Cardiac MRI and 3D contrast-enhanced MR angiography in pediatric and young adult patients with Turner syndrome

Authors: HASAN YİĞİT, AŞAN ÖNDER, SENEM ÖZGÜR, ZEHRA AYCAN, SELMİN KARADEMİR, VEHBİ DOĞAN

Abstract: Background/aim: This study aimed to describe the spectrum and frequency of cardiovascular abnormalities in pediatric and young adult patients with Turner syndrome (TS) using cardiac MRI and MR angiography. Materials and methods: This prospective study consisted of 47 female patients of pediatric age and young adults with a karyotypically confirmed diagnosis of TS. All patients underwent cardiac MRI and contrast-enhanced MR angiography. A second examination after 9-26 months was performed for 28 of these patients. Results: Elongation of the transverse aortic arch (ETA) was the most frequent abnormality with a rate of 37%. The rate of partial anomalous pulmonary venous connection (PAPVC) was 21.7%, bicuspid aortic valve (BAV) was 19.6%, coarctation was 6.5%, ascending aorta dilatation was 28.3%, and descending aorta dilatation was 15.2%. The diameters of the aorta and the rate of aortic dilatation per unit of time was greater in the patients with BAV (P < 0.05). ETA was less observed in the patients who were receiving growth hormone therapy (P < 0.05). Conclusion: The most common cardiovascular abnormalities in TS patients are aortic arch anomalies such as ETA and coarctation, aortic dilatation, PAPVCs, and BAV. The presence of BAV is an important risk factor for the aortic dilatation.

Keywords: Ullrich-Turner syndrome, cardiovascular system, aortic arch, magnetic resonance imaging, magnetic resonance angiography

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