Vascular disease

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The article covers vascular disease. Vasculitides are covered in a separate article called vasculitides.

Great vessels

When things go wrong here, you see a cardiac surgeon.

Aortic dissection

Associations

Heritable:[1]

  • Marfan's syndrome.
  • Loeys-Dietz syndrome.[2]
    • Marfan-like syndrome.
  • Ehlers-Danlos syndrome.
  • Bicuspid aortic valve.

Others:

  • Tertiary syphilis.

Cystic medial degeneration

General

  • AKA cystic medial necrosis.[3]
    • Often not cystic and not necrotic.

Microscopic

Features:[4][5]

  • Basophilic ground substance in the media (seen on Movat's stain).
  • Disruption of the elastic lamina (seen on elastic trichrome stain).
  • +/-Focal necrosis.

Images: Marfan's syndrome (radiographics.rsna.org).

Medial calcific sclerosis

  • AKA Moenckeberg medial calcific sclerosis.

General

  • Usually of no clinical consequence.

Microscopic

Features:[6]

  • Medial calcification (purple irregular stuff -- calcium phosphate).

Note:

  • Lumen unaffected.

See also

References

  1. Gleason TG (2005). "Heritable disorders predisposing to aortic dissection". Semin. Thorac. Cardiovasc. Surg. 17 (3): 274-81. doi:10.1053/j.semtcvs.2005.06.001. PMID 16253833.
  2. Loeys BL, Schwarze U, Holm T, et al (August 2006). "Aneurysm syndromes caused by mutations in the TGF-beta receptor". N. Engl. J. Med. 355 (8): 788-98. doi:10.1056/NEJMoa055695. PMID 16928994. http://content.nejm.org/cgi/content/full/355/8/788.
  3. URL: http://emedicine.medscape.com/article/756835-overview. Accessed on: 12 August 2010.
  4. URL: http://emedicine.medscape.com/article/756835-overview. Accessed on: 12 August 2010.
  5. Ha HI, Seo JB, Lee SH, et al. (2007). "Imaging of Marfan syndrome: multisystemic manifestations". Radiographics 27 (4): 989–1004. doi:10.1148/rg.274065171. PMID 17620463. http://radiographics.rsna.org/content/27/4/989.full.
  6. Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 7. ISBN 978-1416002741.