Heart and Turner syndrome.
Turner syndrome (TS) is a rare disease (ORPHA #881) which affects about 50 in 100 000 newborn girls. Their karyotype shows a complete or partial loss of the second X chromosome. In TS, congenital cardiovascular malformations, such as bicuspid aortic valves and aortic coarctation are frequent, affecting 20-30% and 7-18% of the TS population, respectively. The morbidity and mortality of these patients are high and related to the presence of hypertension and/or aortic dilatation (40%), inducing aortic dissection. European guidelines published in 2017 have indicated how to monitor patients using magnetic resonance imaging (MRI) and/or echography. Different studies have shown that a cardiovascular lifelong follow-up is necessary and therefore education of patients with TS and their families represents a major issue. This review will present recent data concerning the progression of aortic diameters as well as current molecular knowledge of the cardiovascular system in patients with TS.
Copyright © 2020 Elsevier Masson SAS. All rights reserved.
Overview publication
| Title | Heart and Turner syndrome. |
| Date | 2021-06-01 |
| Issue name | Annales d'endocrinologie |
| Issue number | v82.3-4:135-140 |
| DOI | 10.1016/j.ando.2020.12.004 |
| PubMed | 33307001 |
| Authors | |
| Keywords | Aortic coarctation, Aortic dilatation, Aortic dissection, Bicuspid aortic valve, Coarctation aortique, Dilatation aortique, Dissection aortique, Grossesse, Heart disease, Maladie cardiaque, Maladie rare, Pregnancy, Rare disease, Syndrome de Turner, Turner syndrome, Valve aortique bicuspide |
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