title: Vascular manifestations of syndromic aortopathies: role of current and emerging imaging techniques.
abstract: Patients with connective tissue diseases such as Marfan's syndrome, Loeys-Dietz syndrome, and vascular Ehlers-Danlos syndrome comprise a small but important group of patients who present early with acute aortic syndrome comprising aneurysmal dilation, rupture, or aortic dissection. Cardiovascular pathologies are an important yet treatable cause of morbidity and mortality in these patients. Imaging plays an important role in initial diagnosis, surveillance, and identification of complications. Furthermore, these patients are prone to developing complications in other vascular territories. Effective screening and surveillance will allow early diagnosis and elective treatment thus reducing the morbidity and mortality associated with presentation with acute complications. In this article, we will provide an overview of the role of magnetic resonance and computed tomography angiography in the management of syndromic aortopathies.
authors.affiliation: Department of Radiology, Guy's and St Thomas' NHS Foundation Trust, UK.
authors.forename: O
authors.initials: O
authors.lastname: Westerland
authors.affiliation: Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, UK.
authors.forename: A
authors.initials: A
authors.lastname: Frigiola
authors.affiliation: Department of Clinical Genetics, Guy's and St Thomas' NHS Foundation Trust, UK.
authors.forename: L
authors.initials: L
authors.lastname: Robert
authors.affiliation: Department of Radiology, Guy's and St Thomas' NHS Foundation Trust, UK.
authors.forename: A
authors.initials: A
authors.lastname: Shaw
authors.affiliation: Department of Radiology, Guy's and St Thomas' NHS Foundation Trust, UK.
authors.forename: L
authors.initials: L
authors.lastname: Blakeway
authors.affiliation: Department of Radiology, Guy's and St Thomas' NHS Foundation Trust, UK.
authors.forename: K
authors.initials: K
authors.lastname: Katsanos
authors.affiliation: Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, UK.
authors.forename: C
authors.initials: C
authors.lastname: Kiesewetter
authors.affiliation: Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, UK.
authors.forename: N
authors.initials: N
authors.lastname: Chung
authors.affiliation: Department of Radiology, Guy's and St Thomas' NHS Foundation Trust, UK. Electronic address: narayan.karunanithy@gstt.nhs.uk.
authors.forename: N
authors.initials: N
authors.lastname: Karunanithy
mesh_terms: D000784:Aortic Dissection / Q000150:complications* / Q000175:diagnosis; D000792:Angiography; D001011:Aorta / Q000473:pathology*; D001014:Aortic Aneurysm / Q000150:complications* / Q000175:diagnosis; D003240:Connective Tissue Diseases / Q000150:complications*; D006801:Humans; D008279:Magnetic Resonance Imaging; D014057:Tomography, X-Ray Computed