Vascular disease

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The article covers vascular disease, i.e. diseases of blood vessels. These keep vascular surgeons and cardiac surgeon busy.

Vasculitides are covered in a separate article called vasculitides.

Normal blood vessels

Comparing arteries and veins:[1]

Feature Artery Vein
Internal elastic lamina (IEL) prominent/thick, usu. complete thin & incomplete
External elastic lamina (EEL) present, thick absent
Shape circular / lumen wide open collapsed
Wall thickness thick thin
 
Artery and vein. (WC)

Great vessels

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

Atherosclerosis

General

  • A leading cause of death, esp. in the Western world.
  • May have multi-system manifestations.

Location and associated pathology:

Clinical risk factors:

  • Age.
  • Blood pressure (high) - modifiable (antihypertensives).
  • Cholesterol - modifiable (statins, diet).
  • Diabetes mellitus - modifiable (hypoglycemic medications, diet, lifestyle).
  • Smoking - modifiable (cessation).
  • Family history.

Microscopic

Features:

  • Intimal hyperplasia.
  • Lipid deposition.
  • Foamy macrophages within intima & media.
  • Cholesterol clefts
  • Luminal narrowing.


Notes:

  • Considered "complex" if any of the following are present:[2]
    • Calcifications.
    • Thrombosis.
    • Haemorrhage.

Image

Stains

Aortic dissection

  • Abbreviated AoD.

General

Risk factors:

Note:

  • Diabetes uncommon in thoracic aortic dissection.[4]

May be separated into two groups:[7]

  1. Hypertension + atherosclerosis, typically 40-60 years old.
  2. Connective tissue disorders - "young".

Associations

Heritable:[8]

Others:

Classification

Two classification exist:

  • DeBakey.
  • Stanford.

Stanford dissection classification:[12]

  • Type A - aortic root to the left subclavian artery.
    • Considered a surgical emergency.
  • Type B - distal to (left) subclavian artery.
    • Generally, treated conservatively.

Gross

  • Entry intimal tear +/- exit intimal tear.
    • Blood between layers of the vessel wall.

Images

www:

Microscopic

Features:

  • Defect in the tunica media (muscle layer).
  • "Abundant" fresh blood in the adventitia.
  • +/-Changes of cystic medial degeneration - a specific histopathologic cause of aortic dissection.

DDx:

Images

www:

Cystic medial degeneration

General

  • Nonspecific finding - may be seen in a number of conditions.

Note about cystic medial necrosis:

  • Often not cystic and not necrotic.

Microscopic

Features:[14][15]

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

Images

www:

Stains

Medial calcific sclerosis

  • AKA Moenckeberg medial calcific sclerosis, calcific medial sclerosis of Monckeberg, and Monckeberg's arteriosclerosis.

General

  • Usually of no clinical consequence.

Microscopic

Features:[16]

  • Medial calcification (purple irregular stuff on H&E -- calcium phosphate).

Note:

  • Lumen unaffected.

Images

www:

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RIGHT LEG, BELOW KNEE AMPUTATION:
- MINIMAL-TO-MILD LARGE VESSEL ATHEROSCLEROSIS, SEE COMMENT.
- MEDIAL CALCIFIC SCLEROSIS.
- SKIN WITH DERMAL FIBROSIS.

COMMENT:
The sections may not be representative of disease in the distal vascular bed.

Hyperplastic arteriolosclerosis

General

Note:

  • Hyperplasia = proliferation of cells.

Microscopic

Features:[16]

  • Onion-skin appearance of intima & media due to:
    • Intimal hyperplasia.
    • Smooth muscle hyperplasia.

Image: Hyperplastic arteriolosclerosis (utah.edu).

Fibromuscular dysplasia

  • Abbreviated FMD.

General

Etiology:

  • Unknown, possibly genetic.

Gender:

  • Women > men.
  • May be seen in virtually any artery.
  • Reported as a cause of sudden death with involvement of the artery supplying the AV node.[18]

Gross/radiologic

  • Segmental - thinning and thickening.[19]

Classical locations:[19]

Microscopic

Features:[19]

  • Smooth muscle hyperplasia - key feature.
  • Elastic fibre fragmentation.
  • Luminal narrowing.

Images:

Stains

Thromboangiitis obliterans

Thrombosis

See also: Cerebral venous thrombosis.

General

Definition:

  • Blood clot formation within a vessel.

Complications:

Risk factors:

  • The classic pimping question is what "Virchow's triad?"
    • Stasis, hypercoagulability, endothelial injury.
  • A long list is found in: risk factors for VTE.

Gross

See: Pulmonary embolism - gross features.

Microscopic

Features:

  • Lines of Zahn.
  • Fibrin - pink acellular stuff on a H&E stain.

Image

Cholesterol embolism

  • Abbreviated CE.

