Aortic size predicts aortic dissection in Turner syndrome—a 25-year prospective cohort study
Open accessAbstract
Background Women with Turner syndrome (TS) have an increased risk of aortic dissection. The current recommended cutoff to prevent aortic dissection in TS is an aortic size index (ASI) of ≥2.5 cm/m 2 . This study estimated which aortic size had the best predictive value for the risk of aortic dissection, and whether adjusting for body size improved risk prediction. Methods A prospective, observational study in Sweden, of women with TS, n = 400, all evaluated with echocardiography of the aorta and data on medical history for up to 25 years. Receiver operating characteristic (ROC) curves, sensitivity and specificity were calculated for the absolute ascending aortic diameter (AAD), ascending ASI and TS specific z-score. Results There were 12 patients (3%) with aortic dissection. ROC curves demonstrated that absolute AAD and TS specific z-score were superior to ascending ASI in predicting aortic dissection. The best cutoff for absolute AAD was 3.3 cm and 2.12 for the TS specific z-score, respectively, with a sensitivity of 92% for both. The ascending ASI cutoff of 2.5 cm/m 2 had a sensitivity of 17% only. Subgroup analyses in women with an aortic diameter ≥ 3.3 cm could not demonstrate any association between karyotype, aortic coarctation, bicuspid aortic valve, BMI, antihypertensive medication, previous growth hormone therapy or ongoing estrogen replacement treatment and aortic dissection. All models failed to predict a dissection in a pregnant woman. Conclusions In Turner syndrome, absolute AAD and TS-specific z-score were more reliable predictors for aortic dissection than ASI. Care should be taken before and during pregnancy.
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The risk of aortic dissection in TS is increased in those with increased aortic diameter.
“The risk of aortic dissection in TS is increased in those with increased aortic diameter.232”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [232]
The most frequently used indexing methods are the Z-score, the aortic height index (AHI), and the aortic size index (ASI).
“The most frequently used indexing methods are the Z-score (Z, dimensionless unit indicating the number of standard deviations from the population mean), the aortic height index (AHI, aortic diameter in millimeters divided by body length in meters), and the aortic size index (ASI, aortic diameter in centimeters divided by body surface area (BSA) per square meters) (Table 12).232,233,441-443”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [232]
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- Authors
- Thunström, S., Thunström, E., Naessén, S., Berntorp, K., Laczna Kitlinski, M., Ekman, B., Wahlberg, J., Bergström, I., Bech-Hanssen, O., Krantz, E., Laine, C. M., Bryman, I., & Landin-Wilhelmsen, K.
- Affiliations
- Sahlgrenska University Hospital (SE)
- University of Gothenburg (SE)
- Karolinska Institutet (SE)
- Lund University (SE)
- Linköping University (SE)
- Örebro University (SE)
- Karolinska University Hospital (SE)
- Hallands sjukhus Halmstad (SE)
- Corresponding author
- Thunström, Sofia
- ORCID
- Venue
- International Journal of Cardiology
- Publisher
- Elsevier BV
- Published
- 2023-02
- Volume
- 373
- Pages
- 47-54
- ISSN
- 0167-5273 (ISSN-L)
- OpenAlex
- W4309670341
- Cited by
- 26
- References
- 24
- Citations by year
- 2026: 6 · 2025: 8 · 2024: 5 · 2023: 7
- FWCI
- 3.22
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- International Journal of Cardiology (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- KTH Publication Database DiVA (KTH Royal Institute of Technology) (submittedVersion, other-oa)
- Lund University Publications (Lund University) (submittedVersion)
- Funders
- Heart and Lung Foundation
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Keywords
- Turner syndrome, aortic dissection, aortic size index, ascending aortic diameter, risk prediction, coarctation of the aorta, bicuspid aortic valve, pregnancy, women
- MeSH terms
- Aortic Dissection, Aorta, Aortic Coarctation, Female, Humans, Pregnancy, Prospective Studies, Turner Syndrome
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