This research study aims to evaluate the effect of a 6-month adapted physical activity program (APA) on the endurance capacities (evaluated as the maximum oxygen consumption \[VO2 peak\] on a cardiopulmonary exercise test) of children and adolescents with Marfan syndrome or related.
Genetically confirmed Marfan syndrome or related syndrome
Girl or boy aged 7 to 17
Informed and written consent signed by at least one of the two holders of parental authority
Patient affiliated to a social security scheme or equivalent
Severe aortic dilation (aortic diameter > 45 mm)
and/or left ventricular failure (left ventricular ejection fraction <45%)
and/or severe mitral leakage ≥ grade 3
Pregnancy