Effects of aging on aortic diameters and distensibility in Marfan's syndrome: comparison with healthy subjects

The relationships between age, aortic diameter, and aortic distensibility were examined in patients with Marfan's syndrome. Aortic diameters were measured at the sinus of Valsalva, the ascending aorta, the aortic arch, and the abdominal aorta in 20 patients (aged 5-63 years) with Marfan's syndrome and 30 age-matched normal control subjects. The aortic distensibility was calculated as follows: aortic distensibility = 2 (change in aortic diameter)/(diastolic aortic diameter) (systolic pressure-diastolic pressure). Aortic diameter increased with age in both groups. The diameters of the sinus of Valsalva and the ascending aorta were greater in the Marfan group than in the control group, and increased even after adolescence in patients with Marfan's syndrome. Aortic distensibility was the highest at the abdominal aorta and the lowest at the sinus of Valsalva in both groups. Aortic distensibility decreased with age in both groups, but aortas were less distensible in the Marfan group than in the control group at all ages. The sinus of Valsalva and the ascending aorta dilate abnormally after adolescence in patients with Marfan's syndrome. However, aortic elastic properties in patients with Marfan's syndrome were abnormal even in childhood.