Is physical activity a future therapy for patients with Marfan syndrome?

Abstract

Introduction The international recommendations tend to avoid physical activity (PA) for patients with Marfan syndrome (MFS). However, exceptions have recently been made in the most recent recommendations for these patients, suggesting benefits from doing PA at low intensity only. Furthermore, there is no evidence that moderate aerobic or weight training can worsen the disease symptoms and increase mortality of MFS patients. The present review sums up the work carried out in the field of PA and MFS. The review aims to (1) identify the different types of exercise testing and training protocols and (2) discuss the feasibility and potentially beneficial nature of PA as an innovative way to manage MFS patients. Methods The scientific literature was reviewed using the following words: Marfan syndrome, training, physical activity, evaluation, weight training, arterial disease, aneurysms, lung damage, aortic dissection, rupture. A total of 345 studies were prospected and 43 studies were included. Conclusions A limited number of studies were done in humans, however one demonstrated the feasibility of the management of MFS patients with PA. There were potential beneficial effects of PA on arterial structures, but this review also showed deleterious effects when PA was conducted at high intensities, corresponding to 75–85% of the maximal oxygen uptake. However, these effects have only been reported in animal studies.

Physical fitness in children with Marfan and Loeys-Dietz syndrome: associations between cardiovascular parameters, systemic manifestations, fatigue, and pain

“…Transgenic mouse models of MFS reported that moderate dynamic exercise mitigated the progression of cardiovascular phenotypes associated with MFS [ 8 , 9 ]. Regular physical activity reduced cardiovascular risks in the general population and in patients with cardiovascular diseases and other chronic childhood conditions [ 12 ]. Furthermore, children with MFS reported about the adverse impact of systemic manifestations, such as musculoskeletal problems, on their physical activities [ 14 – 16 ].…”

“…Then, reviews on pediatric cardiac rehabilitation [ 10 ] and obesity in children [ 11 ] reported improved physical fitness following rehabilitation exercise programs. Moreover, the benefits of regular physical activity are well-established, particularly in reducing cardiovascular risks in both the general population and patients with cardiovascular, as well as other chronic diseases [ 12 , 13 ].…”

"Therapeutic Rehabilitation Program and Appreciating the Academic Performance of a Child with Marfan Syndrome at Inclusive School Settings"

“…The regimen of mild PA appears to have beneficial effects. Cardiorespiratory and muscular endurance are improved as a result of physical exercise [14]. The MFS must continue to be active in order to maintain their physical health.…”

L’exercice physique dans les maladies vasculaires

“…En raison de ces directives appelant à la prudence, la prescription de l'exercice chez ces patient-e-s est difficile à mettre en place, surtout en l'absence de programme d'entraînements clairement établis. 6 Pourtant, la sédentarité et l'inactivité physique qui en découlent augmentent le risque de complications vasculaires, notamment le risque de développer/péjorer une hypertension artérielle. Cette dernière augmente le risque de rupture d'anévrisme aortique chez les patient-e-s avec syndrome de Marfan par exemple.…”

“…Les rares études sur l'exercice et le syndrome de Marfan montrent globalement un effet favorable de l'entraînement, notamment sur la capacité cardiorespiratoire et musculaire. 6 Il en résulte que la sédentarité fait partie des facteurs de risque cardiovasculaire dont il faut activement s'occuper chez ces patient-e-s. Les activités d'intensité légère/ modérée, pratiquées de manière régulière, comme la marche, la randonnée, la natation ou le vélo sont à favoriser. Pour être sûr de se trouver dans cette fourchette d'intensités sécuritaires, il faut pouvoir parler avec aisance pendant l'effort, sans devoir s'arrêter pour reprendre son souffle.…”