8/24- Pathology of the Aorta Flashcards

1
Q

Describe the intima (of the aorta)?

A

Single layer of endothelial cells, loose CT (GAG) and internal elastic lamina (diffusion of thin intima)

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2
Q

Describe the media (of the aorta)?

How does it receive nutrients?

A
  • Smooth muscle

- Elastic lamellae: // to distribute pressure load

  • Collagen: cross-linked: provide strength

Pressure shouldn’t deform!

Gets nutrients from diffusion from lumen through intima

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3
Q

Describe the adventitia (of the aorta)?

A

Encircles media contains loose CT, nerves, and arteries (vaso vasorum)

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4
Q

What is the number 1 cause of hypertension in kids?

A

Always secondary; here, in the aorta– due to coarctation

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5
Q

What is a key finding in aortic coarctation?

A

Rib notching on CXR

  • Due to decreased blood flow from posterior intercostals (come straight from aorta; below coarctation) with increased blood flow from anterior intercostals (from subclavian)
  • Results in wavy/tortuous arteries that can be see on CXR
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6
Q

What are the different types of aortic coarctation?

A
  • Infantile or pre-ductal
  • Adult or post-ductal
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7
Q

What is the treatment for aortic coarctation?

A

Ressection and end-anastamosis (treat early!)

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8
Q

What is the cause of morbidity/mortality with aortic coarctation?

A

- HTN

- CHF (L heart failure due to hypertrophy and then R)

- Rupture (increased P in head and neck)

- Endocarditis

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9
Q

Case)

  • 24 yo male; 6’4” thin male…
A

Think Marfan’s!

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10
Q

What causes problems in Marfan’s syndrome?

A

Defective synthesis of fibrillin -> increased TGF-beta activity and weakening of the elastic tissue -> progressive dilation due to remodeling of the inelastic media

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11
Q

Why is aneurysm and dissection mostly in the ascending aorta in Marfan patients?

A

Related to LaPlace’s law: WS ~ Pr/2h

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12
Q

What are some congenital diseases that predispose to aneurysm formation:

A
  • Marfan’s syndrome
  • Loeys Dietz syndrome
  • Ehlers-Danlos syndrome
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13
Q

What is the mechanism behind Loeys-Dietz syndrome?

A

Mutations in TGF-beta cause abnormal elastin and collagen I and III

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14
Q

What is the mechanism behind Ehlers-Danlos syndrome?

A

Defective type III collagen

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15
Q

What are some acquired aortic disease and age-related changes?

A
  • Decrease in elastic fiber: dilatation and aneurysms
  • Atherosclerosis:AA aneurysms
  • ?
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16
Q

Aortic root dilatation causes what?

A

Aortic valve incompetence (insufficiency?)

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17
Q

T/F: unfolding of the aorta is a change associated with aging?

A

True

  • Aorta straightens out
  • Increased length of descending aorta
  • (Must be distinguished from aortic aneurysm)
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18
Q

T/F: atherosclerosis is a change associated with aging?

A

True

  • In the elderly, atherosclerosis is more often irreversible (in middle age there was more adaptive thickening; reversible)
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19
Q

Pathophysiology of atherosclerosis?

A

Shear stress -> thickening -> high BP -> shear stress…

20
Q

What is xanthoma?

A

Cholesterol deposition in intima (part of the atherosclerosis process)

21
Q

Case)

  • 54 yo Af AM male presents to ER with acute onset of abdominal and low back pain
  • PMH: DMII, HTN, Hypercholesteremia
  • …. Dx? What are his risk factors?
A

Dx: rupture abdominal aortic aneurysm

  • Pic here is “fusiform aneurysm”- circumferential dilatation

Risk factors:

  • DM -> poor nourishment -> wall weakness
  • HTN
  • Hypercholesteremia, DM, and obesity are all implicated in development of atherosclerosis
22
Q

Different types of aneurysm?

A

True aneurysm

  • Saccular
  • Fusiform

False aneurysm: extravascular CT; hematoma with extravasation of blood

Dissection: tear in intima with extravasation of blood

23
Q

Pathogenesis of aneurysms?

A
  • Arterial ECM is in continual remodeling; balanced synthesis and degradation
  • Increased degradation vs. synthesis (with age) through: apoptosis, proteolytic enzymes, and matrix metalloproteinase (MMP) degrade the ECM
  • Ischemia: loss of SM and increase in GAGs
24
Q

What are the 2 most common disorders that predispose to aortic aneurysms?

A
  • Atherosclerosis
  • Hypertension
25
Q

T/F: Aortic aneurysms occur most commonly in the abdomen?

A

True

  • Atherosclerosis is commonly most severe at the distal aorta
  • Results in atherosclerotic abdominal aneurysms
  • Also have fewer vaso vasorum here
26
Q

What are conditions that weaken vessel walls resulting in aneurysms?

