Aortopathy

Question 1

Ectasia

Aneurysm

Answer 1

Dilation Less than 150% of normal

Greater than 150% of normal

Question 2

Aortic segments

Answer 2

Prox - ascending and tvs arch

Distal - descending, suprarenal, infrarenal

Question 3

Aortic dissection

Pseudoaneurysm

Answer 3

Dissection - disruption of media with bleeding iwthin wall of aorta

Dilation of aorta due to disruption of wall wall layers with extravasation of blood contained by periartial tissue - blunt trauama or rapid decelraiton…not contained by arterial wall

Question 4

Aneurysm vs. dissection

Answer 4

Aneurysm - dilation with no tear

Dissection - tear creating true and false lume n

Question 5

TAA risk factors

Answer 5

HTN, SMoking, genetics with medial degereation

Question 6

Genetic causes of Marfan, EdS, LDS, Turner

Answer 6

Fib 1 
Type 3 collagen
TGFbR1
TGFbR2
45,X0 karyotype

Question 7

TGF-beta path and aoritc aneurysm

Answer 7

In a fibrillin def mouse model, enhanced TGFbeta signlaing was ID’d

Question 8

LDS

Answer 8

Mutations of TGFb1 and 2 receptors

LD type 1 - traid of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula/cleft palata

Type 2 - minimal craniofacial, lucent skin and poor wound healing

Question 9

LDS tx

Answer 9

Surgical repair at 4.2 cm by TEE or 4.4-4.6 cm by CT or MR

Question 10

Turner syndrome

Answer 10

Coarctation in 12%
Elongation of tcs arch, BAV or aoritc root dilation in 33%

Sceen all for BAC, CoA and aoritc root dilatation and repeat every 5-10 yrs

Question 11

FTAAD recommendaiton

Answer 11

Refer to geneticist to screen 1st degree relatives

Question 12

BAV

Answer 12

Notch1 gene mutation auto dom

Higher gradient and more severe aotic regurg

Rapid progression

Males>females

Question 13

Screening in genetically based TAA

Answer 13

Screen all known 1st degree relatives of pts

Question 14

TAA pathogeneiss

Answer 14

MEdial degen from

Disruptiin and loss of elastic fibers
Loss of smooth muslce in aortic media
Inc deposition of proteoglycans

Question 15

TAA clinical

Answer 15

Most asx

Signs of compression, chest pain, aoritc valve murmu

Large may have cough

Pain - neck and jaw pajin (arch), back and intrascap/left hsoulder pain (descneidng TAA0

Question 16

Signs of compression

Answer 16

Hoarseness due to left recurent laryngeal stretch

Stridor due to tracheal/bronchial compression

Dyspena - lung compresion
Plethora/edema due to SVC ocmp

Question 17

TAA dx

Answer 17

Order TEE

Once TAA Id’d…use CT or MRI

Question 18

Surgery for TAA

Answer 18

Indicated if sx and acute eventOR

Asx but Root or ascending >5.5, arch aneurysm over 6…rapidly growing

Hx have been open procedures

Question 19

TEVAR

Answer 19

Recommended in pts with descending aneurysm and
- degen or trauamtic aneurysm over 5.5, saccular aneurysm, post op pesuoaneuryms, or stridoer

NOT recommended ofr pts with conn tissue dz

Question 20

TEVER vs. open

Answer 20

Reduced all-cause mortality

Perioperative mortality reduce in pts with intact and rupture thoracic aneurysm

Question 21

Medical mg of TAA

Answer 21

Atherosceloritc dz risk reduction with statin

BP control - 140/90 without diabetes…130/80 if diabetes, chronic renal dz or chronic dissection

Marfan - beta blocker and/or ARB

Question 22

Imaging surveillance

Answer 22

Every 6 mos if aneurysm over 4…every 12 if under 4

Following TEVER - 1 mo, 6 mo , 12m o and annually CT

Question 23

Types of AAAA

Answer 23

Atheroscleoritc - excess MMP

Congenital - MArfan, EDS, BAV

inflam - form of atheroscleortic…wall thicked with dense, shiny, white fibrosis and adherence to surrounding tissue

Infection - stpha or salmonella

Question 24

AAA risk factors

Answer 24

NOT DIABETES…actually dec

Question 25

AAA complications

Answer 25

Rupture Fistulizaion - aortocaval - high output HF...aortoenteric - sudden GI blled Mural thrombus

Question 26

AAA path

Answer 26

Aoritc wall loses elasticity through disruption of elastin fibers and deg of collagen Lymphocytes and macros infiltrate vessel wall Proteases destory elastin and collagen Mooth muscle cells lost and media thins NEovascularization occurs

Question 27

AAA clinicam

Answer 27

Vague and chronic ab pain Low back pain Mid-ab or flank pain may radiate to back Hematuria GI hemorrhage

Question 28

AAA dx

Answer 28

US - segmental thickenes over 3 cm or 1.5 times expected CT to determien surgial repair method

Question 29

AAA surgical tx

Answer 29

Emergency done by open procedure...if sx Elective - open or EVAR...over 1 cm/yr expansion or infrarenal/juxtarenal AAA>5.5

Question 30

Open vs. EVAR

Answer 30

Open if asx with large or cannot comply iwth LT surveillance EVAR - lower perioperative mortality but similar others Inc rupture rate and need for reintervention (may be better for older)

Question 31

Surveillance in AAA

Answer 31

Monitor endoleak Confirm graft position Document shrinkage CT if over 4 cm every 6-12 mos...US every 2-3 years if under 4

Question 32

AAA med tx

Answer 32

Statins BP control SMoking cessation

Question 33

AAA screening

Answer 33

65-75...should get 1 time screen if ever smoked

Question 34

Aoritc dissection

Answer 34

Can occur without aneurysm Aortic ulcer may be precuror to intramural hemoatoma 90% localized to descending

Question 35

COmps of dissection

Answer 35

Intramural hematoma Penetrating aortic ulcer Pericardial effuson Ext into branch vessels End-organ injury

Question 36

Aortic dissection path

Answer 36

Thikcening and fibrosis of intimal layer and degradation and apoptosis of medial smooth muscle cells Elastic ocmp of wall become ncrotic and fibrotic Wall becomes still and weaknened

Question 37

Aortic dissection clinical

Answer 37

Chest paid sharp and ripping Pulse deficit or BP diff Syncope Renal failure MI Pleural effusion

Question 38

AOrtic dissection dx

Answer 38

ECG in ALL to sule out MI TTE as initial imaging modality Dx confirmed byID of flap...CT is specific

Question 39

Aortic disseciton surgical

Answer 39

A - urgent | B - TEVAR

Question 40

Aortic dissection medical managmeent

Answer 40

Preferred with acute arch or B type dissections as long as no malperfusion, aoritc rupture or subactue aortic leaking Control BP and HR...give IV B-blocker to get HR down...then give ACEI and vasodilators Chonric - ASCVD risk reduciton,...BP control

Question 41

Aoritc diss imaging surv

Answer 41

Acute - 1mo, 6mo, then annually Chronic - 1 yr then every 2-3 yr Acute hematoma or ulcer - 1,3,6 mos and then anual