google ads
Cardiac: Marfan Syndrome Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus
Imaging Pearls ❯ Cardiac ❯ Marfan Syndrome

-- OR --

  • “The classic phenotypic appearance of the aorta in patients with Marfan syndrome is annuloaortic ectasia, with dilatation of the aortic annulus and sinuses of Valsalva, and effacement of the sinotubular junction, resulting in a tulip-shaped configuration of the aortic root.”

    Pre- and Postoperative Imaging of the Aortic Root 
Hanneman K et al. 
RadioGraphics 2016; 36:19-37
  • “Aneurysm size is the primary indicator for surgical aortic root repair of asymptomatic aneurysms. In most instances, surgery is indicated for asymptomatic patients without another underlying cardiovascular condition or disease, with aneurysms measuring greater than or equal to 5.5 cm.” 


    Pre- and Postoperative Imaging of the Aortic Root 
Hanneman K et al. 
RadioGraphics 2016; 36:19-37
  • “Performing nonenhanced imaging is particularly important in the postoperative setting, as surgical material may be most conspicuous during this phase. Synthetic grafts are usually composed of polyethylene terephthalate and are slightly hyperattenuating on noncontrast CT images relative to the native aortic wall, and are typically visualized as a thin, curvilinear hyperattenuating structure.” 


    Pre- and Postoperative Imaging of the Aortic Root 
Hanneman K et al. 
RadioGraphics 2016; 36:19-37
  • “The sinotubular junction is the landmark between the aortic root and the tubular portion of the ascending aorta, and normally forms a waist in the aortic contour between the sinus of Valsalva and the remainder of the ascending aorta. The sinotubular junction is the highest level at which the aortic valve cusps and commissures are attached to the aortic wall.”

    Pre- and Postoperative Imaging of the Aortic Root 
Hanneman K et al. 
RadioGraphics 2016; 36:19-37
  • “Aneurysms of the ascending aorta result most often from cystic medial degeneration. Aortic root enlargement is associated with many underlying conditions including connective tissue disorders such as Marfan syndrome, bicuspid aortic valve, Loeys-Dietz syndrome, hypertension, and atherosclerosis. Less common underlying causes include infections such as syphilis, vasculitides, trauma, and familial thoracic aneurysm disease.” 


    Pre- and Postoperative Imaging of the Aortic Root 
Hanneman K et al. 
RadioGraphics 2016; 36:19-37
  • “Marfan syndrome is an autosomal dominant, inherited connective-tissue disorder that is mainly caused by mutations in the gene (FBN1) that encodes fibrillin-1. Approximately 25% of cases result from a sporadic new mutation in FBN1. Estimates of prevalence are approximately 1 case per 3500–5000 population.” 


    Pre- and Postoperative Imaging of the Aortic Root 
Hanneman K et al. 
RadioGraphics 2016; 36:19-37
  • “Bicuspid aortic valve is the most common congenital cardiac abnormality, with an estimated prevalence of 1%–2%. The inheritance pattern is autosomal dominant with incomplete penetrance. Bicuspid aortic valve shares common histopathologic findings with Marfan syndrome, including cystic medial degeneration, increased levels of matrix metalloproteinases, and decreased levels of fibrillin-1 in the aortic wall. The Sievers classificationof bicuspid aortic valve is based on the number of raphes and the positions of cusps and raphes. Fusion of the right and left coronary cusps is the most common type.”

    Pre- and Postoperative Imaging of the Aortic Root 
Hanneman K et al. 
RadioGraphics 2016; 36:19-37

Privacy Policy

Copyright © 2024 The Johns Hopkins University, The Johns Hopkins Hospital, and The Johns Hopkins Health System Corporation. All rights reserved.