reduction in exercise capacity is caused by abnormal cardiovascular anatomy and physiology or to what degree insufficient physical activity contributes is not known. To support the advancements in paediatric cardiac care, increased knowledge regarding physical performance, physical activity level, body composition and the effects of exercise training among adults with CHD is required.

Methods In a cross-sectional study skeletal- and respiratory muscle function, physical activity level and exercise self-efficacy was investigated among 85 adults with various forms of CHD and 42 control subjects. A second study was conducted to analyse height, weight and body mass index (BMI) in 538 adults with complex CHD and 1886 adults with simple CHD. Data were extracted from the Swedish registry on congenital heart disease (SWEDCON) and compared to data from a national population survey. In a third study, factors associated with self-reported quality of life (QoL) were analysed using SWEDCON data on 315 adults with congenital aortic valve disease. Finally, a randomised controlled trial was conducted to investigate the effects of interval exercise training among adults with complex CHD.

Results Adults with complex CHD showed impaired muscle function compared to both patients with simple CHD and controls. In addition, patients with complex CHD had a lower exercise self-efficacy compared to controls. Patients with CHD were equally active at moderate-to-vigorous level as the controls. However, approximately 50% of both patients and controls failed to reach the recommended physical activity level. In general patients with CHD had the same height, weight and BMI, as the general population. However, compared to the general population, men with CHD were more commonly underweight and less commonly overweight/obese. Additionally, especially male patients with complex CHD were shorter compared to the general population. Among adults with congenital aortic valve disease, a higher physical activity level was associated with better QoL. Furthermore, interval training increased exercise capacity and endurance among adults with complex CHD.

Conclusion A higher physical activity level was associated with better self-reported QoL in patients with congenital aortic valve disease which implies that QoL might be possible to improve, by adopting a physically active life-style. Adults with CHD were equally active as controls at a moderate-to-vigorous physical activity level. However, approximately half of both groups were insufficiently physically active based on current recommendations. This indicates that low physical activity, on group level, does not explain the lower exercise capacity commonly found among patients with CHD. In addition, this is consistent the finding that the majority of patients followed the same pattern regarding BMI as seen in the general population. However, impaired muscle function in combination with the shorter stature and higher prevalence of underweight found in men, especially with complex CHD, implies an altered body composition in this group. The findings of the present thesis suggests an indication for physiotherapy targeting increased physical activity level and individualized exercise training in this patient population. Moreover, regular evaluation of muscle function, exercise self-efficacy and QoL, in addition to exercise capacity, might be useful for monitoring disease development over time.

BACKGROUND: Patients with CHD exhibit reduced isometric muscle strength and muscle mass; however, little is known how these parameters relate. Therefore, the aim was to investigate the relation between isometric limb muscle strength and muscle mass for patients in comparison to age- and sex-matched control subjects.

METHODS: Seventy-four patients (35.6 ± 14.3 years, women n = 22) and 74 matched controls were included. Isometric muscle strength in elbow flexion, knee extension, and hand grip was assessed using dynamometers. Lean mass, reflecting skeletal muscle mass, in the arms and legs was assessed with dual-energy x-ray absorptiometry.

RESULTS: Compared to controls, patients had lower muscle strength in elbow flexion, knee extension, and hand grip, and lower muscle mass in the arms (6.6 ± 1.8 kg versus 5.8 ± 1.7 kg, p < 0.001) and legs (18.4 ± 3.5 kg versus 15.9 ± 3.2 kg, p < 0.001). There was no difference in achieved muscle force per unit muscle mass in patients compared to controls (elbow flexion 0.03 ± 0.004 versus 0.03 ± 0.005 N/g, p = 0.5; grip strength 0.008 ± 0.001 versus 0.008 ± 0.001 N/g, p = 0.7; knee extension 0.027 ± 0.06 versus 0.028 ± 0.06 N/g, p = 0.5). For both groups, muscle mass in the arms correlated strongly with muscle strength in elbow flexion (patients r = 0.86, controls, r = 0.89), hand grip (patients, r = 0.84, controls, r = 0.81), and muscle mass in the leg to knee extension (patients r = 0.64, controls r = 0.68).

