Aortic dilatation and dissection in Turner syndrome

Abstract

Background: The risk for aortic dissection is increased among relatively young women with Turner syndrome (TS). It is unknown whether aortic dilatation precedes acute aortic dissection in TS and, if so, what specific diameter predicts impending deterioration.

Methods and results: Study subjects included 166 adult volunteers with TS (average age, 36.2 years) who were not selected for cardiovascular disease and 26 healthy female control subjects. Ascending and descending aortic diameters were measured by magnetic resonance imaging at the right pulmonary artery. TS women were on average 20 cm shorter, yet average aortic diameters were identical in the 2 groups. Ascending aortic diameters normalized to body surface area (aortic size index) were significantly greater in TS, and approximately 32% of TS women had values greater than the 95th percentile of 2.0 cm/m2. Ascending diameter/descending diameter ratios also were significantly greater in the TS group. During approximately 3 years of follow-up, aortic dissections occurred in 3 women with TS, for an annualized rate of 618 cases/100,000 woman-years. These 3 subjects had ascending aortic diameters of 3.7 to 4.8 cm and aortic size indices > 2.5 cm/m2.

Conclusions: The risk for aortic dissection is greatly increased in young women with TS. Because of their small stature, ascending aortic diameters of < 5 cm may represent significant dilatation; thus, the use of aortic size index is preferred. Individuals with a dilated ascending aorta defined as aortic size index > 2.0 cm/m2 require close cardiovascular surveillance. Those with aortic size index > or = 2.5 cm/m2 are at highest risk for aortic dissection.

Trial registration: ClinicalTrials.gov NCT00006334.