Aneurysm

Question 1

The two major segments of the aorta

Answer 1

Proximal aorta
Distal aorta

Question 2

The proximal aortic segment includes

Answer 2

Ascending aorta and transverse aortic arch

Question 3

The distal aortic segments includes

Answer 3

Descending thoracic aorta and abdominal aorta

Question 4

defined as a permanent, localized dilatation of the aorta to a diameter that is at least 50% greater than is normal at that anatomic level

Answer 4

Aneurysm

Question 5

If the entire aorta is aneurysmal, this is condition is termed as

Answer 5

Mega-aorta

Question 6

Criado landing zone _:
ascending aorta and the origin of the innominate artery

Answer 6

0

Question 7

Criado landing zone _: left common carotid artery

Answer 7

1

Question 8

Criado landing zone _: left subclavian artery origin

Answer 8

2

Question 9

Criado landing zone _: a short section of the aorta that comprises the 2 cm immediately distal to the origin of the left subclavian artery

Answer 9

3

Question 10

Criado landing zone _: begins where zone 3 ends

Answer 10

4

Question 11

True aneurysms can take two forms: fusiform and saccular. Which form is more common and is described as symmetrical dilatations of the aorta

Answer 11

Fusiform

Question 12

Form of true aneurysm which are localized outpouchings of the aorta

Answer 12

Saccular aneurysms

Question 13

Also called pseudoaneurysms. These are leaks in the aortic wall that are contained by the outer layer of the aorta and/or the periaortic tissue

Answer 13

False aneurysm

caused by disruption of the aortic wall and lead blood to collect in pouches of fibrotic tissue

Question 14

The normal aorta derives its elasticity and tensile strength from which layer

Answer 14

Media

Question 15

Elastin content is highest within the?

Answer 15

Ascending aorta

Due to its compliant nature

Question 16

This is the most common cause of thoracic aortic disease.

Histologic findings of mild medial degeneration, including fragmentation of elastic fibers and loss of smooth muscle cells, are expected in the aging aorta

Answer 16

Nonspecific Medial Degeneration

Question 17

This usually begins as a tear in the inner aortic wall which initiates a progressive separation of the medial layers and creates two channels within the aorta

Answer 17

Aortic dissection

Weakens the outer wall

Represents a major, distinct cause of thoracic aortic aneurysms

Question 18

An autosomal dominant genetic disorder characterized by a specific connectivetissue defect that leads to aneurysm formation.

Phenotype includes: a tall stature, high palate, joint hypermobility, eye lens disorders, mitral valve prolapse, and aortic aneurysms

Answer 18

Marfan Syndrome

Question 19

Gene mutation involved in Marfan syndrome

Answer 19

Fibrillin gene located on the long arm of chromosome 15

abnormal fibrillin causes degeneration of the aortic wall matrix by increasing the activity of transforming growth factor beta (TGF-β)

Question 20

The MCC of death among patients with Marfan syndrome

Answer 20

Aortic dissection and aortic complications

Question 21

It is characterized as an aneurysmal syndrome with widespread systemic involvement that is aggressive; an autosomal dominant condition distinguished by triad of arterial tortuosity and aneurysms, hypertelorism (widely spaced eyes), and bifid uvula or cleft palate

Answer 21

Loeys-Dietz Syndrome

Question 22

It is caused by heterozygous mutations in the genes encoding TGF-β receptors

Answer 22

Loeys-Dietz Syndrome

Question 23

This syndrome includes a spectrum of inherited disorders of collagen synthesis

Answer 23

Ehlers-Danlos Syndrome

Question 24

This is characterized by an autosomal dominant defect in type III collagen synthesis, which can have life-threatening cardiovascular manifestations.

Answer 24

Vascular type Ehlers-Danlos Syndrome

Question 25

The MCC of death among patients with Ehlers-Danlos syndrome

Answer 25

Spontaneous arterial rupture (mesentericc vessels)

Question 26

Associated with joint and skin hyperextensibility and aortic dilation; caused by mutations in the gene encoding **filamin A** (FLNA)

Answer 26

Ehlers-Danlos variant of periventricular heterotopia

Question 27

an actin-binding protein that links the smooth muscle cell contractile unit to the cell surface

Answer 27

Filamin A

Question 28

The involved mutations are characterized by autosomal dominant inheritance with decreased penetrance and variable expression.

Answer 28

Familial Thoracic Aortic Aneurysm and Dissection

Question 29

mutations present in approximately 14% of families with familial thoracic aortic aneurysms and dissections.

Answer 29

ACTA2 mutations

Question 30

an autosomal dominant disorder characterized by aortic and arterial aneurysms, arterial tortuosity, aortic dissection, mild craniofacial abnormalities, and early-onset osteoarthritis.

Answer 30

Aneurysm-OA syndrome

Question 31

Aneurysms-osteoarthritis syndrome is caused by mutations in the gene encoding _.

Answer 31

SMAD3 ## FOOTNOTE This is a transcription factor for TGF-β

Question 32

The most common congenital malformation of the heart or great vessels

Answer 32

Congenital Bicuspid Aortic Valve

Question 33

Dilatation of COngenital BIcuspid Aortic Valve is occassionaly found in the

Answer 33

Arch

Question 34

a common origin of the innominate and left common carotid arteries

Answer 34

Bovine Aortic Arch

Question 35

Mean aortic growth rate considered as precursor of aortic aneurysm

Answer 35

0.29 cm/year

Question 36

Most common causative agents of thoracic aortic aneurysm

Answer 36

Staphylococcus aureus, Staphylococcus epidermidis, Salmonella and Sreptococcus

Question 37

Infection often produces (fusiform, saccular) aneurysms

Answer 37

Saccular ## FOOTNOTE located in areas of aortic tissue destroyed by the infectious process

Question 38

causes an obliterative endarteritis of the vasa vasorum that results in medial ischemia and loss of the elastic and muscular elements of the aortic wall

Answer 38

Treponema pallidum ## FOOTNOTE The ascending aorta and arch are the most commonly involved areas

Question 39

The dense aortic infiltrate responsible for the fibrosis consists of

Answer 39

Lymphocytes, plasma cells and giant cells

Question 40

generally produces obstructive lesions related to severe intimal thickening, but associated medial necrosis can lead to aneurysm formation

Answer 40

Aortic Takayasu arteritis

Question 41

granulomatous inflammation may develop that involves the entire thickness of the aortic wall, causing intimal thickening and medial destruction.

Answer 41

Giant cell arteritis (Temporal arteritis)

Question 42

an uncommon systemic disease that is associated with rheumatoid arthritis and ankylosing spondylitis. The resulting medial inflammation and fibrosis can affect the aortic root, causing annular dilatation, aortic valve regurgitation, and ascending aortic aneurysm formation

Answer 42

Rheumatoid aortitis

Question 43

usually represent chronic leaks that are contained by surrounding tissue and fibrosis

Answer 43

Pseudoaneurysms ## FOOTNOTE the wall develops from organized thrombus and associated fibrosis