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Qual Life Res. 2016;25(7):1779-1790. doi.org/10.1007/s11136-015-1214-1 Title: A review of physical activity and exercise for people with familial thoracic aortic aneurysms and aortic dissections Abstract Purpose : To explore and summarize knowledge about physical activity and exercise for people with familial thoracic aortic aneurysms and aortic dissections (FTAAD): Marfan syndrome, vascular Ehlers-Danlos syndrome and Loeys-Dietz syndrome. Design and method : Review based on systematic literature searches in relevant databases. Material : 13 articles are included. Results : All articles describe dilemmas regarding physical activity and FTAAD diagnoses, because people with FTAAD are recommended to be physically active, and also receive advice on physical restrictions. We found three review articles and 10 primary studies (no RCT). The review articles emphasize that current recommendations are based on consensus documents from The American Heart Association that recommend low to moderate-intensity exercise. There is agre- ement that physical activity is considered important for improving health, reducing stress and pain and im- proving quality of life. Recent studies contribute with knowledge about how people experience and relate to advice about physical activity. Most people have received advice, but the advice can be difficult to put into practice. Many exercise less than recommended. It is challenging to find the balance between what is healthy and what can potentially involve health risks, especially related to the cardiovascular system and the musculoskeletal system. Conclusion : There are few studies on the health risks and benefits of physical activity and exercise for peo- ple with FTAAD. The studies we found indicate that low-to-moderate physical activity is recommended and that practical follow-up from professionals such as physiotherapists can help people with FTAAD di- agnoses to get started and maintain healthy exercise habits. International evidence-based guidelines are in demand. Keywords : Adults, Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, Marfan syndrome, physical activity, exercise, restrictions, review.

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