Mj. Roman et al., PROGNOSTIC-SIGNIFICANCE OF THE PATTERN OF AORTIC ROOT DILATION IN THEMARFAN-SYNDROME, Journal of the American College of Cardiology, 22(5), 1993, pp. 1470-1476
Objectives. The present study examines the incidence of aortic complic
ations (dissection, marked dilation requiring surgery or progressive m
oderate to severe aortic regurgitation) and their relation to clinical
features and aortic root morphology in patients with the Marfan syndr
ome. Background. Considerable phenotypic variability exists in the Mar
fan syndrome, and the prospective prediction of the risk for aortic co
mplications in individual patients remains elusive. Methods. One hundr
ed thirteen patients with the Marfan syndrome underwent anthropometric
and echocardiographic evaluation and were followed up for 49 +/- 24 (
mean +/- SD) months. Aortic root dilation was defined as localized whe
n confined to the sinuses of Valsalva (based on two dimensional echoca
rdiographic confidence limits utilizing age and body size) and general
ized if dilation additionally involved the supraaortic ridge and proxi
mal ascending aorta. Results. Aortic root dilation was present in 80%
of patients and was localized in 28% and generalized in 51%. Aortic co
mplications occurred during follow up in none of 23 patients with norm
al initial aortic size, in 2 (6%) of 32 patients with initially locali
zed dilation and in 19 (33%) of 58 patients with generalized dilation
(p < 0.0005). Complications were associated with larger initial aortic
size (p < 0.00005), higher systolic blood pressure (p < 0.005), heigh
t (p < 0.05), aortic growth rate (p < 0.05) and older age (p < 0.01).
The only independent predictor of aortic complications was initial aor
tic root size (p < 0.005). However, when aortic size, one of the indic
ations for surgical referral, was excluded from analyses, the only ind
ependent predictor of aortic complications was generalized aortic dila
tion (p < 0.005). Conclusions. The present study indicates that genera
lized aortic root dilation is a potent marker of an increased risk for
subsequent aortic complications in Marfan syndrome.