Assessment of aortic morphology and compliance in children and adolescents with Ullrich-Turner syndrome (UTS) using magnetic resonance imaging (MRI)

• Published in: Journal of Pediatric Endocrinology & Metabolism Vol. 27; no. 9; pp. 915 - 922
• Main Authors: Kneppo, Carolin, Eichhorn, Joachim G., Ley, Sebastian, Schenk, Jens P., Gorenflo, Matthias, Bettendorf, Markus
• Format: Journal Article
• Language: English
• Published: Germany De Gruyter 01.09.2014; Walter de Gruyter GmbH
• Subjects: Adolescent; Aorta - pathology; aortic compliance; Aortography; Child; Cross-Sectional Studies; Female; Humans; Magnetic Resonance Imaging; Male; MR angiography; Prospective Studies; Turner Syndrome - diagnostic imaging; Turner Syndrome - pathology; UTS
• ISSN: 0334-018X; 2191-0251
• DOI: 10.1515/jpem-2013-0332

The aim of this study was to evaluate the morphology and elastic properties of the aorta in children and adolescents with Ullrich-Turner syndrome (UTS) treated with growth hormone, by using magnetic resonance imaging (MRI). Thirty-seven conscious UTS patients were examined using a 1.5-T whole-body MRI. Contrast-free three-dimensional (3D)-MR angiographies were performed, including 2D cine MRI, to calculate the aortic compliance (C) and cine of the aortic valve. Changes of aortic morphology were evident in 40% of the patients, whereas six had more than one alteration. A bicuspid aortic valve was identified in three patients that were missed by previous echocardiography. The aortic compliances in UTS patients were similar to those in healthy persons. This study shows that aortic morphology and compliance can be assessed by MRI without using contrast agents and without sedation in children and adolescents with UTS.