PROGNOSTIC-SIGNIFICANCE OF THE PATTERN OF AORTIC ROOT DILATION IN THEMARFAN-SYNDROME

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.