Thoracic Aneurysms Flashcards Preview

Vascular Surgery > Thoracic Aneurysms > Flashcards

Flashcards in Thoracic Aneurysms Deck (15)
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1
Q

What are the structural elements of blood vessels?

A

type I collagen
type III collage
elastin, firbillin

type IV collagen, laminin
type V, VI vollagen
proteoglycans

2
Q
What is the mutation of
Marfans?
Ehlers-danlos IV?
Loeys-dietz?
Familial Thoracic Aortic Aneurysm?
A

FBN1
COL3A1
TGFBR1 TGFBR2
MYH11, TGFBR2

3
Q

What are the three systems involved in Marfans?

A

cardiovascular, ocular, skeletal

4
Q

What are clinical manifestations of Marfans?

A
skeletal
pectus
dispropotionate limbs
scoliosis
elevated palate

ocular
ectopia lentis

CV
aneurysm
MVP
DCM

5
Q

In what setting should you stent marfans?

what are the consequences?

A

only in acute rupture
25% will have new dissection
40% primary graft failure (mort 42%)

6
Q

What is the transmission pattern of EDS IV

A

Autosomal dominant

50% new mutations

7
Q

What are feature of EDS IV?

A

Joint hypermobility, skin hypermobility, tissue fragility affecting the skin, joints, rupture blood vessels, internal organs

8
Q

At what diameter do vessels rupture in EDS?

A

no particular diameter

can be normal

9
Q

What is the complication rate associated with EDS and angiography?

A

67%

12% mortalityvg

10
Q

What aneurysm pattern is unique to EDS?

A

iliac aneurysms that spare the bifurcation

11
Q

What are the features of LDS?

A

type I severe craniofacial defects and aortic aneurysm
type II less severe craniofacial and aortic aneurysm (usually only bifid uvula or high palate)
hypertelorism (wide set eyes)

12
Q

What are the leading causes of death in LDS?

A

thoracic dissection followed by abdominal dissection and cerebral haemorrhage

13
Q

What is the pathogenesis of the Marfans mutation?

A

fragmented elastin with microfibrils that are susceptible to enzymatic digestion

14
Q

What is the pathogenesis of the EDS mutation?

A

type III collagen formed incorrectly and never excreted from the cell.

15
Q

what is the pathogenesis of the LD mutation?

A

similar to marfans

lower elastin contents with elastin fragmented and in disarray.