Vascular

Question 1

Connective tissue causes of thoracic aortic aneurysm and associated mutations

Answer 1

Marfan syndrome (FBN- 1 Mutations)
Elhers danklos type IV (Col3A1 Mutations)
Loeys-Dietz syndrome (TGF beta receptor 1 and 2)
Turner (X0 syndrome)
Bicuspid aortic valve dz (no single mutation)
Familial thoracic aortic aneurysms (several mutations)

Question 2

Inflammatory causes of thoracic aortic aneurysm

Answer 2

Giant cell arteritis 
Takayasu arteritis 
Psoriatic aortitis 
Inflammatory bowel disease 
Behcet's disease 
Reiters syndrome 
ankylosing spondylitis (HLA-B27)
Sarcoidosis

Question 3

Infections to come to mind causing aortic aneurysm

Answer 3

syphilis (treponema pallidum)

Mycobacterium tuberculosis

Question 4

mechanism behind aneurysm formation

Answer 4

Medial degeneration which involves a process of;
loss of smooth muscle cells
elastic fiber fragmentation
Deposition of proteoglycans

Question 5

Indication for repair of asymptomatic TAA in genetic syndrome

Answer 5

4 - 5cm depending on specific condition and family history

Question 6

Indication for repair of asymptomatic TAA in pt w/ BAV

Answer 6

> 4.5cm at time of operation of severe valve disease
5cm if additional risk factor for dissection present.
5.5cm

Question 7

what is the Bental procedure

Answer 7

Bental procedure - involves replacement of the aortic valve and root

Question 8

complications of AAA

Answer 8

1. compression of adjacent structures
   - Abd pain, chronic flank or back pain, uretral obstruction
2. Acute or subacute symptoms caused by change in aneurysm size with inflammation and thrombosis
   - - Localized dissection or intramural hematoma
   - - aorta-enteric fistula with GI bleeding
   - - contained retro peritoneal rupture.
   - - distal air embolism
3. Peritoneal free rupture
   - hypotension, lactic acidosis, DIC, shock, death.