Vascular Flashcards
Thoracic Aorta
overview
The thoracic aorta is divided anatomically into four regions; the root, the ascending aorta,
the transverse aorta (arch), and the descending aorta. The “root” is defined as the portion of the aorta
extending from the aortic valve annulus to the sino-tubular junction. The diameter of the thoracic
aorta is largest at the aortic root and gradually decreases (average size is 3.6 cm at the root, 2.4 cm in
the distal descending).
Thoracic Aorta
sinuses of valsalva
There are 3 outpouchings (right,
left, posterior) above the annulus that terminate at the
ST Junction. The right and left coronaries come off the
right and left sinuses. The posterior cusp is sometimes
called the “non-coronary cusp.”
Thoracic Aorta
isthmus
The segment of the aorta between the origin of the left Subclavian and the ligamentum arteriosum.
Thoracic Aorta
ductus bump
Just distal to the isthmus is a contour
bulge along the lesser curvature, which is a normal structure (not a pseudoaneurysm).
Aortic Arch Variants
bovine arch
left common carotid coming off
the brachiocephalic proper
(10%)
Aortic Arch Variants
left CC off brachiocephalic
Bovine Arch
( 15%) - common origin of
brachiocephalic artery and left
common carotid artery
Aortic Arch Variants
separate origins
5% of people
the left vertebral artery
originates separately from the
arch.
Pulmonary Sling
Aberrant Left pulmonary artery coming off the
right pulmonary artery.
-Unique as the only anomaly to create indentations in the posterior
trachea and anterior esophagus.
-Unique as the only anomaly that can cause stridor in a patient with
a normal left sided arch.
Adamkiewicz
The thoracic aorta puts off multiple
important feeders including the great
anterior medullary artery (Artery of
Adamkiewicz) which serves as a dominate feeder of the spinal cord.
This thing usually comes off on the left side (70%) between T9-T12.
“Beware o f the Hairpin Turn”
-The classic angiographic appearance of the artery is the “hairpin turn” as its anastomosis with the anterior spinal artery.
Mesenteric Branches
overview
The anatomy of the SMA and IMA is high yield, and can be shown on a MIP coronal CT, or Angiogram. I think that knowing the inferior pancreaticoduodenal
comes off the SMA first, and that the left colic (from IMA) to the middle colic (from SMA) make up the Arc of Riolan are probably the highest yield facts.
Celiac Branches
The classic branches of the celiac axis are the common hepatic, left gastric, and the splenic arteries.
The “common” hepatic artery becomes the “proper” hepatic artery after the GDA.
This “traditional anatomy” is actually only seen in 55% of people.
V a r ia n t H e p a tic A r te ry A n a tom y
overview
The right hepatic artery and left hepatic arteries
may be “replaced” (originate from a vessel other than the proper hepatic) or duplicated -
which anatomist called “accessory.” This distinction o f “replaced” vs “accessory” would
make a great multiple choice question.
- Replaced = Different Origin, usually off the left gastric or SMA
- Accessory = Duplication o f the Vessel, with the spare coming o ff the left gastric or SMA
V a r ia n t H e p a tic A r te ry A n a tom y
thinkgs to know
• If you see a vessel in the fissure of the Iigamentum venosum (where there is not normally a vessel), it’s probably an accessory or replaced left hepatic artery arising from the left gastric artery. • The proper right hepatic artery is anterior to the right portal vein, whereas the replaced right hepatic artery is posterior to the main portal vein. This positioning o f the replaced right increases the risk o f injury in pancreatic surgeries.
Ilia c A na tom y
The branches o f the internal iliac are high yield, with the most likely
question being “which branches are from the posterior or anterior divisions?” A useful mnemonic is “I Love Sex, ” Illiolumbar, Lateral Sacral, Superior Gluteal, for the posterior
division.
My trick for remembering that the mnemonic is for posterior and not anterior is to think o f
that super religious girl I knew in college — 1 Like Sex in the butt / posterior.
ovarian artery trivia
The ovarian arteries arise from the anterior-medial aorta 80-90% o f time.
