Aortic Dissection/Aortopathy Flashcards
Histology of normal aorta
Fibrocellular intima with endothelial lining
Media - elastic, collagen/ECM proteoglycans and smooth muscle cells
Connective tissue adentitia with vasa vasorum and stem cells
Thoacic vs. abdominal aorta
Thoracic - 56 lamellar units
Abdominal - 28
Abdominal has no vasa vasorum…uses trnasmedial diffusion
Outer 1/2 of throacic noursiehd by vasa vasorum…inner uses diffusion
Cyctic medial degeneration of the media
Fragmented elastic fibers
Lamellar unit of aortic media
SMA and elastin rich lamellae with interconnections
SMC has TGFBeta 1 and 2
Myosin proteins
SMAD pathways
EL hve large round fenestrations and associated thick collagen 3 fibers
Right SMC connect to lower EL via fibrillin and collagen type 4
Next basal lamina layer, larger deposits contain type 4 collagen and heaprin sulfate proteoglycan
Marfan
E-D
Loeys-Dietz
Genetics
Fibrillin 1
Type 3 colagen
TGFbR1 and TGFbR2
Parts of lamella affected in LDS, EDS, MFS
LDS - TGFbeta 1,2
EDS - collagen type 3
MFS - fibrillin
Marfan synrome
Defect in fibrillin 1
ECM microfibrils defective
Elastic tissues of vessels, joints, and lens
Long bone growth
Thoracic aortic dilation
Aortic aneurysm and dissection
AVI
MV prolaspe
EDS
Type 3 collagne abnoramilies
Overlaps with LDS type 2 (TGF beta receptor)
Fragile blood vessels prone to rupture (aorta)
Thin, translucent skin that bruises easily
Collapsed lung (pneumothorax)
MItral valve prolapse and BAV
Aortic dissection Def Presentation Signs Path
Intraluminal tear forming a false lumen
Tearing of chest pain at sudden onset radiating to back
Unqeual BP in arms
False lumen can be limited to ascending aorta and propogate to descending or vice versa
Pericardial tamponda, aortic rupte and death
Dissection most oftne deu to
Disease of aortic medial layer
AKA aortopathy
BAV
Most common abnormality of the heart
Predilection to develop endocarditis
Dissecting aneuryms of aorta due to intrinsic medial aortopathy
Arterial FMD
Stenosis of renal artery (HTN)
Female over 25
Sensitive to smoking cessation
Medial artery abnormality
Takayasu aortitis
Aortic arch syndrome, pulseless dz with occlussive thromboartopathy…oung female panarteritis
More in Asian/African famela
20-25 y/o
Buerger’s dz
Thromboangiitis obliterans
Non AS inflam vaso-occlusion of mediaum size arts and veins
Distal extemities of young male cigarreteee smokes
Regress with smoking
May cause cluadication or gangrene
Buergers histology
Acute - medial microabscess and thrombosis - giant cells in a thrombus***
Chronic - obliteration from cellular organizing thrombosis of arteries and veins
