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