Anterior division of internal iliac artery mnemonic
courses posteromedially
I love going places in my very own underwear
iliolumbar -posterior lateral sacral - posterior gluteal (superior and inferior) -posterior pudendal (internal) inferior veiscal (vaginal in females) middle rectal artery vaginal artery obturator umbilical arter and uterine artery (females)
Divisions of the internal iliac superior to inferior
posterior
iliolumbar
lateral sacral
superior gluteal
anterior umbilical (superior vesical comes off) inferior vesicle obturator (runs through obturator foramen) uterine middle rectal internal pudendal inferior gluteal
P e rs is te n t S c ia tic A r te r y
An anatomic variant, which is a continuation o f the
internal iliac. It passes posterior to the femur in the thigh and then will anastomose with the
distal vasculature. Complications worth knowing include aneurysm formation and early
atherosclerosis in the vessel. The classic vascular surgery boards question is “external iliac is
acutely occluded, but there is still a strong pulse in the foot” , the answer is the patient has a
persistent sciatic.
Mesenteric Arterial Collateral Pathways
celiac to SMA
The conventional collateral pathway is Celiac -> Common Hepatic -> GDA -> Superior Pancreatic Duodenal -> Inferior Pancreatic Duodenal -> SMA.
Arc ofBuhler: This is a variant anatomy (seen in like 4% o f people), that represents a collateral pathway from the celiac to the SMA. The arch is independent o f the GDA and inferior pancreatic arteries. This rare collateral can have an even more rare aneurysm, which occurs in association with stenosis o f the celiac axis.
Mesenteric Arterial Collateral Pathways
IMA to llia c s
The conventional collateral pathway is IMA -> Superior Rectal -> Inferior Rectal -> Internal Pudendal -> Anterior branch o f internal iliac
Mesenteric Arterial Collateral Pathways
SMA to IMA
The conventional collateral pathway is SMA -> Middle Colic -> Left Branch o f the Middle Colic -> Arc o f Riolan (as below) -> Left Colic - > IMA.
Arc o f Riolan - Also referred to as the
meandering mesenteric artery. Classically a connection between the middle colic o f the SMA and the left colic o f the IMA.
Marginal Artery’ o f Drummond - This is another SMA to IMA connection. The
anastomosis o f the terminal branches o f the ileocolic, right colic and middle colic arteries o f the SMA, and o f the left colic and sigmoid branches o f the IMA, form a continuous arterial circle or arcade along the inner border o f the colon.
Mesenteric Arterial Collateral Pathways
winslow pathway
This is a collateral pathway that is seen in the setting o f aorto-iliac
occlusive disease. The pathway apparently can be inadvertently cut during transverse
abdominal surgery. The pathway runs from subclavian arteries -> internal thoracic (mammary)
arteries -> superior epigastric arteries -> inferior epigastric arteries -> external iliac arteries.
Mesenteric Arterial Collateral Pathways
corona mortis
Classically described as a vascular connection between the obturator
and external iliac. Some authors describe additional anastomotic pathways, but you should
basically think o f it as any vessel coursing over the superior pubic rim, regardless o f the
anastomotic connection. The “crown o f death” is significant because it can (a) be injured in
pelvic trauma or (b) be injured during surgery - and is notoriously difficult to ligate.
Some authors report that it causes 6-8% o f deaths in pelvic trauma. The last piece o f trivia is
that it could hypothetically cause a type 2 endoleak.
Upper Extremity Anatomy
overview
The scalene muscles make a triangle in the neck. If you have ever had the pleasure of reading a
brachial plexus MRI finding this anatomy in a sagittal plane is the best place to start (in my
opinion). The relationship to notice (because it’s testable) is that the subclavian vein runs anterior to
the triangle, and the subclavian artery runs in the triangle (with the brachial plexus).
Upper Extremity Anatomy
tribia
The subclavian artery runs posterior to the subclavian vein.
The subclavian artery has several major branches
the vertebral, the internal thoracic, the
thyrocervical trunk, the costocervical trunk, and the dorsal scapular
Subclavian name changes
- Axillary Artery: Begins at the first rib
- Brachial Artery: Begins at the lower border of the teres major (major NOT minor!)
- Brachial Artery: Bifurcates to the ulnar and radial
H o w T o T e ll T h e U ln a r F rom T h e R a d ia l A r t e r y On A n g io g r am O r CTA:
Around the radial head the brachial artery splits into the radial and ulnar arteries. I have three tricks for telling the radial from the ulnar artery apart (in case it’s not obvious).
