Frailty and Cognitive Function in Middle-Aged and Older Adults With Congenital Heart Disease.

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.

Lower bone strength in young patients with Fontan circulation compared to controls.

OBJECTIVES: Previous reports indicate bone deficits in patients with Fontan circulation. However, the consequences of these deficits on bone strength and when these changes occur are unclear. AIM: To compare the tibial bone strength-strain index between young patients (6-19 years) with Fontan circulation and age- and sex-matched controls, and to determine strength-strain-index in subgroups of children (6-12 years) and adolescents (13-19 years) versus controls. METHOD: The tibia was examined with peripheral quantitative CT. Based on the assessed data, bone strength-strain index was calculated in the lateral and anterior-posterior directions. RESULTS: Twenty patients with Fontan and twenty controls (mean age 13.0 ± 4.4 years; 50% females) were examined. Patients had a lower strength-strain index in the lateral direction compared to controls (808.4 ± 416.8mm3 versus 1162.5 ± 552.1mm3, p = 0.043). Subgroup analyses showed no differences regarding strength-strain index in children (6-12 years) with Fontan circulation compared to controls. However, the adolescents (13-19 years) with Fontan circulation had lower strength-strain indexes in both the lateral and anterior-posterior directions compared to controls (1041.4 ± 299.8mm3 versus 1596.4 ± 239.6mm3, p < 0.001, and 771.7 ± 192.4mm3 versus 1084.9 ± 215.0mm3, p = 0.004). When adjusted for height, there were differences between patients (6-19 years) and controls in strength-strain indexes in both the lateral and anterior-posterior directions. In subgroup analyses, the results remained robust. CONCLUSION: Young patients (6-19 years) with Fontan circulation have a lower strength-strain index in the tibia compared to controls. Subgroup analyses show that this deficit is mainly driven by the differences in adolescents (13-19 years), which might suggest that bone strength decreases with age.

Accelerometer derived physical activity and subclinical coronary and carotid atherosclerosis: cross-sectional analyses in 22 703 middle-aged men and women in the SCAPIS study.

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.

Rationale, design and methodology of APPROACH-IS II: International study of patient-reported outcomes and frailty phenotyping in adults with congenital heart disease.

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.

Effect of a Lifestyle-Focused Web-Based Application on Risk Factor Management in Patients Who Have Had a Myocardial Infarction: Randomized Controlled Trial.

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. TRIAL REGISTRATION: ClinicalTrials.gov NCT03260582; https://clinicaltrials.gov/ct2/show/NCT03260582. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): RR2-10.1186/s13063-018-3118-1.

Vitamin D, liver-related biomarkers, and distribution of fat and lean mass in young patients with Fontan circulation.

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.

Calf Muscle Oxygenation is Impaired and May Decline with Age in Young Patients with Total Cavopulmonary Connection.

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.

Prevalence of fatigue in adults with congenital heart disease.

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.

Enablers and barriers for being physically active: experiences from adults with congenital heart disease.

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.

Isotemporal Substitution of Time Between Sleep and Physical Activity: Associations With Cardiovascular Risk Factors in Early Rheumatoid Arthritis.

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 ß (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.

Despite reductions in muscle mass and muscle strength in adults with CHD, the muscle strength per muscle mass relationship does not differ from controls.

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.

Low bone mineral density in adults with complex congenital heart disease.

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/cm2vs. 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.

Impaired knee extension muscle strength in adolescents but not in children with Fontan circulation.

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.

Health-related quality of life in patients with heart failure eligible for treatment with sacubitril-valsartan.

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.

Slower Skeletal Muscle Oxygenation Kinetics in Adults With Complex Congenital Heart Disease.

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°) to exhaustion, and during recovery. RESULTS: The patients with CHD performed fewer shoulder flexions (40 ± 17 vs 69 ± 40; P < 0.001), had lower muscle oxygen saturation (StO2) at rest (58 ± 18% vs 69 ± 18%; P < 0.001), slower desaturation rate at exercise onset (-9.7 ± 5.9 vs -15.1 ± 6.5% StO2 × 3.5 s-1, P <0.001), and slower resaturation rate post exercise (4.0 ± 2.7 vs 5.4 ± 3.6% StO2 × 3.5 s-1; P = 0.009) compared with the controls. CONCLUSIONS: In comparison with age- and sex-matched controls, adults with complex CHD had slower oxygenation kinetics. This altered skeletal muscle metabolism might contribute to the impaired skeletal muscle endurance capacity shown and thereby also to the reduced aerobic capacity in this population.

Sarcopenia is common in adults with complex congenital heart disease.

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 ±â€¯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 ±â€¯6.6 cm vs. 180.9 ±â€¯6.7 cm, p = 0.009) and weighed less (76.0 ±â€¯10.8 kg vs. 82.0 ±â€¯12.4 kg, p = 0.01) than controls. Also, patients had a lower appendicular lean mass-index (ALM-index) (7.57 ±â€¯0.97 kg/m2vs. 8.46 ±â€¯0.90 kg/m2, p < 0.001). Patients' relative tissue fat mass (27.9 ±â€¯7.0% vs. 25.4 ±â€¯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 ±â€¯8.7 cm vs. 166.7 ±â€¯5.9 cm, p = 0.05) but had a higher BMI (25.7 ±â€¯4.2 vs. 23.0 ±â€¯2.5, p = 0.02) than controls. Patients also had a lower ALM-index (6.30 ±â€¯0.75 vs. 6.67 ±â€¯0.55, p = 0.05), but their relative body fat mass (40.8 ±â€¯7.6% vs. 32.0 ±â€¯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.

Factors associated with health-related quality of life among adults with tetralogy of Fallot.

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.

Aerobic capacity in adolescence is associated with time to intervention in adult men with atrial septal defects.

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 ±â€¯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 ±â€¯51 W vs. 276 ±â€¯52 W, p = 0.49). Men with a higher exercise capacity (≥1 SD) had their intervention earlier (21.9 ±â€¯8.6 years vs. 27.5 ±â€¯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.

Effect of a lifestyle-focused electronic patient support application for improving risk factor management, self-rated health, and prognosis in post-myocardial infarction patients: study protocol for a multi-center randomized controlled trial.

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. TRIAL REGISTRATION: ClinicalTrials.gov, NCT03260582 . Retrospectively registered on 24 August 2017.