Pulmonary function, working capacity and strength in young adults with Marfan syndrome.

Abstract

To investigate pulmonary function, working capacity and isokinetic muscle strength in subjects with Marfan syndrome. Seventeen subjects, mean age 23 years, performed spirometry, maximal ergometer cycle tests and knee extension and flexion isokinetic torque tests. The subjects with Marfan syndrome had increased total and residual lung volume values compared with predicted values in healthy subjects and reduced peak oxygen uptake compared with healthy subjects of the same age. The isokinetic peak torque was reduced only at the highest velocity in the women. Thirteen subjects were able to perform the bicycle test until exhaustion without cardiovascular complications or pulmonary restrictions. However, their aerobic capacity was considerably decreased, which we suggest is caused by deconditioning. Further investigations are needed to reveal how pulmonary and cardiovascular limitations change with ageing and to give guidelines for exercise.