- The ulnar artery is usually bigger.
- The ulnar artery usually gives o ff the common interosseous
- The ulnar artery supplies the superficial palmar arch (usually), and therefore the radial supplies the deep arch (usually).
Upper Extremity Normal Variants
- Anterior Interosseous Branch (Median Artery) persists and supplies the deep palmar arch o f the hand.
- “High Origin o f the Radial Artery” - Radial artery comes o ff either the axillary or high brachial artery (remember it normally comes off at the level of the radial head).
lower Extremity Anatomy
femoral
The nomenclature pearl for the external iliac is that it becomes the common femoral once it gives off the inferior epigastric (at the inguinal ligament). Once the inferior epigastric comes o ff (level o f the inguinal ligament) you are dealing with the common femoral artery(CFA). The CFA divides into the deep femoral (profunda) and
superficial femoral. The deep femoral courses lateral and posterior. The superficial femoral
passes anterior and medial into the flexor muscle compartment (ADDuctor / Hunter’s Canal).
At the point the vessel emerges from the canal it is then the popliteal artery
lower Extremity Anatomy
popliteal
At the level o f
the distal border o f the popliteus muscle the popliteal artery divides into the anterior tibialis
(the first branch) and the tibioperoneal trunk. The anterior tibialis courses anterior and
lateral, then it transverses the interosseous membrane, running down the front o f the anterior
tibia and terminating as the dorsalis pedis.The tibioperoneal trunk bifurcates into the
posterior tibialis and fibular (peroneal) arteries.
A common quiz is “what is the most medial
artery in the leg?”
with the answer being the posterior tibial (felt at the medial malleolus).
G a s tric V a ric e s
As described in more detail in the GI chapter, portal hypertension
shunts blood away from the liver and into the systemic venous system. Spontaneous portalsystemic
collaterals develop to decompress the system. The thing to know is that most
gastric varices are formed by the left gastric (coronary v e in ) . That is the one they
always show big and dilated on an angiogram. Isolated gastric varices are secondary to
splenic vein thrombosis. Gastric Varices (80-85%) drain into the inferior phrenic and then
into the left renal vein, forming a gastro-renal shunt.
Left Gastric
(Coronary) drains
cardia
Posterior and Short
Gastric
fundus
S p len o re n a l Shunt
Another feature o f portal hypertension, this is an abnormal
collateral between the splenic vein and renal vein. This is actually a desirable shunt because
it is not associated with GI bleeding. However, enlarged shunts are associated with
hepatic encephalopathy (discussed in greater detail in the BRTO section o f the IR chapter).
A common way to show this is an enlarged left renal vein and dilatation o f the inferior vena
cava at the level o f the left renal vein.
L e ft S id ed SVC
overview
- Most commonly associated CHD is the ASD
- Associated with an unroofed coronary sinus
- Nearly always (92% o f the time) it drains into the coronary sinus
L e ft S id ed SVC
trivia
- Most commonly associated CHD is the ASD
- Associated with an unroofed coronary sinus
- Nearly always (92% o f the time) it drains into the coronary sinus
D u p lic a te d SVC
“Left Sided SVC” almost never occurs in
isolation. Instead, there is almost always a “normal” right sided SVC, in addition to the left
sided SVC. It is in this case (which is the majority o f cases o f left sided SVC) that the
terminology “duplicated SVC” is used. It is so common that people will use the terms
duplicated and left sided interchangeably. But, technically to be duplicated you need a right
sided and left sided SVC (even if the right one is a little small - which is often the case in the
setting o f a left SVC).
D u p lic a te d IVC
There are two main points
worth knowing about this: (1) that the appearance
is an Aunt Minnie, and (2) it’s associated with
Renal stuff. Renal associations include
horseshoe and crossed fused ectopic kidneys.
Also these dudes often have circumaortic renal
collars
Duplicated IVC
aunt minnie
(IVCs are the bread, Aorta is the cheese or peanut butter…or bacon)
C irc um a o rtic Venou s C o lla r
Very common variant with an additional left renal
vein that passes posterior to the aorta. It only matters in two situations (a) renal transplant,
(b) IVC filter placement. The classic question is that the anterior limb is superior, and the
posterior limb is inferior.
