Abstract
AbstractBackgroundMarfan syndrome (MFS) is a rare genetic disorder affecting the vascular and musculoskeletal systems. Exercise is classically contraindicated and there are no data on the limitations associated with the syndrome and the benefits of training in this population. This study aimed to characterise the quality of life (QoL) and physical capacity of patients with MFS and to evaluate the benefits of a 3-month online personal training program.MethodsMFS patients were compared with healthy subjects (H-S) at baseline. They were then randomized 1:1 into a training group (MFS-T) and a control group (MFS-C). The training consisted of 2 supervised online training sessions per week at home for 3 months, and the session program was selected based on the initial assessment. The main outcome measure was QoL as assessed by the MOS SF-36. The evolution of parameters during training was compared between MFS-T and MFS-C.ResultsAt baseline, QoL in all dimensions was lower in MFS. Peak oxygen uptake (VDO2peak) was also 25% lower, as was muscle elasticity. Training significantly improved 1) QoL (+20.2±14.3 MFS-Tvs.0.7±0.5 MFS-C), 2) VDO2peak (+34% MFS-Tvs.14% MFS-C), 3) muscle elasticity index (11.5±8.2 MFS-Tvs.+1.2±1. 7 MFS-C), reduced blood pressures during isometric squat (systolic −19±30 MFS-Tvs.0±6 MFS-C; diastolic −27±39 MFS-Tvs.+2±15 MFS-C), reduced pulse wave velocity (PWV) at rest (-1.20±1.89 MFS-Tvs.−0.40±1.61 MFS-C) and after peak exercise (-0.42±0.45 MFS-Tvs.0.08±0.48 MFS-C). Aorta diameter remained stable in both groups (MFS-T −0.19±1.1vs.0.11±0.78 MFS-C). After training, QoL remained lower in MFS-T than in H-S, but peak VDO2, PWV at rest and after exercise were similar to those of H-S.ConclusionsA 3-month online training program had a beneficial effect on QoL, cardiovascular and muscular parameters in MFS without affecting aortic root diameter.
Publisher
Cold Spring Harbor Laboratory