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Background: In congenital aortic stenosis (CAS), commissurotomy is an option in patients not suitable to receive a valve prosthesis. However, there is often a need for future additional interventions on the aortic valve. The fate of the aortic valve is, however, essentially unknown. This study reports the need for reinterventions after surgical commissurotomy, based on a national register.

Materials and methods: The national register on congenital heart diseases (CHDs) was searched for patients with CAS, simple or associated with other CHD and an index commissurotomy with later data from follow-up.

Results: 300 patients with CAS and an index commissurotomy (mean age at the operation 7.4±7.8 years, 72.4% males) were identified. After an observation time of 27.4±10.0 years, 54.7% of the patients had a reintervention that occurred 14.2±10.1 years after the index operation. The cumulative incidence of reintervention was approximately 25% 10 years after and 60% 30 years after the index intervention. The prevalence of left ventricular hypertrophy (LVH) was higher among those that needed reintervention (41.3 vs 26.8%, p=0.023). Furthermore, eight patients died with a cumulative incidence of 7% 30 years after the index intervention, where most were (7/8) without reintervention (p=0.025). There were no additional important differences between patients with and without reintervention. The prevalence of left ventricular dysfunction and New York Heart Association class >1 was low.

Conclusion: Most (54.7%) patients with a commissurotomy, more than half of them within 30 years and eventually all will need a reintervention. This is important information to patients, especially for women in childbearing age. The higher prevalence of LVH in the group with reintervention needs attention during follow-up. Furthermore, those without reintervention, for unknown reasons, had a higher mortality. Our data strengthen the arguments for close outpatient follow-up among patients with a previous commissurotomy.

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.

BACKGROUND: When more patients with congenital heart disease reach adult age, increased incidence of long-term complications, including ischemic stroke, are expected. The aim of this study was to analyze cumulative incidence of ischemic stroke, associated factors, and case fatality in adult congenital heart disease.

METHODS AND RESULTS: The study is based on Swedish national registers on congenital heart disease and stroke. Patients with congenital heart disease were followed between 2001 and 2018 for first-ever ischemic stroke events (ischemic stroke due to patent foramen ovale excluded). Factors possibly associated with ischemic stroke were analyzed using Cox regression models. Out of 8914 adult patients with congenital heart disease, 108 suffered ischemic stroke over a mean period of 7.6±4.7 years. The mean age at ischemic stroke was 53.8 years, and the cumulative incidence was 0.15% at 1 year, 0.5% at 5 years, and 1.5% at 10 years. In multivariable analysis, age (hazard ratio [HR], 1.04 [95% CI, 1.03-1.06]), diabetes (HR, 2.9 [95% CI, 1.3-6.4]), ejection fraction <50% (HR, 1.9 [95% CI, 1.1-3.4]), atrial septal defect (HR, 3.0 [95% CI, 1.03-8.5]), and aortic valve lesions (HR, 4.8 [95% CI, 1.6-14.1]) were associated with increased risk. Among those with ischemic stroke, approximately half were on antithrombotic treatment (anticoagulants or antiplatelets) before admission. Case fatality was 6.5%.

CONCLUSIONS: The cumulative incidence of ischemic stroke was 1.5% after 10 years. In adult congenital heart disease, the type of heart lesion, diabetes, ejection fraction <50%, and age were important factors associated with ischemic stroke. Despite a relatively young age, mortality is a significant threat. At time of the ischemic stroke event, approximately half of the patients were on antithrombotic treatment.

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.

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: An association between impaired exercise capacity and risk of mortality has been reported among adults with congenital heart disease (CHD). Over the years, treatment methods have improved and may influence outcome. Hence, we report data from a national cohort reflecting a contemporary population.

Objectives: The purpose of this study was to investigate the association between exercise capacity (workload) and mortality in a large registry-based cohort.

Methods: Data on exercise capacity using cycle ergometer were retrieved from the national registry of CHD. The association between predicted exercise capacity (%ECpred) and mortality was analyzed using Cox regression.

Results: In total, 3,721 adults (>18 years, 44.6% women) with CHD were included. The median age was 27.0 years (IQR: 20.8-41.0 years) and mean %ECpred was 77% ± 20%. Over a mean follow-up of 9.4 ± 6.0 years, there were 214 (5.8%) deaths. The Multivariable Cox regression model showed that moderately and severely impaired exercise capacity (50-<70 %ECpred: HR: 2.1, 95% CI: 1.4-3.2, P < 0.001, and <50 %ECpred: HR: 3.5, 95% CI: 2.1-6.0, P < 0.001) and CHD complexity were associated with higher mortality (moderate complexity: HR: 1.9 95% CI: 1.2-3.0, P = 0.003, great complexity: HR: 2.3 95% CI: 1.3-4.2, P = 0.008) when adjusted for New York Heart Association class, physical activity, cardiovascular medication, sex, impaired systemic ventricular function, and age.

Conclusions: Impaired exercise capacity and CHD complexity are independently associated with all-cause mortality in patients with CHD. Exercise capacity is an easily accessible variable that may be a useful tool for risk assessment in adult patients with CHD, but this needs confirmation in prospective studies.

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.

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.

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.