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Aortic size predicts aortic dissection in Turner syndrome - A 25-year prospective cohort study

Thunström, Sofia ; Thunström, Erik ; Naessén, Sabine ; Berntorp, Kerstin LU ; Laczna Kitlinski, Margareta ; Ekman, Bertil ; Wahlberg, Jeanette ; Bergström, Ingrid ; Bech-Hanssen, Odd and Krantz, Emily , et al. (2023) In International Journal of Cardiology 373. p.47-54
Abstract

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/m2. 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:... (More)

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/m2. 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/m2 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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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Aortic dissection, Echocardiography, Prophylactic surgery, Risk factor, ROC curve, Sensitivity
in
International Journal of Cardiology
volume
373
pages
47 - 54
publisher
Elsevier
external identifiers
  • scopus:85142780121
  • pmid:36410543
ISSN
0167-5273
DOI
10.1016/j.ijcard.2022.11.023
language
English
LU publication?
yes
id
822edf9f-8eac-48e3-a4eb-4cd1b83ec48b
date added to LUP
2023-01-31 14:17:35
date last changed
2024-06-11 01:39:51
@article{822edf9f-8eac-48e3-a4eb-4cd1b83ec48b,
  abstract     = {{<p>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<sup>2</sup>. 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<sup>2</sup> 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.</p>}},
  author       = {{Thunström, Sofia and Thunström, Erik and Naessén, Sabine and Berntorp, Kerstin and Laczna Kitlinski, Margareta and Ekman, Bertil and Wahlberg, Jeanette and Bergström, Ingrid and Bech-Hanssen, Odd and Krantz, Emily and Laine, Christine M. and Bryman, Inger and Landin-Wilhelmsen, Kerstin}},
  issn         = {{0167-5273}},
  keywords     = {{Aortic dissection; Echocardiography; Prophylactic surgery; Risk factor; ROC curve; Sensitivity}},
  language     = {{eng}},
  pages        = {{47--54}},
  publisher    = {{Elsevier}},
  series       = {{International Journal of Cardiology}},
  title        = {{Aortic size predicts aortic dissection in Turner syndrome - A 25-year prospective cohort study}},
  url          = {{http://dx.doi.org/10.1016/j.ijcard.2022.11.023}},
  doi          = {{10.1016/j.ijcard.2022.11.023}},
  volume       = {{373}},
  year         = {{2023}},
}