Aortic Dissection or Aneurysm and In-Hospital Mortality in Patients With Turner Syndrome　- A Retrospective Cohort Study in Japan.

BACKGROUND: Aortic dissection and aneurysm are major causes of early mortality in Turner syndrome. However, data on aortic dissection in Turner syndrome remain limited compared with those for Marfan syndrome. We evaluated the associations of Turner syndrome and Marfan syndrome with in-hospital mortality among female patients hospitalized for aortic dissection or aneurysm, using patients without either syndrome as the common reference group for both comparisons. METHODS AND RESULTS: This retrospective cohort study used data from the Diagnosis Procedure Combination, a national inpatient database in Japan. Adult female patients hospitalized for first-time aortic dissection or aneurysm between 2010 and 2022 were included, with in-hospital mortality as the primary outcome. Associations between Turner/Marfan syndrome and mortality were evaluated using multivariable logistic regression analysis. Of 10,927 eligible female patients aged 16-61 years, 29 (0.27%) had Turner syndrome and 686 (6.3%) had Marfan syndrome. In-hospital mortality was higher for Turner syndrome (odds ratio [OR] 5.35; 95% confidence interval [CI] 1.61-17.8) compared with the group without either syndrome. In contrast, Marfan syndrome was not significantly associated with mortality (OR 0.72; 95% CI 0.44-1.17). Unscheduled admission was similar in Turner syndrome, but less frequent in Marfan syndrome, compared with the group without either syndrome. CONCLUSIONS: In-hospital mortality was higher among patients with Turner syndrome admitted for aortic dissection or aneurysm, highlighting the need to raise awareness of aortic risk in this high-risk population.