Aortic involvement in Turner syndrome
M Ilyas, SL Atkin, V Mathew, C Chu & D Ettles
Background: Turner syndrome has well recognised cardiovascular complications that appear in up to 40% of the patients and are more common in monosomy X. Left sided obstructive lesions are relatively more frequent and predispose to aortic root dilatation and life threatening aortic dissection. Patients with bicuspid aortic valve, hypertension, coarctation and aortic stenosis are at high risk of aortic dilatation and dissection. Various follow up strategies are in use but there is no clear guideline regards the best single test for monitoring this progression.
Study: Routine MR imaging of aorta was introduced with initiation of a dedicated Turner Syndrome clinic with the aim repeated MRI every two years. It was found that seven patients out of a total of seventeen developed aortic anomalies during the course of their illness that included coarctation as well as dilatation. None of these patients had any cardiovascular symptoms and the vascular abnormalities were detected on MR imaging either at presentation or during the course of their follow up. We found that in patients with previously normal aortic imaging, the time interval for the lesion to be detectable varied between 4 and 6 years, though in one patient there was progression of an established lesion over the two years period.
Conclusion: In the few patients presented here, regular imaging every two years would appear to be warranted, though the exact frequency of imaging and by what modality needs still to be ascertained more definitively.