CONCLUSION: The relationship between isometric muscle strength and limb muscle mass in adults with CHD indicates that the skeletal muscles have the same efficiency as in healthy controls.

Background: Adults with complex congenital heart disease (CHD) show reduced aerobic exercise capacity and impaired skeletal muscle function compared with healthy peers. Peripheral muscle factors are presumed to be important contributors to the aerobic capacity, but the mechanisms are poorly understood. The aim of the present study was to investigate differences between adults with CHD and controls in muscle oxygenation kinetics at rest, and during and after exercise.

Methods: Seventy-four patients with complex CHD (mean age 35.6 +/- 14.3 years, female n = 22) were recruited. Seventy-four age- and sex-matched subjects were recruited as controls. Muscle oxygenation was successfully determined on the anterior portion of the deltoid muscle using near-infrared spectroscopy in 65 patients and 71 controls. Measurements were made at rest, during isotonic shoulder flexions (0-90 degrees) to exhaustion, and during recovery.

Background: Peripheral muscle factors are presumed to be contributors to the reduced exercise capacity incongenital heart disease (CHD), but the underlying mechanisms are poorly understood. The aim was to investigate if muscle oxygenation, at rest and during exercise, and the resting blood flow in the calf muscle is impaired in adults with complex CHD in comparison to controls.

Method: Seventy-four adults with complex CHD (35.6 ± 14.3 years, females n = 22 [30%], males n = 52 [70%]) and seventy-four age and sex matched subjects were recruited. Muscle oxygenation was successfully determined using near-infrared spectroscopy on the medial portion of m. gastrocnemius in 63 patients and 67 controls. Measurements were made at rest, during venous occlusion to estimate blood flow (indicated by slope increase of total haemoglobin, HbT), and post arterial occlusion. Additionally, measurements were made at the onset of isotonic unilateral heel-lifts to exhaustion and during recovery post exercise.

Results: Adults with CHD had a slower desaturation rate at exercise onset (-7.7 ± 4.3%StO₂x3.5sec^-1 vs. -11.7 ± 5.8%StO₂x3.5sec^-1, p < 0.001) a slower half recovery time (28.6 ± 21.2s vs. 16.8 ± 11.1s, p < 0.001) and a slower resaturation rate post exercise in comparison to the control subjects (3.9 ± 3.7%StO₂x3.5sec^-1 vs. 6.1 ± 3.8%StO₂x3.5sec^-1, p = 0.002). In contrast, there were no differences in muscle oxygen kinetics at rest.

Conclusion: Slower muscle oxygenation kinetics during muscle exercise found in adults with complex CHD may give insight to the mechanisms for the reduced exercise capacity commonly found in this population. This finding may also provide implications for design of exercise training programs targeting muscle function for these patients.

BACKGROUND: The prognosis in adults with congenital aortic valve disease is usually favourable; nevertheless, a number of medical and social factors might hamper long-term prognosis and quality of life. With a focus on physical exercise level, data from the Swedish National Registry on Congenital Heart Disease (SWEDCON) were analysed and variables associated with health-related quality of life in adults with congenital aortic valve disease were identified.

METHODS: In this registry study, SWEDCON was searched for adult patients with isolated congenital aortic valve disease and valid EuroQol-5Dimensions health questionnaire (EQ-5D) data.

RESULTS: This study identified 315 patients. The majority (n = 202, 64%) reported best possible health status (EQ-5Dindex = 1) whereas 113 (35%) reported some impairment (EQ-5Dindex < 1) with mean EQ-5Dindex 0.73 ± 0.17. In a multivariate logistic regression model, self-reported physical exercise > 3 h/week was independently associated with best possible health status (EQ-5Dindex = 1; p = 0.013). Moreover presence of cardiovascular symptoms (p < 0.001), active smoking (p = 0.002), history of valve surgery (p = 0.017), low educational level (p = 0.022), and higher systolic blood pressure (p = 0.029) were independently associated with impaired health status (EQ-5Dindex < 1).

