Aortic elasticity deterioration proves intrinsic abnormality of the ascending aorta in pediatric Turner syndrome unrelated to the aortic valve morphology
| العنوان: | Aortic elasticity deterioration proves intrinsic abnormality of the ascending aorta in pediatric Turner syndrome unrelated to the aortic valve morphology |
|---|---|
| المؤلفون: | Julian A. Heno, Diana-Alexandra Ertl, Gabriele Häusler, Talin Gulesserian, Christiane Pees, Ina Michel-Behnke |
| المصدر: | Heart and Vessels |
| بيانات النشر: | Springer Japan, 2018. |
| سنة النشر: | 2018 |
| مصطلحات موضوعية: | Aortic valve, Male, medicine.medical_specialty, Adolescent, Vascular Malformations, Coarctation of the aorta, Turner Syndrome, 030204 cardiovascular system & hematology, Vascular disease, Pediatrics, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Vascular Stiffness, Risk Factors, medicine.artery, Internal medicine, Turner syndrome, Ascending aorta, medicine, Genetics, Humans, Abnormalities, Multiple, Child, Aorta, business.industry, Infant, Newborn, Infant, medicine.disease, Elasticity, Cardiac surgery, Dissection, medicine.anatomical_structure, Echocardiography, Aortic Valve, Child, Preschool, Cardiology, cardiovascular system, Original Article, Female, Cardiology and Cardiovascular Medicine, business |
| الوصف: | Turner syndrome (TS) is a common genetic disorder in females with high incidence of ascending aortic dilatation and even dissection occurring as early as in the second decade. Known risk factors (RF) are bicuspid aortic valves (BAV), coarctation of the aorta (CoA), and arterial hypertension. Since 10% of dissections occur in patients without RF, an intrinsic aortic wall abnormality has been postulated. This study aimed to investigate the elasticity of the ascending aorta as a surrogate marker of aortic wall texture. Forty-six pediatric patients with genetically proven TS were prospectively examined for the morphology of their aortic valve, and size and elasticity indices of the adjacent aorta. Cohorts of 46 female subjects with tricuspid aortic valves (TAV) and ten non-syndromic females with BAV were investigated as separate control groups. Comparison of healthy controls with TS patients revealed significantly deteriorated elasticity indices in those with TS. Furthermore, normalized aortic dimensions were greater in TS patients, but dilatations of the ascending aorta with z-score levels above two were restricted to those with BAV (14/46). Deteriorated elasticity indices were measured in TS patients, independent of aortic dilatation, BAV, and CoA, and were comparable to those of patients with isolated, non-syndromic BAVs. By measuring elasticity levels as a surrogate for aortic wall texture, we were able to gather evidence that TS presents with an intrinsic abnormality of the ascending aorta even in patients without concomitant BAV, CoA or dilatations as early as in childhood. |
| اللغة: | English |
| تدمد: | 1615-2573 0910-8327 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2b511fc9ca42846aa8f163c905be46bbTest http://europepmc.org/articles/PMC6208677Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....2b511fc9ca42846aa8f163c905be46bb |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 16152573 09108327 |
|---|