Women with Turner syndrome have twenty times higher risk of having an aortic dissection compared to the general population

Background Aortic dissection in women with Turner syndrome have been reported to be up to 60 times higher compared to the general population. Due to the increased risk, specific guidelines for Turner syndrome, including cardiovascular monitoring, were introduced in 2000 and later revised in 2016 and 2024. However, no studies to date have assessed whether these guidelines have effectively reduced the risk of aortic dissection within this population. Aim To examine the incidence of aortic dissection in a national registry cohort and evaluate whether the implementation of the 2016 guidelines have reduced the risk of dissection. Methods This registry-based cohort study included all women with Turner syndrome living in Sweden between 1987 and 2023 (n=1,465). Participants were identified through the Swedish National Inpatient and Outpatient Registry (NPR), with data collected from both the NPR and the Swedish National Cause of Death Registry. For both registries the overall coverage is approximately >95 % for cardiovascular events. Out of these women 1258 had Turner diagnosis and were alive at any time during 2001-2016, and 1329 women had Turner diagnosis and were alive at any time during the time-period 2017-2023. Endpoints: Time to first aortic dissection or rupture, and time to death due to aortic dissection or rupture, over two time periods. Results Among the 1,465 women with Turner syndrome, 36 experienced an aortic dissection or rupture. The event rate for aortic dissection/rupture was 116 events per 100,000 patient-years between 2001 and 2016, decreasing slightly to 109 events per 100,000 patient-years from 2017 to 2023. Mortality due to aortic dissection/rupture declined from 51 to 30 deaths per 100,000 patient-years between the two periods. Conclusion Aortic dissection is over twenty times more common in women with Turner syndrome compared to contemporary reports on Swedish women from the general population. Following the implementation of the Turner guidelines in 2016, there appears to be a slight decline in aortic dissection cases and a reduction of more than 40% in deaths due to dissection or rupture. This could suggest a potential preventive effect or a reduction in diagnostic delays when Turner patients seek medical attention for chest pain.