CONCLUSIONS: Physical exercise >3 h/week was, as a single variable, associated with best possible health status in adults with congenital aortic valve disease. In contrast, a number of medical and social factors are associated with worse self-reported health status. Among these, symptoms, smoking, and educational level are potential targets for modification and intervention. There is a need for studies investigating the effect of increased level of physical exercise in patients with congenital aortic valve disease.

Introduction: Impaired isometric muscle strength was previously reported in adults with Fontan circulation. However, it is unclear if this impairment is present in children and adolescents with Fontan circulation. We investigated isometric muscle strength of the lower limb in patients (6–18 years) with Fontan circulation in comparison with healthy controls.

Method: In this cross-sectional study, 43 patients (6–18 years) with Fontan circulation and 43 age- and sex-matched controls were included. Isometric knee extension and plantar flexion muscle strength were assessed using dynamometry (Newton, N). Lean mass of the legs was assessed with dual-energy X-ray absorptiometry. Analyses were performed on group level (n = 43), and for subgroups that included children aged 6–12 years (n = 18) and adolescents aged 13–18 years (n = 25).

Results: On group level, the patients with Fontan circulation had impaired isometric knee extension strength in comparison with the controls (p = 0.03). In subgroup analyses, impaired isometric knee extension strength was present in the adolescents (p = 0.009) but not in the children groups. For plantar flexion, there was no difference between patients and controls. There was no difference in lean mass between patients and controls (9.6 ± 4.3 kg vs. 10.8 ± 5.6 kg, p = 0.31). However, the lean mass was highly correlated to isometric knee extension strength (patients r = 0.89, controls r = 0.96, p < 0.001) and isometric plantar flexion strength (patients r = 0.7, controls r = 0.81, p < 0.001).

Conclusion: The finding of impaired isometric knee extension muscle strength in adolescents (13–18 years) with Fontan circulation and no corresponding impairment in the children group (6–12 years) could imply that isometric muscle strength gets more impaired with age.

Objective: The beneficial effects of exercise training in acquired heart failure and coronary artery disease are well known and have been implemented in current treatment guidelines. Knowledge on appropriate exercise training regimes for adults with congenital heart disease is limited, thus further studies are needed. The aim of this study was to examine the effect of home‐based interval exercise training on maximal endurance capacity and peak exercise capacity.

Design: Randomized controlled trial.

Methods: Twenty‐six adults with complex congenital heart disease were recruited from specialized units for adult congenital heart disease. Patients were randomized to either an intervention group—12 weeks of home‐based interval exercise training on a cycle ergometer (n = 16), or a control group (n = 10). The latter was instructed to maintain their habitual physical activities. An incremental cardiopulmonary exercise test and a constant work rate cardiopulmonary exercise test at 75% of peak workload were performed preintervention and postintervention.

Results: Twenty‐three patients completed the protocol and were followed (intervention n = 13, control n = 10). Postintervention exercise time at constant work rate cardiopulmonary exercise test increased in the intervention group compared to controls (median[range] 12[–4 to 52]min vs 0[–4 to 5]min, P = .001). At incremental cardiopulmonary exercise test, peak VO₂ increased 15% within the intervention group (P = .019) compared to 2% within the control group (P = .8). However, in comparison between the groups no difference was found (285[–200 to 535] ml/min vs 17[–380 to 306] ml/min, P = .10). In addition, peak workload at incremental cardiopulmonary exercise test increased in the intervention group compared to controls (20[–10 to 70]W vs 0[–20 to 15]W, P = .003).

Conclusion: Home‐based interval exercise training increased endurance capacity and peak exercise capacity in adults with complex congenital heart disease. Aerobic endurance might be more relevant than peak oxygen uptake with regard to daily activities, and therefore a more clinically relevant measure to evaluate.

Background: Adults with complex congenital heart disease (CHD) have reduced aerobic capacity and impaired muscle function. We therefore hypothesized that patients have a lower skeletal muscle mass and higher fat mass than controls.

