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Flashcards in Aortopathy Deck (41)
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1
Q

Ectasia

Aneurysm

A

Dilation Less than 150% of normal

Greater than 150% of normal

2
Q

Aortic segments

A

Prox - ascending and tvs arch

Distal - descending, suprarenal, infrarenal

3
Q

Aortic dissection

Pseudoaneurysm

A

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

4
Q

Aneurysm vs. dissection

A

Aneurysm - dilation with no tear

Dissection - tear creating true and false lume n

5
Q

TAA risk factors

A

HTN, SMoking, genetics with medial degereation

6
Q

Genetic causes of Marfan, EdS, LDS, Turner

A
Fib 1 
Type 3 collagen
TGFbR1
TGFbR2
45,X0 karyotype
7
Q

TGF-beta path and aoritc aneurysm

A

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

8
Q

LDS

A

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

9
Q

LDS tx

A

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

10
Q

Turner syndrome

A

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

11
Q

FTAAD recommendaiton

A

Refer to geneticist to screen 1st degree relatives

12
Q

BAV

A

Notch1 gene mutation auto dom

Higher gradient and more severe aotic regurg

Rapid progression

Males>females

13
Q

Screening in genetically based TAA

A

Screen all known 1st degree relatives of pts

14
Q

TAA pathogeneiss

A

MEdial degen from

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

15
Q

TAA clinical

A

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

16
Q

Signs of compression

A

Hoarseness due to left recurent laryngeal stretch

Stridor due to tracheal/bronchial compression

Dyspena - lung compresion
Plethora/edema due to SVC ocmp

17
Q

TAA dx

A

Order TEE

Once TAA Id’d…use CT or MRI

18
Q

Surgery for TAA

A

Indicated if sx and acute eventOR

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

Hx have been open procedures

19
Q

TEVAR

A

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

20
Q

TEVER vs. open

A

Reduced all-cause mortality

Perioperative mortality reduce in pts with intact and rupture thoracic aneurysm

21
Q

Medical mg of TAA

A

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

22
Q

Imaging surveillance

A

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

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

23
Q

Types of AAAA

A

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

24
Q

AAA risk factors

A

NOT DIABETES…actually dec

25
Q

AAA complications

A

Rupture

Fistulizaion - aortocaval - high output HF…aortoenteric - sudden GI blled

Mural thrombus

26
Q

AAA path

A

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

27
Q

AAA clinicam

A

Vague and chronic ab pain

Low back pain

Mid-ab or flank pain may radiate to back

Hematuria

GI hemorrhage

28
Q

AAA dx

A

US - segmental thickenes over 3 cm or 1.5 times expected

CT to determien surgial repair method

29
Q

AAA surgical tx

A

Emergency done by open procedure…if sx

Elective - open or EVAR…over 1 cm/yr expansion or infrarenal/juxtarenal AAA>5.5

30
Q

Open vs. EVAR

A

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)

31
Q

Surveillance in AAA

A

Monitor endoleak
Confirm graft position
Document shrinkage

CT if over 4 cm every 6-12 mos…US every 2-3 years if under 4

32
Q

AAA med tx

A

Statins
BP control
SMoking cessation

33
Q

AAA screening

A

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

34
Q

Aoritc dissection

A

Can occur without aneurysm

Aortic ulcer may be precuror to intramural hemoatoma

90% localized to descending

35
Q

COmps of dissection

A

Intramural hematoma

Penetrating aortic ulcer

Pericardial effuson

Ext into branch vessels

End-organ injury

36
Q

Aortic dissection path

A

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

37
Q

Aortic dissection clinical

A

Chest paid sharp and ripping

Pulse deficit or BP diff

Syncope
Renal failure
MI
Pleural effusion

38
Q

AOrtic dissection dx

A

ECG in ALL to sule out MI

TTE as initial imaging modality

Dx confirmed byID of flap…CT is specific

39
Q

Aortic disseciton surgical

A

A - urgent

B - TEVAR

40
Q

Aortic dissection medical managmeent

A

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

41
Q

Aoritc diss imaging surv

A

Acute - 1mo, 6mo, then annually

Chronic - 1 yr then every 2-3 yr

Acute hematoma or ulcer - 1,3,6 mos and then anual