C irc um a o rtic Venou s C o lla r
superior slice
renal vein is anterior to aorta
C irc um a o rtic Venou s C o lla r
inferior
Renal vein is posterior to aorta
Azygos C o n tin u a tio n
This is also known as absence o f the hepatic segment o f the
IVC. In this case, the hepatic veins drain directly into the right atrium. Often the IVC is
duplicated in these patients, with the left IVC terminating in the left renal vein , which then
crosses over to join the right IVC.
The first thing you should think when I say azygous continuation
is polysplenia (reversed IVC/Aorta is more commonly associated with asplenia).
Azygos Continuation
quick
No IVC in the Liver, Dila ted A z y g o s in the C h e s t
There are 3 “acute aortic syndromes”
aortic dissection,
intramural hematoma, and penetrating ulcer.
3 layers of aorta internal to external
intima
media
externa
Penetrating Ulcer
classification
3 AASs can be classified as type A or B Stanford, based on their locations before (type A) or after the takeoff the o f the left subclavian (type B).
1 risk factor
Penetrating Ulcer
Atherosclerosis
Delicious Burger King and Tasty Cigarettes
Penetrating Ulcer
classic scenario
Elderly patient with hypertension and
atherosclerosis usually involving the descending thoracic aorta
Penetrating Ulcer
genesis
Eating like a pig and smoking results in
atherosclerosis. Nasty atherosclerotic plaque erodes
through the intima. Hematoma forms in the media (intramural
hematoma). With severe disease can eventually
progress to a pseudo aneurysm (and maybe even rupture)
Penetrating Ulcer
pearl
3 AASs can be classified as type A or B Stanford, based on their locations before (type A) or after the takeoff the o f the left subclavian (type B).
Penetrating Ulcer
saccular morphology
These things often result in a saccular morphology around the arch. In general, sac like aneurysm above the diaphragm is related to penetrating ulcer. Sac like aneurysm below the diaphragm is gonna be septic (“mycotic”).
R e la tio n sh ip b e tw e e n P e n e tra tin g U lc e r and D is s e c tio n :
—Controversial (which usually means it won’t he tested) - famous last words
If forced to answer questions on this relationship, 1 would go with the following:
• Penetrating Ulcers are caused by atherosclerosis (this is a fact)
• Penetrating Ulcer can lead to Dissection (this is probably true in some cases)
• Atherosclerosis does NOT cause Dissection (which is confusing, may or may not be true,
and is unlikely to be tested). What is true is that the presence o f dense calcified plaque can
stop extension o f a dissection tear.
• Dissections often occur in the aortic root - where you have the highest flow pressures
• Penetrating Ulcers nearly never occur in the root - as these flow pressures prevent
atherosclerosis (wash those cheeseburger crumbs away).
T re a tm e n t o f P e n e tra tin g U lc e r ?
medical
Similar to Type B Dissections. If they do get treated (grafted e tc …) they tend
to do WORSE than dissections (on average)
T re a tm e n t o f P e n e tra tin g U lc e r ?
when are they surgical
Hemodynamic instability, Pain, Rupture, Distal Emboli, Rapid Enlargement
Dissection
overview
- The most common cause o f acute aortic syndrome (70%)
- Hypertension is the main factor - leads to an intimal tear resulting in two lumens
- Marfans, Turners, and other Connective Tissue Diseases increase risk
Dissection
classic testable scenarios
- Pregnancy — known to increase risk
- Cocaine Use in a young otherwise healthy person
- Patient with “Hypertension” and a sub-sternal “Tearing Sensation.”
Dissection
chicken vs egg
Some people say that hypertensive pressures kill the vasa vasorum (the
little vessels inside the vessel walls) leading to development o f intramural hematoma which
then ruptures into the intima. This is the “inside out” thinking. Other people think the
hypertensive forces tear the inner layer directly (“outside in” thinking).
Honestly… who gives a shit? Not even sure why I mentioned that.
Dissection
classifications
1) Time: Acute (< 2 weeks), or Chronic
(2) Location:
• Stanford A: Account for 75% of dissections and involves the ascending aorta and arch proximal
to the take-off of the left subclavian. These guys need to be treated surgically.
