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.