Methods: Body composition was examined with full body Dual-Energy x-ray Absorptiometry (DXA) in 73 patients with complex CHD (mean age 35.8 ± 14.3, women n = 22) and 73 age and sex matched controls. Patients fulfilling criteria for low skeletal muscle mass in relation to their height and fat mass were defined as sarcopenic.

Results: Male patients (n = 51) were shorter (177.4 ± 6.6 cm vs. 180.9 ± 6.7 cm, p = 0.009) and weighed less (76.0 ± 10.8 kg vs. 82.0 ± 12.4 kg, p = 0.01) than controls. Also, patients had a lower appendicular lean mass-index (ALM-index) (7.57 ± 0.97 kg/m2 vs. 8.46 ± 0.90 kg/m2, p < 0.001). Patients’ relative tissue fat mass (27.9 ± 7.0% vs. 25.4 ± 8.6%, p = 0.1) did not differ. Forty-seven percent of the men (n = 24) were classified as sarcopenic.

Female patients (n = 22) were also shorter (163.5 ± 8.7 cm vs. 166.7 ± 5.9 cm, p = 0.05) but had a higher BMI (25.7 ± 4.2 vs. 23.0 ± 2.5, p = 0.02) than controls. Patients also had a lower ALM-index (6.30 ± 0.75 vs. 6.67 ± 0.55, p = 0.05), but their relative body fat mass (40.8 ± 7.6% vs. 32.0 ± 7.0%, p < 0.001) were higher. Fifty-nine percent of the women (n = 13) were classified as sarcopenic.

Conclusions: The body composition was altered toward lower skeletal muscle mass in patients with complex CHD. Approximately half of the patients were classified as sarcopenic. Contrary to men, the women had increased body fat and a higher BMI. Further research is required to assess the cause, possible adverse long-term effects and whether sarcopenia is preventable or treatable.

Aims: The majority of children with complex congenital heart disease (CHD) survive into adulthood due to advances in medical care. Adult patients with CHD have an increased incidence of diagnoses related to ageing such as heart failure, dementia, cancer and sarcopenia, despite a relatively low age. They also have a shorter life expectancy. It is unknown if their bone structures also show signs of premature ageing. We therefore investigated Bone Mineral Content (BMC) and bone mineral density (BMD) in an adult population with complex CHD.

Methods: The total body BMC and BMD was examined using dual energy X-ray absorptiometry (DXA) in 73 adults with complex CHD (mean age 35.8 ± 14.3, women n = 22) and 73 age and sex matched controls.

Results: The adults with complex CHD had lower total body BMC (2.6 ± 0.5 kg vs. 2.9 ± 0.5 kg, p < 0.001) and BMD (1.18 ± 0.12 g/cm² vs. 1.26 ± 0.11 g/cm², p < 0.001) compared to controls. BMD was lower for patients with single ventricle physiology and for the other complex diagnoses, and it persisted after correction for most common risk factors for osteoporosis.

Conclusion: Adults with complex CHD have reduced total body BMC and BMD compared to healthy controls. These results are a sign of frailty that conforms with other previously reported signs of premature ageing. The risk of osteoporosis is low in our relatively young population, but it is assumed to increase with ageing. We recommend that clinicians pay close attention to risk factors for osteoporosis, and are generous in administering DXA-measurements in order to prevent future fractures among adults with complex CHD.

