Prevalence and progression of aortic dilatation in adult patients with Turner syndrome: a cohort study - Sorbonne Université Accéder directement au contenu
Article Dans Une Revue European Journal of Endocrinology Année : 2020

Prevalence and progression of aortic dilatation in adult patients with Turner syndrome: a cohort study

Résumé

Objective: Turner syndrome (TS) is a rare disorder affecting 1/2500 female newborn. Aortic dilatation (AD) and aortic dissection represent a major concern in TS. The aims of our study were to describe the aortic root growth, potential aortic dilatation (AD) risk factors and cardiovascular outcomes in a cohort of patients with TS. Methods: Among 204 adult patients included, 197 were studied using a standardized 1.5 Tesla MRI protocol. AD was defined as an aortic diameter ≥20 mm/m2 at the Valsalva sinuses and/or at the ascending aorta, when indexed to body surface area. Results: At baseline, AD was present in 81/197 (41.1%) and 32/197 (16.2%) of patients, at the levels of Valsalva and ascending aorta, respectively. The aortic Valsalva diameter was larger in patients treated for thyroiditis (P < 0.001). Potential risk factors of AD were aging (P < 0.001) and the presence of bicuspid aortic valve (BAV) (P = 0.002). The hazard ratio (HR) of AD occurrence in the presence of BAV was 2.2 (95% CI: 1.33-3.71). After a median follow-up period of 5.1 years (n = 143), AD was present in 58/143 (40.6%) and 25/143 (17.5%) of patients at the levels of Valsalva and ascending aorta, respectively. The median aortic growth of the Valsalva sinuses remained stable. At the ascending aorta, it increased by 0.14 ± 0.61 mm/year. Only one aortic-related death was observed. Conclusion: AD is common in adult patients with TS. However, our results are rather reassuring, as the median aortic diameters remained stable after 5.1 years and few aortic events were observed.
Fichier principal
Vignette du fichier
Donadille et al. - 2020 - Prevalence and progression of aortic dilatation in.pdf (1.25 Mo) Télécharger le fichier
Origine Fichiers produits par l'(les) auteur(s)
Loading...

Dates et versions

hal-02946683 , version 1 (23-09-2020)

Identifiants

Citer

Bruno Donadille, Sophie Tuffet, Clement Cholet, Mariana Nedelcu, Nathalie Bourcigaux, et al.. Prevalence and progression of aortic dilatation in adult patients with Turner syndrome: a cohort study. European Journal of Endocrinology, 2020, 183 (4), pp.463-470. ⟨10.1530/EJE-20-0284⟩. ⟨hal-02946683⟩
156 Consultations
132 Téléchargements

Altmetric

Partager

Gmail Mastodon Facebook X LinkedIn More