| アブストラクト | 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. |
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| ジャーナル名 | Circulation journal : official journal of the Japanese Circulation Society |
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| Pubmed追加日 | 2026/8/23 |
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| 投稿者 | Ikeda Kurakawa, Kayo; Okada, Akira; Kawai, Masanobu; Inoue, Takafumi; Inoue, Reiko; Matsuo, Yuichiro; Fushimi, Kiyohide; Matsui, Hiroki; Yasunaga, Hideo; Yamauchi, Toshimasa; Nangaku, Masaomi; Kadowaki, Takashi; Yamaguchi, Satoko |
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| 組織名 | Department of Prevention of Diabetes and Lifestyle-Related Diseases, Graduate;School of Medicine, The University of Tokyo.;Department of Molecular Genetics and Endocrinology, Osaka Women's and Children's;Hospital.;Research Committee on Clinical Management of Growth Disorders and Differences of;Sex Development, Research on Measures for Intractable Diseases, Health and Labor;Sciences Research Grants.;Department of Cardiovascular Surgery, Saitama Eastern Cardiovascular Hospital.;Department of Clinical Epidemiology and Health Economics, Graduate School of;Medicine, The University of Tokyo.;Department of Health Policy and Informatics, Institute of Science Tokyo.;Department of Diabetes and Metabolic Diseases, Graduate School of Medicine, The;University of Tokyo.;Division of Nephrology and Endocrinology, Graduate School of Medicine, The;Toranomon Hospital. |
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| Pubmed リンク | https://www.ncbi.nlm.nih.gov/pubmed/42633938/ |
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