- Poster presentation
- Open Access
An evaluation of vascular anomalies and incidental findings in patients with Turner syndrome
© Kanfi et al; licensee BioMed Central Ltd. 2012
- Published: 1 February 2012
- Aortic Arch
- Subclavian Artery
- Superior Vena Cava
- Turner Syndrome
- Bicuspid Aortic Valve
Multiple thoracic vascular anomalies have been described in the setting of Turner syndrome. Careful evaluation of both venous and arterial anatomy in the chest is prudent when evaluating these cases. By highlighting the less commonly associated vascular malformations in Turner syndrome through key images, we intend to raise awareness and familiarity of these potentially clinically significant anomalies. We also hope to reinforce the importance of evaluating extra-cardiac structures to uncover the additional incidental but significant findings.
Prior studies have demonstrated a high prevalence of congenital cardiovascular malformations in Turner syndrome patients. Classic associations with bicuspid aortic valves and coarctation of the aorta are well known, but with the development of 3D MR angiography, additional vascular malformations have been highlighted. Patients with Turner syndrome have increased associations with aortic arch anomalies, including bovine arch configurations and aberrant right subclavian artery origins. Additionally, venous drainage malformations such as partial anomalous pulmonary venous return (PAPVR) and persistent left-sided superior vena cava (SVC) have been identified.
We reviewed a total of 29 cardiac Magnetic Resonance Imaging (MRI) exams performed on twenty patients with Turner syndrome. The studies were performed on General Electric HDX 1.5 Tesla MRI machines with gadolinium enhanced magnetic resonance angiograms (MRA) of the thoracic vessels. Image post-processing was performed using a General Electric Advanced Windows workstation.
Several vascular anomalies were noted: Aortic coarctation (30%; 6 patients), PAPVR (15%, 3 patients), bicuspid aortic valves (45%, 9 patients), persistent left sided SVCs, (25%, 5 patients), and aberrant right subclavian arteries (15%, 3 patients). One patient had an aortic arch origin of the left vertebral artery. Two patients demonstrated two right renal arteries. Additional incidental MR findings, such a hepatic hemangioma and a thyroid mass were also seen in two patients.
This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.