
A 3‐month online training supervised exercise program appears to produce substantial improvements in quality of life (QoL) and the cardiovascular and muscular health of patients with Marfan syndrome (MFS), according to a study.
For the study, researchers recruited 70 patients with MFS, all of whom were receiving beta blockers and had a history of aortic dilation and global joint hypermobility, and 35 healthy controls. Close to half of the patients with MFS had a history of scoliosis, and 61 percent had had ankle sprains. Sex and age were similar in the MFS and control groups. However, compared with controls, MFS patients were heavier and taller.
Patients with MFS were randomly allocated to the training group (MFS‐T) or the control group (MFS‐C). The training involved two supervised online sessions weekly for 3 months for a total of 24 sessions. The exercise intensity during the training sessions was chosen based on heart rate (HR) at the first ventilatory threshold and the HR peak achieved during CPET.
At baseline, the MFS group had lower QoL (assessed using the Medical Outcomes Study Short‐Form 36 questionnaire) in all dimensions and peak oxygen uptake, as well as diminished muscle elasticity compared with the control group.
At 3 months after the intervention, the MFS-T vs the MFS-C subgroup showed significant improvements in QoL (20.2 vs 0.7), peak oxygen uptake (34 percent vs 14 percent), and muscle elasticity index (11.5 vs 1.2). Patients in the MFS-T subgroup also had greater reductions in blood pressure during isometric squats (systolic: −19 vs 0 mm Hg; diastolic: −27 vs 2 mm Hg) and pulse wave velocity at rest (−1.20 vs −0.40) and postexercise (−0.42 vs 0.08). The aortic diameter did not significantly differ between the MFS-T and MFS-C subgroups (−0.19 vs 0.11).
At 3 months, QoL remained lower in MFS‐T than in healthy controls. But they had similar peak oxygen uptake and pulse wave velocity at rest and postexercise.