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.

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Background Coarctation of the aorta (CoA) constitutes 5-8 % of all congenital heart disease (CHD) and is associated with long-term complications such as hypertension (HTN) and left ventricular hypertrophy (LVH). Factors associated with HTN, LVH, and diffuse myocardial fibrosis, are not yet fully explored in this population. Methods Papers I-III: The Swedish national register of congenital heart disease (SWEDCON) was used to identify adult patients with repaired CoA. Paper IV: Data on 2,424 adult patients with CHD was extracted from SWEDCON and compared to controls (n = 4,605) regarding height, weight and body mass index (BMI). Paper V: Adults with CoA (n = 21, age 28.5 (19.1-65.1) years, 33.3 % female) referred for CMR were investigated with T1 mapping to determine left ventricular extracellular volume fraction (ECV). Results Papers I-II: Out of 653 patients, 344 (52.7 %) had HTN. In a multivariable model, age (years) (OR 1.07, CI 1.05-1.10), sex (male) (OR 3.35, CI 1.98-5.68) and BMI (kg/m2) (OR 1.09, CI 1.03-1.16) were associated with having HTN, and so was systolic arm-leg blood pressure (BP) gradient where an association was found at the ranges (10, 20] mmHg (OR 3.58, CI 1.70-7.55) and > 20 mmHg (OR 11.38, CI 4.03-32.11), in comparison to the range [0, 10] mmHg. When investigating 243 patients with diagnosed HTN, 127 (52.3 %) had elevated BP (≥ 140/90 mmHg). Age (years) (OR 1.03, CI 1.01-1.06) was associated with elevated BP, and so was systolic arm-leg BP gradient in the ranges (10, 20] mmHg (OR 4.92, CI 1.76-13.79), and > 20 mmHg (OR 9.93, CI 2.99-33.02), in comparison to the reference interval [0, 10] mmHg. Patients with elevated BP had more classes of anti-hypertensive medication classes prescribed (1.9 vs 1.5, p = 0.003). Paper III: Out of 506 patients, 114 (22.5 %) were found to have LVH. Systolic BP (mmHg) (OR 1.02, CI 1.01-1.04), aortic valve disease, (OR 2.17, CI 1.33–3.53), age (years) (OR 1.03, CI 1.01–1.05), and HTN (OR 3.02, CI 1.81-5.02), were associated with LVH, while sex (female) (OR 0.41, CI 0.24-0.72) was negatively associated with LVH. Paper IV: There was no difference in height, weight, or BMI between patients with CoA (n = 414) and the reference population. Paper V: In the population of 21 patients, an increased left ventricular myocardial ECV was found in 6 cases (28.6 %). Of the patients with increased ECV, 5/6 (83.3 %) were female (p = 0.002). Patients with increased ECV did not otherwise differ from the rest of the study population. iv Conclusions In adults with repaired CoA, HTN and LVH were common, and many patients with HTN had elevated BP despite treatment. The potentially modifiable factors BMI and systolic arm-leg BP gradient were associated with HTN, and the gradient was also associated with elevated BP among patients with diagnosed HTN. The gradient’s significance remained even within what the current guidelines consider acceptable ranges. Potentially modifiable factors associated with LVH were systolic BP and aortic valve disease. We found no general difference in height, weight, or BMI between patients with CoA and the reference population. While LVH was more common among men, increased myocardial ECV was more common among women.

Background: Patients with d-transposition of the great arteries after Senning or Mustard procedure are at increased risk for arrhythmias. Especially atrial arrhythmias represent a major threat in this patient population. Early detection of clinical and subclinical arrhythmias may prevent major cardiac events. Therefore, this study aimed to evaluate the ability of the Medtronic REVEAL LINQ to detect atrial arrhythmias in adult patients after atrial switch procedure and compare the effectiveness of detecting clinically relevant arrhythmias to conventional Holter monitoring. METHODS: The database of the Heart Center Leipzig, Department for pediatric cardiology was analyzed for patients after atrial switch procedure and implantation of a Medtronic REVEAL LINQ (Medtronic Inc., Minneapolis, Minnesota). After informed consent was obtained, patients were followed up for 1 year including Holter ECG tracings and device interrogation every 3 months. In addition, symptoms were determined using a standardized questionnaire completed by the patient. RESULTS: The analysis is based on 10 patients aged 34.5 years (range 27-46 years), of which 8 had a Senning - and 2 had a Mustard procedure. The comparison of the number of arrhythmia episodes detected by Holter and LINQ showed significantly more detected episodes using LINQ (p 0.014). Data showed that no therapy adjustments were recorded as a consequence of Holter ECG findings, whereas two changes in medication were documented due to arrhythmia detection via LINQ. When analyzing possible correlations between subjective symptoms reported in the standardized questionnaire and the number of episodes documented, there was a significant correlation of subjective symptoms and arrhythmia episodes in LINQ (p 0.014), whereas no correlation could be demonstrated between subjective symptoms and the number of episodes detected by Holter monitoring (p 0.394). CONCLUSION: In patients after Senning or Mustard procedure, the Medtronic REVEAL LINQ seems to be superior in the identification of subclinical arrhythmia burden in comparison to standard Holter ECG.