A
  • Diabetes mellitus
  • Trauma
  • Vasculitis
  • Congenital defects (e.g. berry aneurysms)
  • Infections (mycotic aneurysms)
27
Q

What may cause thoracic aortic aneurysms?

A

Tertiary syphilis

  • Obliterative endoarteritis
  • Predeliction for small vessels
  • Involvement of vasa vasorum of aorta can -> aneurysmal dilation
28
Q

What is the most likely COD in pt with abdominal aortic aneurysm, HTN, DMII..?

A

Rupture of aortic aneurysm

29
Q

Clinical Outcomes of AAAs?

A
  • Rupture (into peritoneal or retroperitoneal cavity) with potentially fatal hemorrhage
  • Obstruction of vessel -> ischemic injury (iliac, renal, mesenteric, vertebral branches)
  • Impingement of adjacent structures (ureter, erosion of vertebrae)
  • Embolism from atheroma or mural thrombus
30
Q

T/F: mycotic AAAs are due to systemic fungal infections?

A

False

  • Mycotic AAAs are atherosclerotic aneurysms that have become infected by circulating organisms which may be fungal but are more commonly due to bacteremia (e.g. Salmonella gastroenteritis)
31
Q

How might a pt present with thoracic aortic aneurysms?

A
  • Encroachment of mediastinal structures
  • Respiratory difficulties
  • Difficulty swallowing
  • Bone pain from erosion of ribs or vertebra
  • Aortic valve dilation with resulting valvular insufficiency
  • Narrowing of coronary ostia – ischemia
  • Rupture
32
Q

What factor is directly associated with increased risk of rupture?

A

Risk of rupture is directly related to size

  • LaPlace’s law: WS ~ diam and internal P
  • Blood pressure is the most important factor affecting increase in size (increases WS directly and indirectly through gradual increase in size)
33
Q

Best treatment for thoracic aortic aneurysm?

A

Surgery is not better than management unless you have impending aneurysm

34
Q

Case)

  • 60 yo woman presents to ED with acute onset, severe ripping upper back pain
  • PMH: HTN
  • HR: 100
  • BP: 210/105
  • SpO2: 100% RA

What is this?

Risk factors (general, not just hers)?

A

Dx: Aortic dissection

  • Dissection of blood between laminar planes of the media
  • Formation of channel within aortic wall
  • Can rupture outward resulting in massive hemorrhage

Risk factors:

  • Age
  • HTN
  • Sex
35
Q

What is seen here?

A

Aortic dissection

  • Blood-filled channel within the aortic wall
36
Q

Epidemiology of aortic dissection?

A

> 90% of pts with dissection are men 40-60 yo with hx of HTN

  • Minority are younger with CT disorders
37
Q

Describe the gross findings in this picture?

A

Hemocardium

  • Consequence of (ascending) thoracic aortic dissection with retrograde channeling of blood into the heart
  • Can lead to cardiac tamponade
38
Q

Risk of complications in aortic dissection depend on what?

Difference between classes?

A

Level of aorta affected

  • (DeBakey I-III)

- Type A: treated surgically; pressure could impinge on coronary arteries -> ischemia and valve dysfunction; very symptomatic

- Type B: treated medically

39
Q

What are some consequences of retrograde dissection of the aorta?

A
  • Aortic valvulopathy
  • Cardiac tamponade
  • Myocardial infarction (due to decreased perfusion)
  • Extension into great arteries of the neck
  • Compression of adjacent vessel
40
Q

What is the most common false aneurysm (pseudoaneurysm)?

A

Post MI rupture

  • Contained by pericardial adhesion or at the junction of a vascular graft with a natural artery (anastomosis)
41
Q

Case)

  • 54 yo white male with abdominal pulsatile mass had bilateral hydronephrosis

Diagnosis?

A

AAA may impinge on the ureters as they pass into the pelvic cavity to attach to the bladder

42
Q

Describe the gross findings?

A

Thoracic aortic dissection

  • Dissection area is black -> blood clot within the wall of thoracic aorta
43
Q

What is an acquired connective tissue disorder that may dispose to aneurysms and thus dissections?

A

Vitamin C deficiency

  • Scurvy
  • Defective collagen cross-linking
  • Results in aneurysm formation (cystic medial degeneration)
44
Q

What is the cause of morbidity/mortality with aortic coarctation?

A
  • HTN
  • CHF
  • Rupture
  • Endocarditis
45
Q

What is an anuerysm? Classifications/causes?

A

A localized abnormal dilation

  • Congenital
  • Acquired

True aneurysm:

  • Atherosclerotic
  • Syphilitic
  • Congenital aneurysm
  • Ventricular aneurysm

False aneurysm (pseudo-aneurysm)