Thoracic Aneurysms Flashcards Preview

Vascular Surgery > Thoracic Aneurysms > Flashcards

Flashcards in Thoracic Aneurysms Deck (15):
1

What are the structural elements of blood vessels?

type I collagen
type III collage
elastin, firbillin

type IV collagen, laminin
type V, VI vollagen
proteoglycans

2

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

FBN1
COL3A1
TGFBR1 TGFBR2
MYH11, TGFBR2

3

What are the three systems involved in Marfans?

cardiovascular, ocular, skeletal

4

What are clinical manifestations of Marfans?

skeletal
pectus
dispropotionate limbs
scoliosis
elevated palate

ocular
ectopia lentis

CV
aneurysm
MVP
DCM

5

In what setting should you stent marfans?
what are the consequences?

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

6

What is the transmission pattern of EDS IV

Autosomal dominant
50% new mutations

7

What are feature of EDS IV?

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

8

At what diameter do vessels rupture in EDS?

no particular diameter
can be normal

9

What is the complication rate associated with EDS and angiography?

67%
12% mortalityvg

10

What aneurysm pattern is unique to EDS?

iliac aneurysms that spare the bifurcation

11

What are the features of LDS?

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

What are the leading causes of death in LDS?

thoracic dissection followed by abdominal dissection and cerebral haemorrhage

13

What is the pathogenesis of the Marfans mutation?

fragmented elastin with microfibrils that are susceptible to enzymatic digestion

14

What is the pathogenesis of the EDS mutation?

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

15

what is the pathogenesis of the LD mutation?

similar to marfans
lower elastin contents with elastin fragmented and in disarray.