Aortic growth rates in a Swedish cohort of women with Turner syndrome.

Background: Aortic dilation, cardiac malformations and hypertension are known risk factors for aortic dissection in Turner syndrome (TS). In the current guidelines, rapid growth of the aorta has been added as a risk marker. This study aimed to estimate the growth of the ascending aorta over time, to identify risk factors of aortic growth, and to describe aortic complications in TS.

Methods: A transthoracic echocardiogram was performed at least twice in 101 women with TS, mean age 28 years, with a mean follow-up of 8.3 ± 3.4 (range 1-17) years. The investigator was blinded to the clinical status. Logistic regression analysis was used to identify risk factors of aortic growth.

Results: The prevalence of ascending aortic dilation (ASI >20 mm/m) was 26 % and the mean ascending aortic diameter was 27.0 ± 4.8 mm at baseline. Significant aortic growth was found at sinus of Valsalva 1.08 (±2.11) mm, sinotubular junction 1.07 (±2.23) mm, and the ascending aorta 2.32 (±2.93) mm, p < 0.001. The mean ascending aortic growth rate was 0.25 (±0.35) mm/year, and higher compared to the general female population, 0.12 (±0.05) mm/year, p < 0.0001. No risk factors for aortic growth (bicuspid aortic valve, coarctatio, hypertension or karyotype) other than body weight could be identified, Odds ratio 1.05 (95 % CI 1.00-1.09), p = 0.029. Eight women had an aortic event of whom all had bicuspid aortic valves.

Conclusions: The growth rate of the ascending aorta in TS was increased compared to the general female population. Congenital cardiovascular malformations were not predictive of aortic growth.