Background: High BMI is a risk factor for cardiovascular disease and, in contrast, low BMI is associated with worse prognosis in heart failure. The knowledge on BMI and the distribution in different BMI-classes in adults with congenital heart disease (CHD) are limited. Methods and results: Data on 2424 adult patients was extracted from the Swedish Registry on Congenital Heart Disease and compared to a reference population (n = 4605). The prevalence of overweight/obesity (BMI >= 25) was lower in men with variants of the Fontan procedure, pulmonary atresia (PA)/double outlet right ventricle (DORV) and aortic valve disease (AVD) (Fontan 22.0% and PA/DORV 15.1% vs. 43.0%, p = 0.048 and p < 0.001) (AVD 37.5% vs. 49.3%, p < 0.001). Overt obesity (BMI >= 30) was only more common in women with AVD (12.8% vs. 9.0%, p = 0.005). Underweight (BMI < 18.5) was generally more common in men with CHD (complex lesions 4.9% vs. 0.9%, p < 0.001 and simple lesions 3.2% vs. 0.6%, <0.001). Men with complex lesions were shorter than controls in contrast to females that in general did not differ from controls. Conclusion: Higher prevalence of underweight in men with CHD combined with a lower prevalence of over-weight/obesity in men with some complex lesions indicates that men with CHD in general has lower BMI compared to controls. In women, only limited differences between those with CHD and the controls were found. The complexity of the CHD had larger impact on height in men. The cause of these gender differences as well as possible significance for prognosis is unknown.

Purpose: Complex congenital heart disease is often associatedwith impaired physical functioning, usually measured as peak oxygen uptake in an exercise test. Skeletal muscle function is, however, less studied in these patients.

Methods: Unilateral isotonic shoulder flexion was tested in 79 adultpatients (mean age 36.6±14.8 years, 31 females) with congenital heartdisease, classed as either "complex" (n=41, 51.9%) or "simple" (n=38, 48.1%). The patients were sitting comfortably in a chair with their back touching the wall and holding a weight (2 kg for women and 3 kg for men) in the hand of the tested side. The patients were asked to elevate the arm, from 0 to 90 degrees flexion, as many times as possible. The pace of 20 contractions per minute was held using a metronome.

Results: Patients with complex lesions performed less shoulder flexions compared with patients with simple lesions (29.2±10.0 vs. 54.6±25.8, p<0.001). In univariate analysis including a number of demographic and clinical variables, only complexity of cardiac lesion (p<0.001) and on-going cardiac medications (p=0.012) were associated with shouldermuscle function, of which complexity (p<0.001) remained significant in multivariate analysis.

Conclusion: There is a marked difference in shoulder muscle functionbetween patients with complex and simple congenital heart disease. Such differences might affect ability to perform daily activities and contribute to impaired overall physical functioning. Rehabilitation targeting muscle function may be indicated in patients with complexcongenital heart disease.

Background Adults with congenital heart disease (ACHD) usually have reduced aerobic exercise capacity compared with controls. However, their skeletal muscle function is less studied. Material and methods In this cross-sectional study, unilateral isotonic shoulder flexion, unilateral isotonic heel-lift, maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) were tested in 85 patients with ACHD (35 women, mean age 36.814.8 years), classed as either complex' (n=43) or simple' (n=42), and 42 age and gender matched controls (16 women, mean age 36.914.9). Maximum number of shoulder flexions and heel-lifts were measured. MIP/MEP was tested using a handheld respiratory pressure meter. Exercise self-efficacy, measuring confidence in performing exercise training, was evaluated. Results Adults with complex lesions performed fewer shoulder flexions compared with controls and patients with simple lesions (28.2 +/- 11.1 vs. 63.6 +/- 40.4, p<0.001 and 28.2 +/- 11.1 vs. 54.9 +/- 24.9, p<0.001), as well as fewer heel-lifts compared with controls and patients with simple lesions (17.6 +/- 7.7 vs. 26.3 +/- 12.8, p<0.001 and 17.6 +/- 7.7 vs. 23.2 +/- 7.0, p=0.024), lower MIP than controls (80.7 +/- 26.7 vs. 111.1 +/- 29.9cm H2O, p<0.001) and lower MEP compared with controls (110.8 +/- 39.9 vs. 141.8 +/- 39.5, p<0.001). Their exercise self-efficacy was lower than controls (28.0 +/- 8.3 vs. 33.4 +/- 6.1, p=0.002). In a linear regression model complex heart lesions were independently associated with impaired limb muscle function. Conclusion Adults with complex congenital heart disease have impaired skeletal muscle function compared with patients with simple lesions and healthy controls. They also had lower confidence in performing exercise training. Thus, this population might have a potential for rehabilitation focusing on improving muscle function and confidence in performing exercise training.

