Table 1 Physical evaluations and training programs in Marfan syndrome

Humans

Benninghoven et al. [11]

5 times a week for 3 weeks combining ergo cycle training, Nordic walking, and gymnastics 18 MFS patients

(Nordic Walking 666 m P < 0,05% and ergo cycle 0.23 Watt/kg P < 0.001) Improvement of physical capacities

A protocol of physical activity with light intensity seems to have positive effects

Giske et al. [22]

Assessment of lung capacity, the volume of oxygen uptake, and muscle strength

(1) Decrease in strength up to 48% vs. control

MFS patients exhibits a decrease in muscle strength. This impairment increase with the speed of exercises

Kolonics-Farkas [23]

Assessment of lung capacity and VO₂ peak

Decreased lung function

The spirometry-specific equation for MFS patients

Otremski [52]

Correlation between spine and chest wall deformities and lung function

(1) Thoracic kyphosis (mean 19.3°; − 32° to 54°) had a strong positive correlation with FEV1/FVC (r = 0.65; P < 0.001)

(2) Significant decrease in FEV1/FVC below 80% occurred at kyphosis less than 15° (P = 0.004)

(3) The chest wall had a strong negative correlation with FEV1/FVC (r =  − 0.61; P = 0.001)

The deformation of the rib cage and spine impairs lung function

Percheron et al. [25]

Evaluation of the muscle strength of the lower limbs with an isokinetic apparatus

Correlation between muscle strength and muscle mass (r = 0.68; P = 0.0048)

The strength depended on the movement speed was about 10% lower than the control group with a significant difference of P < 0.001

A significant difference in muscle strength was shown for MFS patients

Animals

Gibson et al. [26]

5-month training program with different training intensities from 55 to 85% of VO₂ max

(1) Number of segments of elastin fibers

(2) Length of the elastin fiber

(3) Improvement and organization of elastin fibers

(4) The compliance of the aorta

(5) Reduction of MMP 2–9 at 55% of VO₂ max

(6) Reduction of the aortic diameter

(7) Decreased tensions

(8) High-intensity exercises (75–85% Vo₂max) induced aortic aneurysm but this was not observed after moderate intensity exercises (55%-65% Vo₂max)

Physical activity (forced treadmill or voluntary wheel) dramatically improves the structural architecture of the aorta in a mouse model of MFS (Fbn1^C1039G/+). The tensions are reduced and allows to stop the dilation as well as to reduce it

Mas-Stachurska et al. [27]

5-month training program on treadmill, 20 cm/s, 12° positive slope for 60 minutes/day, 5 days/week.

(1) aortic root dilation rate blunted by training

(2) No improvement of aortic stiffness by training

(3) Training did not induce additional structural damage in the tunica media of aorta

(4) Regression of left ventricle hypertrophy with training

Training prevented aortic root dilation and partially reversed cardiac hypertrophy in a mouse model of MFS (Fbn1^C1039G/+)