Aortopathy Flashcards
Ectasia
Aneurysm
Dilation Less than 150% of normal
Greater than 150% of normal
Aortic segments
Prox - ascending and tvs arch
Distal - descending, suprarenal, infrarenal
Aortic dissection
Pseudoaneurysm
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
Aneurysm vs. dissection
Aneurysm - dilation with no tear
Dissection - tear creating true and false lume n
TAA risk factors
HTN, SMoking, genetics with medial degereation
Genetic causes of Marfan, EdS, LDS, Turner
Fib 1 Type 3 collagen TGFbR1 TGFbR2 45,X0 karyotype
TGF-beta path and aoritc aneurysm
In a fibrillin def mouse model, enhanced TGFbeta signlaing was ID’d
LDS
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
LDS tx
Surgical repair at 4.2 cm by TEE or 4.4-4.6 cm by CT or MR
Turner syndrome
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
FTAAD recommendaiton
Refer to geneticist to screen 1st degree relatives
BAV
Notch1 gene mutation auto dom
Higher gradient and more severe aotic regurg
Rapid progression
Males>females
Screening in genetically based TAA
Screen all known 1st degree relatives of pts
TAA pathogeneiss
MEdial degen from
Disruptiin and loss of elastic fibers
Loss of smooth muslce in aortic media
Inc deposition of proteoglycans
TAA clinical
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
Signs of compression
Hoarseness due to left recurent laryngeal stretch
Stridor due to tracheal/bronchial compression
Dyspena - lung compresion
Plethora/edema due to SVC ocmp
TAA dx
Order TEE
Once TAA Id’d…use CT or MRI
Surgery for TAA
Indicated if sx and acute eventOR
Asx but Root or ascending >5.5, arch aneurysm over 6…rapidly growing
Hx have been open procedures
TEVAR
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
TEVER vs. open
Reduced all-cause mortality
Perioperative mortality reduce in pts with intact and rupture thoracic aneurysm
Medical mg of TAA
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
Imaging surveillance
Every 6 mos if aneurysm over 4…every 12 if under 4
Following TEVER - 1 mo, 6 mo , 12m o and annually CT
Types of AAAA
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
AAA risk factors
NOT DIABETES…actually dec
AAA complications
Rupture
Fistulizaion - aortocaval - high output HF…aortoenteric - sudden GI blled
Mural thrombus
AAA path
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
AAA clinicam
Vague and chronic ab pain
Low back pain
Mid-ab or flank pain may radiate to back
Hematuria
GI hemorrhage
AAA dx
US - segmental thickenes over 3 cm or 1.5 times expected
CT to determien surgial repair method
AAA surgical tx
Emergency done by open procedure…if sx
Elective - open or EVAR…over 1 cm/yr expansion or infrarenal/juxtarenal AAA>5.5
Open vs. EVAR
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)
Surveillance in AAA
Monitor endoleak
Confirm graft position
Document shrinkage
CT if over 4 cm every 6-12 mos…US every 2-3 years if under 4
AAA med tx
Statins
BP control
SMoking cessation
AAA screening
65-75…should get 1 time screen if ever smoked
Aoritc dissection
Can occur without aneurysm
Aortic ulcer may be precuror to intramural hemoatoma
90% localized to descending
COmps of dissection
Intramural hematoma
Penetrating aortic ulcer
Pericardial effuson
Ext into branch vessels
End-organ injury
Aortic dissection path
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
Aortic dissection clinical
Chest paid sharp and ripping
Pulse deficit or BP diff
Syncope
Renal failure
MI
Pleural effusion
AOrtic dissection dx
ECG in ALL to sule out MI
TTE as initial imaging modality
Dx confirmed byID of flap…CT is specific
Aortic disseciton surgical
A - urgent
B - TEVAR
Aortic dissection medical managmeent
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
Aoritc diss imaging surv
Acute - 1mo, 6mo, then annually
Chronic - 1 yr then every 2-3 yr
Acute hematoma or ulcer - 1,3,6 mos and then anual
