Children and Adolescents With Marfan Syndrome: 10,000 Healthy Steps and Beyond

Marfan syndrome is an inherited disorder of the connective tissue, which provides material
and support for the skeleton, muscles, and blood vessels. Marfan patients are at risk of
sudden death due to weakening of the wall of the large blood vessel leading from the heart
(aorta). The wall of the aorta weakens and dilates which can rupture, leading to death, and
sometimes during intense exercise.

Typically, the weakening process starts when elastin fibers in the aorta become fragmented.
There is some evidence in Marfan patients that a stiffer aorta increases risk for rupture.

For some time, clinical care has focused on what type of exercise these patients should avoid
due to risk for aortic dissection. Little clinical emphasis has been placed on encouraging
patients to engage in routine and safe exercise such as walking. These young patients also
frequently choose sedentary lifestyles, most likely due to limitations imposed by parents as
well as adolescents' own perceptions of what is safe for them and their physical
capabilities. Another complicating factor is that these patients often experience difficulty
coping with their diagnosis. Informed by this evidence, the investigators propose to
collaboratively investigate whether regular exercise improves aortic health and coping skills
in adolescent Marfan patients.

Inclusion Criteria:

1. 10-19 years of age,

2. MFS by revised Ghent criteria,

3. Cardiac clearance to exercise by the primary cardiologist.

Exclusion Criteria:

1. Ventricular dysfunction,

2. Prior history of aortic surgery.