• Stanford B: Occur distal to the take-off of the left subclavian and arc treated medically unless
there are complications (organ ischemia etc…)
Dissection
true lumen
Continuity with
undissected portion of
aorta
Smaller cross sectional
areas (with higher velocity
blood)
Surrounded by
calcifications (if present)
Usually contains the origin
of celiac trunk, SMA, and
RIGHT renal artery
Dissection
false lumen
“CobWeb Sign” - slender
linear areas of low
attenuation
Larger cross section area
(slower more turbulent
flow)
Beak Sign - acute angle at
edge of lumen - seen on
axial plane
Usually contains the origin
of LEFT renal artery
Surrounds true lumen in
Type A Dissection
Dissection
floating viscera sign
This is a classic angiographic sign of abdominal aortic dissection.
It is shown as opacification of abdominal aortic branch vessels during aortography (catheter placed in the aortic true lumen), with the branch vessels— ( celiac axis, superior mesenteric artery, and right renal artery) arising out of nowhere.
They appear to be floating, with little or no antegrade opacification of the aortic true lumen.
Dissection Flap in the Abdomen - Vocab Trivia:
Static = dissection flap in the feeding artery (usually treated by stenting)
Dynamic = dissection flap dangling in front of ostium (usually treated with fenestration).
It can be hard to tell these apart. I f a sk ed I ‘d expect them to ju s t use the vocab words.
THIS vs THAT: Aneurysm with Mural Thrombus VS Thrombosed Dissection
- The dissection should spiral, the thrombus tends to drop straight down
- Intimal Calcs - the dissection will displace them.
THIS vs THAT: Aneurysm with Mural Thrombus VS Thrombosed Dissection
- The dissection should spiral, the thrombus tends to drop straight down
- Intimal Calcs - the dissection will displace them.
Intramural hematoma
htn
blasted vaso vasorum > intramural hematoma > intima tears > dissection
blasted vaso vasorum > intramural hematoma >serosal rupture > psuedoaneurysm
Intramural hematoma
atherosclerosis
focal plaque ruptures > focal intramural hematoma > dissection
focal plaque ruptures > focal intramural hematoma > serosal rupture > pseudoaneurysm
IMH
mechanism flow
can occur as
- primary event secondary to htn
- seconary event usually from atherosclerosis, but also as a focal hematoma on the road to dissection
for multible choice the cause is htn
IMH imaging findings
crescent sign
hyperdense on noncon
contrast CT difficult to distinguish from plaque
T1 bright crescent
IMH
treatment
Also uses the Stanford A vs B idea
Some people will say Type A = Surgery, Type B medical
• This is controversial and unlikely to be tested
IMH predictors of outcome
- Most o f these will spontaneously regress. These are the things that make that less likely:
- Hematoma Thickness Greater than 2 cm
- Association with aneurysmal dilation o f the aorta - 5 cm or more
- Progression to dissection or penetrating ulcer
- IMH + Penetrating Ulcer has a worse outcome compared to IMH + Dissection
THIS vs THAT: A n eu ry sm v s P s eu d o -a n e u r y sm -
The distinction between a true
and false aneurysm lends itself well to multiple choice testing. A true aneurysm is an
enlargement o f the lumen o f the vessel to 1.5 times its normal diameter. True = 3 layers are
intact. In a false (pseudo) aneurysm all 3 layers are NOT intact, and it is essentially a
contained rupture. The risk o f actual rupture is obviously higher with false aneurysm. It
can sometimes be difficult to tell, but as a general rule fusiform aneurysms are true, and
saccular aneurysms might be false. Classic causes o f pseudoaneurysm include trauma,
cardiologists (groin sticks), infection (mycotic), pancreatitis, and some vasculitides. On
ultrasound they could show you the classic yin/yang sign, with “to and fro” flow on pulsed
Doppler. The yin/yang sign can be seen in saccular true aneurysms, so you shouldn’t call it
on that alone (unless th at’s all they give you). To and Fro flow within the aneurysm neck +
clinical history is the best way to tell them apart.
