Abstract
Marfan syndrome (MS) is a dominant autosomal disease caused by mutations in chromosome 15, the locus controlling fibrillin 1 synthesis, and may exhibit skeletal, ocular, cardiovascular, and other manifestations. Pulse wave velocity (PWV) is used to measure arterial elasticity and stiffness and is related to the elastic properties of the vascular wall. Since the practice of exercise is limited in MS patients, it was of interest to analyze the acute effect of submaximal exercise on aortic distensibility using PWV and other hemodynamic variables in patients with MS with either mild or no aortic dilatation. PWV and physiological variables were evaluated before and after submaximal exercise in 33 patients with MS and 18 controls. PWV was 8.51 + or - 0.58 at rest and 9.10 + or - 0.63 m/s at the end of exercise (P = 0.002) in the group with MS and 8.07 + or - 0.35 and 8.98 + or - 0.56 m/s in the control group, respectively (P = 0.004). Comparative group analysis regarding PWV at rest and at the end of exercise revealed no statistically significant differences. The same was true for the group that used beta-blockers and the one that did not. The final heart rate was 10% higher in the control group than in the MS group (P = 0.01). Final systolic arterial pressure was higher in the control group (P = 0.02). PWV in MS patients with mild or no aortic dilatation did not differ from the control group after submaximal effort.
Highlights
Marfan syndrome is a dominant autosomal disease caused by mutations in chromosome 15, the locus controlling fibrillin 1 synthesis, and may exhibit skeletal, ocular, cardiovascular, skin, neurological, and pulmonary manifestations; patients often have a family history of the condition [1]
As the practice of exercise is treated with caution in this population and there are practically no studies on this issue, it was of interest to analyze the acute effect of submaximal exercise on aortic distensibility using Pulse wave velocity (PWV) and hemodynamic parameters in patients with Marfan syndrome with either mild or no aortic dilatation
The patients with Marfan syndrome had a PWV of 8.51 ± 0.58 at rest and 9.10 ± 0.63 m/s at the end of exercise (P = 0.002), while the pulse wave propagation time in this group was 78.27 ± 4.98 at rest and 73.48 ± 5.57 ms at the end of exercise (P = 0.015)
Summary
Marfan syndrome is a dominant autosomal disease caused by mutations in chromosome 15, the locus controlling fibrillin 1 synthesis, and may exhibit skeletal, ocular, cardiovascular, skin, neurological, and pulmonary manifestations; patients often have a family history of the condition [1]. A reduction in aortic distensibility, as measured by PWV in patients with Marfan syndrome, has been reported [6,7]. The 2005 Guidelines of the 36th Bethesda Conference in the field of sports cardiology recommend that patients with Marfan syndrome and aortic dilatation greater than 40 mm (in adults) only play sports such as golf, billiards and bowling, as these sports offer no potential risk of bodily collisions [8,9]. As the practice of exercise is treated with caution in this population and there are practically no studies on this issue, it was of interest to analyze the acute effect of submaximal exercise on aortic distensibility using PWV and hemodynamic parameters in patients with Marfan syndrome with either mild or no aortic dilatation
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