Coarctation of the aorta

  • AKA aortic coarctation.

General

  • Uncommon.

Classification:

  • Preductal.
  • Postductal.

Associations:

Clinical

Presentation:[22]

  • Heart failure.
  • Hypertension - esp. upper extremity vs. lower extremity.

Gross

  • Narrowing (stenosis) of the aorta proximal or distal to the ductus arteriosis.

Image

Intracranial berry aneurysm

See also

References

  1. URL: http://www.lab.anhb.uwa.edu.au/mb140/corepages/vascular/vascular.htm. Accessed on: 13 January 2011.
  2. Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 4. ISBN 978-1416002741.
  3. Braverman, AC. (Oct 2011). "Aortic dissection: prompt diagnosis and emergency treatment are critical.". Cleve Clin J Med 78 (10): 685-96. doi:10.3949/ccjm.78a.11053. PMID 21968475.
  4. 4.0 4.1 4.2 LeMaire, SA.; Russell, L. (Feb 2011). "Epidemiology of thoracic aortic dissection.". Nat Rev Cardiol 8 (2): 103-13. doi:10.1038/nrcardio.2010.187. PMID 21173794.
  5. Leontyev, S.; Borger, MA.; Legare, JF.; Merk, D.; Hahn, J.; Seeburger, J.; Lehmann, S.; Mohr, FW. (Mar 2012). "Iatrogenic type A aortic dissection during cardiac procedures: early and late outcome in 48 patients.". Eur J Cardiothorac Surg 41 (3): 641-6. doi:10.1093/ejcts/ezr070. PMID 22345184.
  6. Teman NR, Peterson MD, Russo MJ, et al. (September 2013). "Outcomes of patients presenting with acute type A aortic dissection in the setting of prior cardiac surgery: an analysis from the International Registry of Acute Aortic Dissection". Circulation 128 (11 Suppl 1): S180–5. doi:10.1161/CIRCULATIONAHA.112.000342. PMID 24030404.
  7. URL: http://path.upmc.edu/cases/case84/dx.html. Accessed on: 13 May 2014.
  8. 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.
  9. 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.
  10. Cruz, RP.; Marrone, LC.; Marrone, AC. (Nov 2010). "Chronic syphilitic aortic aneurysm complicated with chronic aortic dissection.". Am J Surg 200 (5): e64-6. doi:10.1016/j.amjsurg.2010.02.017. PMID 20864080.
  11. Carlson, M.; Silberbach, M. (2009). "Dissection of the aorta in Turner syndrome: two cases and review of 85 cases in the literature.". BMJ Case Rep 2009: bcr0620091998. doi:10.1136/bcr.06.2009.1998. PMID 21731587.
  12. Finkelmeier BA (September 1997). "Dissection of the aorta: a clinical update". J Vasc Nurs 15 (3): 88-93. PMID 9362838.
  13. URL: http://emedicine.medscape.com/article/756835-overview. Accessed on: 12 August 2010.
  14. URL: http://emedicine.medscape.com/article/756835-overview. Accessed on: 12 August 2010.
  15. 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.
  16. 16.0 16.1 Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 7. ISBN 978-1416002741.
  17. URL: http://library.med.utah.edu/WebPath/IMMHTML/IMM028.html. Accessed on: 11 May 2011.
  18. 18.0 18.1 Lee, S.; Chae, J.; Cho, Y. (Dec 2006). "Causes of sudden death related to sexual activity: results of a medicolegal postmortem study from 2001 to 2005.". J Korean Med Sci 21 (6): 995-9. PMID 17179675.
  19. 19.0 19.1 19.2 Hata, D. (Sep 2001). "Fibromuscular dysplasia.". Intern Med 40 (9): 978-9. PMID 11579971.
  20. Braverman, AC.; Güven, H.; Beardslee, MA.; Makan, M.; Kates, AM.; Moon, MR. (Sep 2005). "The bicuspid aortic valve.". Curr Probl Cardiol 30 (9): 470-522. doi:10.1016/j.cpcardiol.2005.06.002. PMID 16129122.
  21. Hjerrild, BE.; Mortensen, KH.; Sørensen, KE.; Pedersen, EM.; Andersen, NH.; Lundorf, E.; Hansen, KW.; Hørlyck, A. et al. (2010). "Thoracic aortopathy in Turner syndrome and the influence of bicuspid aortic valves and blood pressure: a CMR study.". J Cardiovasc Magn Reson 12: 12. doi:10.1186/1532-429X-12-12. PMID 20222980.
  22. Peres, A.; Martins, JD.; Paramés, F.; Gil, R.; Matias, C.; Franco, J.; Freitas, I.; Trigo, C. et al. (Jan 2010). "Isolated aortic coarctation: experience in 100 consecutive patients.". Rev Port Cardiol 29 (1): 23-35. PMID 20391897.