Marfan Syndrome Moderate Exercise Trial II

Summary

Marfan syndrome (MFS) is a distinctive connective tissue disorder that affects multiple organ systems including the heart, bones, ligaments, and eyes, and is associated with significant risk of aortic dissection. Given limited evidence from in-vitro studies, and theoretical concerns, the majority of patients with MFS are restricted from certain physical activities. The lack of exercise and deconditioning have detrimental effects including increasing weakness, joint pain, decreased endurance, and depressive symptoms. Given the significant paucity of data currently existing on the effects of exercise in humans with MFS, and the recent, optimistic findings in rodent models, this pilot trial was established to assess the effects of moderated dynamic exercise in adolescents and young adults with MFS.

Eligibility

Inclusion Criteria:

* Male and female patients diagnosed with Marfan syndrome (defined by Ghent criteria and either a pathogenic variant in FBN1 or ectopia lentis)
* Age 10-25 years at enrollment

Exclusion Criteria:

1. History of aortic surgery
2. History of spinal surgery with implanted materials that may negatively impact MRI safety or imaging quality.
3. Diagnosis of major congenital heart disease (ASD, VSD, bicuspid aortic valve, and mitral valve prolapse will not qualify as exclusion criteria)
4. Condition limiting the ability to perform moderate exercise.
5. Major concurrent diagnosis that may confound the interpretation of the effect of the proposed intervention on the proposed outcome measures.
6. Aortic dilation meeting threshold for prophylactic aortic surgical intervention (\>/= 4.5 cm in diameter)

Conditions2

Locations1 site

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