Rw. Jeremy et al., RELATION BETWEEN AGE, ARTERIAL DISTENSIBILITY, AND AORTIC DILATATION IN THE MARFAN-SYNDROME, The American journal of cardiology, 74(4), 1994, pp. 369-373
This study examined the relations between age, arterial distensibility
, and systemic hemodynamics in patients with the Marfan syndrome. The
study group included 170 patients referred to a specialist clinic, of
whom 55 (age 26 +/- 12 years) were diagnosed as having Marfan syndrome
. The remaining 115 patients (age 25 +/- 14 years) formed a control gr
oup. Each patient underwent echocardiographic examination, with measur
ement of ascending aorta diameter at end-diastole and end-systole, and
aortic now velocities. The elastic properties of the aorta were index
ed by calculation of aortic distensibility, wall stiffness, and system
ic pulse wave velocity. Mean end-diastolic aortic diameter in the Marf
an group (38 +/- 9 mm) was greater than that in the controls (26 +/- 4
mm, p <0.01). Resting heart rate and aortic now velocities were simil
ar in the 2 groups, but systemic arterial pulse pressure was greater i
n the Marfan group (50 +/- 12 mm Hg) than in the controls (41 +/- 8 mm
Hand p <0.01). Aortic diameter increased with age in both age groups,
but at all ages the Marfan group exhibited greater aortic diameters (
p <0.05). Aortic distensibility was less in the Marfan group (2.6 +/-
1.3 cm(2) . dynes(-1) . 10(-6)) than in the controls (6.2 +/- 2.1 cm(2
) . dynes(-1) . 10(-6), p <0.01), and the aortic wall stiffness index
was greater in the Marfan group (7.9 +/- 3.4) than in the controls (2.
8 +/- 0.6, p <0.01). Aortic wall stiffness increased with age and aort
ic diameter, but at all ages the Marfan group exhibited a stiffer aort
a for a given diameter than did the controls. Systemic pulse wave velo
city was greater in the Marfan group (621 +/- 139 cm . s(-1)) than in
the controls (394 +/- 62 cm . s(-1), p <0.01). In patients with the Ma
rfan syndrome, the aorta exhibits markedly abnormal elastic properties
from an early age, including increased stiffness and increased system
ic pulse wave velocities.