SVC S y n d rom e
Occurs secondary to complete or near complete obstruction o f flow
in the SVC from external compression (lymphoma, lung cancer) or intravascular
obstruction (Central venous catheter, or pacemaker wire with thrombus). A less common
but testable cause is fibrosing mediastinitis (just think histoplasmosis). The dude is gonna
have face, neck, and bilateral arm swelling.
Traum a tic P s e u d o a n e u r y sm
Again a pseudoaneurysm is basically a
contained rupture. The most common place
to see this (in a living patient) is the aortic
isthmus (90%). This is supposedly the
result o f tethering from the ligamentum
arteriosum. The second and third most
common sites are the ascending aorta and
diaphragmatic hiatus - respectively.
Ascending aortic injury is actually probably
number one, it ju st kills them in the field so
you do n ’t see it. They could show you a
CXR with a wide mediastinum, deviation
o f the NG Tube to the right, depressed left
main bronchus, or left apical cap and want
you to suspect acute injury.
Asc end ing A o r tic C a lc ific a tio n s
There are only a few causes o f ascending aortic
calcifications, as atherosclerosis typically spares the ascending aorta. Takayasu and
Syphilis should come to mind. The real-life significance is the clamping o f the aorta may
be difficult during CABG.
A n eu ry sm
Defined as enlargement o f the artery to 1.5 times its expected diameter
(> 4 cm Ascending and Transverse, > 3.5 cm Descending, > 3.0 cm Abdominal).
Atherosclerosis is the most common overall cause. Medial degeneration is the most
common cause in the ascending aorta. Patients with connective tissue (Marfans, Ehlers
Danlos) diseases tend to involve the aortic root. When I say cystic medial necrosis you
should think Marfans. Aneurysms may develop in any segment o f the aorta, but most
involve the infra-renal abdominal aorta. This varies based on risk factors, rate o f growth,
e tc … but a general rule is surgical repair for aneurysms at 6cm in the chest (5.5 cm with
collagen vascular disease) and 5 cm in the abdomen.
Sinus o f V a ls a lv a A n eu ry sm
Aneurysms o f the valsalva sinus (aortic sinus) are
rare in real life, but have been known to show up on multiple choice tests. Factoids worth
knowing are that they are more common in Asian Men, and typically involve the right
sinus. They can be congenital or acquired (infectious). VSD is the most common associated
cardiac anomaly. Rupture can lead to cardiac tamponade. Surgical repair with Bentall
procedure.
R upture / Im p en d in g R u p tu re
Peri-aortic stranding, rapid enlargement (10 mm or
more per year), or pain are warning signs o f impending rupture. A retroperitoneal hematoma
adjacent to an AAA is the most common imaging finding o f actual rupture. The most
common indicator for elective repair is the maximum diameter o f the aneurysm , “Sac Size
Matters,” with treatment usually around 6 cm (5.5 cm in patients with collagen vascular
disease). A thick, circumferential mural thrombus is thought to be protective against rupture.
Enlargement o f the patent lumen can indicate lysis o f thrombus and predispose to rupture.
Findings of impending rupture
Draped Aorta Sign
Posterior wall of the aorta drapes over the vertebral column.
Increased Aneurysm Size
10 mm or more increased per year
Focal Discontinuity in Circumferential Wall Calcifications
Hyperdense Crescent Sign
Well-defined peripheral crescent of increased attenuation. One of the most specific manifestations of impending rupture.
M y c o tic A n eu ry sm
These are most often saccular and most often pseudoaneurysms. They are prone to rupture. They most often occur via hematogenous seeding in the setting o f septicemia (endocarditis). They can occur from direct seeding via a psoas abscess or vertebral osteomyelitis (but this is less common). Most occur in the thoracic or supra-renal aorta (most atherosclerotic aortic aneurysms are infra-renal).
Typical findings include saccular shape, lobular contours, peri-aortic inflammation, abscess, and peri-aortic gas. They tend to expand faster than atherosclerotic aneurysms. In general small, asymptomatic, and unruptured.
M y c o tic A n eu ry sm
gamesmanship
If you see a saccular aneurysm o f the aorta (especially the I f abdominal aorta) you have to lead with infection.
