RecruitingNot ApplicableNCT05809323

Marfan Syndrome Moderate Exercise Trial II

Clinical Trial On The Effects Of Moderate Physical Activity On Health And Well-Being In Adolescents And Young Adults With Marfan Syndrome

Sponsor

Baylor College of Medicine

Enrollment

50 participants

Start Date

Sep 1, 2023

Study Type

INTERVENTIONAL

Conditions

Marfan Syndrome

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

Min Age: 10 YearsMax Age: 25 Years

Plain Language Summary

Simplified for easier understanding

This study — Marfan Syndrome Moderate Exercise Trial II — investigates whether a structured program of moderate physical exercise is safe and potentially beneficial for young people with Marfan syndrome. Marfan syndrome is a genetic connective tissue disorder that affects the heart, blood vessels, eyes, and skeleton. People with this condition have an enlarged or weakened aorta (the body's main artery), which has historically led to blanket restrictions on exercise. However, recent evidence suggests that moderate exercise may not be as dangerous as once thought, and could even offer cardiovascular benefits. Male and female patients aged 10 to 25 who have a confirmed Marfan syndrome diagnosis — defined by the Ghent criteria with either a pathogenic FBN1 gene variant or lens dislocation (ectopia lentis) — are eligible. People who have already had aortic surgery, have aortic enlargement above the threshold for surgical intervention (4.5 cm or larger), or have other major congenital heart disease or conditions limiting exercise ability are not eligible. Participants will engage in a moderate exercise protocol while being monitored through cardiac imaging including MRI. This research is significant because it challenges older assumptions about exercise in Marfan syndrome and could ultimately result in revised guidelines that allow patients to live more active, healthier lives without unnecessary restrictions.

This summary was AI-generated to explain the trial in plain language. It is not medical advice. Always discuss eligibility with your doctor before enrolling in a clinical trial.

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Interventions

OTHERExercise Intervention Group

Patients will be educated on methods of self-evaluating exertion and cardiovascular effort by assessing respiratory rate and perceived exertion. Next, patients will be given options for cardiovascular activities and complete at a moderate level of activity for a minimum of 150 minutes per week. Patients will perform a combination of exercises under the supervision of a physical therapist until a mod intensity level is reached and sustained. Patients will be taught to use the activity tracker to record their heart rate, activity, and PES. Patients will have a phone call every week to assess status, answer questions, and provide guidance on progressing intensity or duration of exercise. At the end of 12 weeks all patients will return for re-assessment.

OTHERControl Group

Patients in the control group will be instructed to continue with baseline physical activities over the duration of the study and be given instructions on the use of the activity tracker. At the end of 12 weeks all patients will return for re-assessment.