Purpose: Many adults with congenital heart disease have reduced exercise capacity but only little is known about habitual physical activityin this group. The aim of this study was to investigate habitual physicalactivity level in a cohort of adults with congenital heart disease.

Methods: Seventy-five adult patients (29 females) aged 37.2±15.1 yearswith congenital heart disease classed as either simple or complex were studied with a combined accelerometer and heart rate monitor (Actiheart). The patients carried the Actiheart during 5 consecutive days, and where encouraged to proceed with their usual daily activities. Data was analysed with the Actiheart Software version 2.2. A mean ofPhysical Activity Level (PAL) (PAL=Total Energy Expenditure/Resting Energy Expenditure) over 4 days was calculated. PAL < 1.45 was categorised as low, 1.45-1.6 as moderate and >1.6 as high.

Results: Forty-three patients (57,3%) had low PAL, 18 (24%) moderate PAL and 14 (16,7%) high PAL. PAL was lower in women compared to men (1.32±0.10 vs. 1.52±0.18, p < 0.001). PAL was higher in patients withsimple compared with complex congenital heart disease (1.50±0.21 vs. 1.39±0.14, p = 0.019). Among men, active smoking (p=0.015) and lower age (p=0.04) were independently associated with higher PAL, whereas no such associations were observed among women.

Conclusion: The majority of the observed patients had low PAL and women had lower PAL compared to men. PAL was related to complexity ofheart lesion. Efforts to increase habitual physical activity may beindicated in this population, especially in women and patients withcomplex congenital heart disease.

Background: Due to improved care, the numbers of patients with tetralogy of Fallot (ToF) are increasing. However, long-term morbidity and need for reinterventions are concerns and also address issues of quality of life (QoL).

Methods: Patients with ToF and valid EuroQol-5 dimensions questionnaire (EQ-5D) were identified in the national Swedish register on congenital heart disease. EQ-5Dindex was calculated and dichotomised into best possible health-related QoL (EQ-5Dindex=1) or differed from 1.

Results: 288 patients met the criteria and were analysed. Univariate logistic regression showed a positive association between New York Heart Association (NYHA) class I (OR 8.32, 95% CI 3.80 to 18.21), physical activity >3 h/week (OR 3.34, 95% CI 1.67 to 6.66) and a better right ventricular function (OR 2.56, 95% CI 1.09 to 6.02). A negative association between symptoms (OR 0.23, 95% CI 0.13 to 0.42), cardiovascular medication (OR 0.31, 95% CI 0.18 to 0.53), age (OR 0.97, 95% CI 0.96 to 0.99) and EQ-5Dindex was observed. In multivariate logistic regression, NYHA I (OR 7.28, 95% CI 3.29 to 16.12) and physical activity >3 h/week (OR 2.27, 95% CI 1.07 to 4.84) remained associated with best possible health-related QoL. Replacing NYHA with symptoms in the model yielded similar results.

Conclusion: In this registry study, self-reported physical activity, staff-reported NYHA class and absence of symptoms were strongly associated with best possible health-related QoL measured by EQ-5D. Physical activity level is a potential target for intervention to improve QoL in this population but randomised trials are needed to test such a hypothesis.

Background: On a group level, patients with repaired tetralogy of Fallot have impaired exercise capacity. Since exercise capacity is related to prognosis, and more patients survive into higher age, it is important to know the expected exercise capacity at different ages. Furthermore, factors associated with exercise capacity and the relation between exercise capacity and mortality need further evaluation.

Methods: The national register of congenital heart disease was searched for exercise tests in adults with repaired tetralogy of Fallot. The results from exercise tests were compared with national reference data.