NF 1
One o f the more common neurological genetic disorders, which you usually think about causing all the skin stuff (Cafe au lait spots and freckling), and bilateral optic gliomas. Although uncommon, vascular findings also occur in this disorder. Aneurysms and stenoses
are sometimes seen in the aorta and larger arteries, while dysplastic features are found in smaller vessels. Renal artery stenosis can occur, leading to renovascular hypertension (found in 5% o f children with NF). The classic look is orificial renal artery stenosis presenting with hypertension in a teenager or child. The mechanism is actually Dysplasia o f the arterial wall itself (less common from peri-arterial neurofibroma).
Marfan Syndrome
overview
Genetic disorder caused by mutations of the fibrillin gene (step 1 question). There are lots o f systemic manifestations including ectopic lens, being tall, pectus
deformity, scoliosis, long fingers etc… Vascular findings can be grouped into aneurysm, dissection, and pulmonary artery dilation:
Marfan Syndrome
aneurysm
Dilation with Marfans is classically described as “Annuloaortic ectasia”,
with dilatation o f the aortic root. The dilation usually begins with the aortic sinuses, and then progresses into the sinotubular junction, ultimately involving the aortic annulus. Dilatation of the aortic root leads to aortic valve insufficiency. Severe aortic regurgitation occurs that may progress to aortic root dissection or rupture. The mechanism for all this nonsense is that disruption o f the media elastic fibers causes aortic stiffening, and predisposes to aneurysm and dissection. The buzzword for the Marfans ascending aneurysm is “tulip bulb. They are usually repaired earlier than normal aneurysm (typically around 5.5 cm).
Marfan Syndrome
dissection
Recurrent dissections are common, and even “triple barreled dissection” can be seen (dissections on both sides o f a true channel).
Marfan Syndrome
pulmonary artery enlargement
Just like dilation o f the aorta, pulmonary artery enlargement favors the root.
Marfans quick
Annuloaortic extasia with dilationof the aortic root
Loeys Dietz Syndrome
overview
Despite the name, this is actually not a Puerto Rican DJ. Instead think o f this as the really shitty version o f Marfans. They have a terrible prognosis, and rupture their aortas all the time. Vessels are very tortuous (twisty). They also have crazy wide eyes (hypertelorism).
Loeys Dietz Syndrome
classic triad
- Hypertelorism (frog eyes),
- Bifid uvula or cleft palate
- Aortic aneurysm with tortuosity
Loeys Ditz classic look
Crazy twisty vertebral arteries
Ehlers -D an los
This one is a disorder in collagen, with lots o f different subtypes. They have the stretchy skin, hypermobile joints, blood vessel fragility with bleeding diatheses.
Invasive diagnostic studies such as conventional angiography and other percutaneous procedures should be avoided because o f the excessive risk o f arterial dissection. Imaging characteristics o f aortic aneurysms in Ehlers-Danlos syndrome resemble those in Marfan syndrome, often involving the aortic root. Aneurysms o f the abdominal visceral arteries are common as well.
S y p h ilitic (L u e tic ) A n eu ry sm
This is super rare and only seen in patients with untreated tertiary syphilis. There is classically a saccular appearance and it involves the ascending aorta as well as the aortic arch. Classic description “saccular asymmetric aortic aneurysm with involvement o f the aortic root branches. ” Often heavily calcified “tree bark” intimal calcifications. Coronary artery narrowing (at the ostium) is seen 30% o f the time. Aortic valve insufficiency is also common.
A o r to e n te ric F is tu la
primary
Very, very, very rare. Refers to an A-E fistula without history o f instrumentation. They are only seen in the setting o f aneurysm and atherosclerosis.
A o r to e n te ric F is tu la
secondary
Much more common. They are seen after surgery with or without stent
graft placement.
Aortenteric fistula
The question is usually what part o f the bowel is involved, and the answer is 3 rd and 4th portions of the duodenum. The second most likely question is A-E fistula vs perigraft infection (without fistula)? The answer to that is unless you see contrast from the aorta into the bowel lumen (usually duodenum), you c an ’t tell. Both o f them have ectopic perigraft gas > 4 weeks post repair, both have perigraft fluid and edema, both lose the fat place between the bowel and aorta (tethering o f the duodenum to the anterior wall o f the aorta), both can have pseudoaneurysm formation.
