Endocrine Abstracts

Introduction: Women with Turner syndrome (TS) have an increased risk of aortic dissection. Aortic dilatation, bicuspid aortic valve (BAV) and hypertension confer increased risk of dissection. However, only some women with these risk factors develop dissection, and others with no risk markers may dissect. Knowledge of the development of the aortopathy over time is limited. We investigate aortic dimension changes in unselected adult TS and associations between aortic growth and risk factors for dissection.

Methods: TS-women aged >16 y with a baseline and follow-up transthoracic echocardiography (TTE). Exclusion criteria: scans with poor visualization. Ascending aorta (AA) and sinuses were assessed by two cardiologists. Age at baseline-TTE, BAV, hypertension and baseline aortic measurements were analyzed.

Results: Sixty-four TS-women who had TTE at baseline age 32±13 years (17–59) with TTE follow-up of 4.9 years (0.7–12.9) were included. Mean baseline measurements were: AA 26.4±4.2 mm (median 25) and sinuses 27.5±4.4 mm (median 27). The aortic growth rate/year was at AA 0.29±0.92 mm/y (−2.41 – 2.72; median 0.22) and at sinuses 0.08±1.06 mm/y (−2.9 – 3.7; median 0.00). One woman experienced dissection; aortic growth was at sinuses 3.4 mm/y and at AA 1.4 mm/y. Women with BAV (13/64) showed higher growth at sinuses (0.90±1.4 mm, P=0.001). Age at baseline and hypertension were not associated with aortic growth. Aortic growth was not dependent on baseline aortic diameter.

Conclusions: This long follow-up study showed a rapid rate of aortic growth in TS compared to general population (0.07 mm/year). Enlargement at aortic sinuses was accelerated in the presence of BAV. We suggest that risk stratification for aortic dissection in TS should include assessment of changes over time in proximal aortic diameter and in the presence of a rapid aortic growth closer follow-up is needed.