 Reference ranges

 End and review

Question 3 of 298

Which of the following changes are most likely to be identified in the aortic wall of a 38 year old lady with a Marfans syndrome and a
dissecting aortic aneurysm?

Transmural aortitis

Cystic medial necrosis

Foamy macrophages

Dense dystrophic calcification

None of the above

Cystic medial necrosis ( or cystic medial degeneration) occurs when basophils and mucoid material lie in between the intimal elastic
fibres of the aorta. It is typically found in the aortic degeneration of Marfans syndrome, but may also be seen in aortic degeneration
in older adults.
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Aortic dissection

 More common than rupture of the abdominal aorta

 33% of patients die within the first 24 hours, and 50% die within 48 hours if no treatment received
 Associated with hypertension
 Features of aortic dissection: tear in the intimal layer, followed by formation and propagation of a subintimal hematoma.
Cystic medial necrosis (Marfan's)
 Most common site of dissection: 90% occurring within 10 centimetres of the aortic valve

Stanford Classification

Type Location Treatment

A Ascending aorta/ aortic Surgery- aortic root replacement

root
Type Location Treatment

B Descending aorta Medical therapy with

antihypertensives

DeBakey classification

Type Site affected

I Ascending aorta, aortic arch, descending aorta

II Ascending aorta only

III Descending aorta distal to left subclavian artery

Clinical features

 Tearing, sudden onset chest pain (painless 10%)

 Hypertension or Hypotension
 A blood pressure difference (in each arm) greater than 20 mm Hg
 Neurologic deficits (20%)

Investigations

 CXR: widened mediastinum, abnormal aortic knob, ring sign, deviation of the trachea/oesophagus
 CT angiography of the thoracic aorta
 MRI angiography
 Conventional angiography (now rarely used diagnostically)

Management

 Beta-blockers: aim HR 60-80 bpm and systolic BP 100-120 mm Hg

 For type A dissections the standard of care is aortic root replacement

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