AORTIC REFERENCE CENTER
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Loeys Dietz Syndrome and the Aorta

Loeys-Dietz syndrome is a connective tissue disorder that is manifested hypertelorism (widely spaced eyes) and a bifid or broad uvula. Abnormal scarring, translucent, thin skin is a common feature. These patients have tortuous blood vessels that are prone to aneurysm formation and dissection.  The most common area of aneurysm formation is the aortic root.  Many patient are misdiagnosed as having Marfan syndrome.  Loeys-Dietz patients need surgery on the aortic root when diameter is 4 cm since dissections have been documented at this diameter. Valve-sparing root replacement is the operation of choice for this group of patients.   






  • Home
  • Basics
    • Glossary
    • Anatomy
    • What is an aortic aneurysm?
    • SURVEY RESULTS
  • Aortic Diseases
    • Acute Aortic Dissections >
      • Type A Aortic Dissection
      • Type B aortic dissection
    • Aortic Aneurysms >
      • Aortic Root Aneurysms >
        • David Procedure
      • Ascending Aortic Aneurysms
      • Aortic Arch Aneurysms
      • Descending and Thoracoabdominal Aortic Aneurysms >
        • Spinal cord/paraplegia
        • Descending Thoracic Aortic Aneurysms
        • Thoracoabdominal Aortic Aneurysms
      • Abdominal Aortic Aneurysms
  • Disorders
    • Marfan Syndrome
    • Loeys-Dietz Syndrome
    • Ehlers-Danlos Syndrome
    • Turner's Syndrome
  • Bicuspid AV
  • Programs
    • Aneurysm Surveillance Program >
      • Sign-up for surveillance program
      • Basics of the Surveillance Program
  • Research
    • Animal Laboratory
    • Clinical Research
    • Basic Science
  • Contact us
  • Glossary
  • Case of the Month
  • Research Survey
  • Aortic Conference
  • NEWSLETTER
  • Health Products