BACKGROUND: Marfan syndrome (MFS) is a genetic disorder affecting the vascular and musculoskeletal systems. Limited knowledge exists regarding the exercise benefits for this population. This study aimed to explore the impact of a structured exercise program on the quality of life (QoL) and physical capabilities of patients with MFS. METHODS AND RESULTS: This was a randomized, controlled, parallel-group trial. Patients with MFS were randomized in a 1:1 ratio to either a training group or a control group. The trial included a 3-month online supervised training program. Seventy patients with MFS were compared with healthy subjects. They were randomized into a training group (MFS-T) and a control group (MFS-C). The training consisted of 2 supervised online sessions weekly for 3 months. The primary outcome was QoL, assessed using the Medical Outcomes Study Short-Form 36 questionnaire. Baseline QoL in all dimensions was lower in patients with MFS. Their peak oxygen uptake was 25% lower, and muscle elasticity was diminished compared with healthy subjects. Postintervention, significant improvements were observed in the MFS-T group relative to the MFS-C group: QoL (+20.2 +/- 14.3 versus +0.7 +/- 0.5), peak oxygen uptake (+34% versus +14%), muscle elasticity index (11.5 +/- 8.2 versus +1.2 +/- 1.7), reduced blood pressures during isometric squats (systolic -19 +/- 30 versus 0 +/- 6; diastolic -27 +/- 39 versus +2 +/- 15), and reduced pulse wave velocity at rest (-1.20 +/- 1.89 versus -0.40 +/- 1.61) and postexercise (-0.42 +/- 0.45 versus +0.08 +/- 0.48). The aortic diameter remained stable in both groups (MFS-T-0.19 +/- 1.1 versus MFS-C+0.11 +/- 0.78). After training, QoL remained lower in MFS-T than in healthy subjects, but peak oxygen uptake, pulse wave velocity at rest, and postexercise were similar to those of healthy subjects. CONCLUSIONS: The 3-month online training program significantly enhanced QoL and cardiovascular/muscular metrics in patients with MFS without affecting aortic root diameter, suggesting its potential as part of a management strategy for MFS. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT04553094.

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