Results: 314 patients were identified (median age 33.2 [IQR 24.8-44.7], 40.8% women). The mean percent of predicted workload was 74.8 (±19.6) % without change across ages. In multivariable analysis, NYHA class I (odds ratio [OR]4.2, 95% confidence interval [CI]1.7-10.0) and higher physical activity level (>3 h/week) (OR 3.6, 95%CI 1.8-7.3) were positively associated with higher exercise capacity, while ongoing cardiovascular therapy (OR 0.4, 95%CI 0.2-0.7) and male sex (OR 0.3, 95%CI 0.2-0.6) were negatively associated with higher exercise capacity. Both exercise capacity (HR 0.96, 95%CI 0.93-0.98) and heart rate reserve (HR 0.96, 95%CI 0.94-0.98) were associated with mortality.

Conclusions: In patients with repaired tetralogy of Fallot the exercise capacity was approximately 75% of expected, regardless of age. Patients with better NYHA class and high physical activity level had a higher exercise capacity. Low exercise capacity and low heart rate reserve were associated with higher mortality. Therefore, evaluating exercise capacity as part of follow up is of importance.

Patients palliated with Total Cavopulmonary Connection have a lower muscle mass and a lower exercise capacity. We assessed calf muscle oxidative metabolism during and after heel raise exercise to exhaustion in young patients with TCPC compared to healthy peers. Near-infrared spectroscopy was used for measuring oxygen metabolism in the medial portion of the gastrocnemius muscle. Forty-three patients with TCPC, aged 6–18 years, were compared with 43 age and sex-matched healthy control subjects. Subgroups were formed to include children (6–12 years) and adolescents (13–18 years) to determine if these age groups influenced the results. During exercise, for the patients compared to controls there was a lower increase in deoxygenated hemoglobin (oxygen extraction) (5.13 ± 2.99au vs. 7.75 ± 4.15au, p = 0.001) and a slower rate of change in total hemoglobin (blood volume) (0.004 ± 0.015au vs 0.016 ± 0.01au, p = 0.001). Following exercise, patients exhibited a slower initial increase in tissue oxygenation saturation index (0.144 ± 0.11au vs 0.249 ± 0.226au, p = 0.007) and a longer half-time to maximum hyperemia (23.7 ± 11.4 s vs 16.8 ± 7.5 s, p = 0.001). On the subgroup level, the adolescents differed compared to healthy peers, whereas the children did not. Young patients with TCPC had impaired oxidative metabolism during exercise and required a longer time to recover. In that the differences were seen in the adolescent group and not in the children group may indicate a declining function with age.

Aims: The aim of this cross-sectional study was to examine the prevalence of the multidimensional phenomenon of fatigue in adults with congenital heart disease.

Background: Adults with congenital heart disease are a growing population, and patient-reported outcomes can provide valuable information about the patient's experience of living with CHD. Fatigue is a multidimensional phenomenon that can be described as an overwhelming feeling of exhaustion with a reduced capacity of mental and physical work. Fatigue can be observed clinically in adults with congenital heart disease, but the actual prevalence is unknown.

Methods: Fatigue was assessed by the Multidimensional Fatigue Inventory which enables the respondent to report the presence of fatigue according to five dimensions: "general fatigue,""physical fatigue,""mental fatigue,""reduced motivation,"and "reduced activity."The questionnaire was sent to 463 patients in Lund and Umeå. Four groups with complex CHD and two groups with moderately complex CHD were included. The reliability (internal consistency) of the Multidimensional Fatigue Inventory was tested for all dimensions and groups of diagnosis.

Results: The response rate was 56.6% (n= 262). In patients with complex CHD, 40.0-59.4% reported severe to very severe general fatigue, and patients with a single ventricle reported the highest prevalence (59.4%). Among patients with complex CHD, 29.2-40.0% reported severe to very severe mental fatigue. The Multidimensional Fatigue Inventory had a high reliability measured with Cronbach's alpha.

Conclusions: The study findings show a relatively high prevalence of fatigue in adults with congenital heart disease, and general fatigue was the most prevalent. Further studies are needed regarding fatigue and its causes and consequences in adults with congenital heart disease.

Relevance to clinical practice: The Multidimensional Fatigue Inventory proved to be an instrument with high reliability and low internal loss, which suggests that the instrument may be suitable to use as a patient-reported outcome in the care of adults with congenital heart disease, preferably at repeated occasions.

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.