Prognostic significance of the pattern of aortic root dilation in the Marfan syndrome.

OBJECTIVES

The present study examines the incidence of aortic complications (dissection, marked dilation requiring surgery or progressive moderate to severe aortic regurgitation) and their relation to clinical features and aortic root morphology in patients with the Marfan syndrome.

BACKGROUND

Considerable phenotypic variability exists in the Marfan syndrome, and the prospective prediction of the risk for aortic complications in individual patients remains elusive.

METHODS

One hundred 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 when confined to the sinuses of Valsalva (based on two-dimensional echocardiographic confidence limits utilizing age and body size) and generalized if dilation additionally involved the supraaortic ridge and proximal ascending aorta.

RESULTS

Aortic root dilation was present in 80% of patients and was localized in 28% and generalized in 51%. Aortic complications occurred during follow-up in none of 23 patients with normal initial aortic size, in 2 (6%) of 32 patients with initially localized 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), height (p < 0.05), aortic growth rate (p < 0.05) and older age (p < 0.01). The only independent predictor of aortic complications was initial aortic root size (p < 0.005). However, when aortic size, one of the indications for surgical referral, was excluded from analyses, the only independent predictor of aortic complications was generalized aortic dilation (p < 0.005).

CONCLUSIONS

The present study indicates that generalized aortic root dilation is a potent marker of an increased risk for subsequent aortic complications in Marfan syndrome.