Diseases of Aorta Flashcards

1
Q

Definition of aneurysm

A

localized/ diffuse dilation of artery with diameter at least 50% greater than normal size (in abdominal aorta >3 cm)

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2
Q

What are the critical points (size) of thoracic aortic aneurysms and abdominal aortic aneurysms for surgical intervention to be necessary?

A

TAA=5.5 cm

AAA= 4.0 cm

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3
Q

Complications of thoracic aortic aneurysms

A

1) Compression of mediastinal structures (e.g. airway/ esophagus)
2) Thrombosis and embolism
3) Stretching of left recurrent laryngeal → brassy cough and voice hoarseness

4) Dilitation of aortic valve root, resulting in aortic regurgitation

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4
Q

Possible reasons why HTN develops in pt with juxtaductal aortic coarctation

A

1) mechanical obstruction
2) Renin-angiotensin-mediated humoral mechanisms: renal artereis are underperfused and activate renin to increase pressure

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5
Q

Clinical presentation of acute dissection (3)

A

1) Sudden onset severe pain (10/10)
2) “Tearing” or “ripping” quality of pain
3) Radiation of pain to the back or scapula

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6
Q

How can dissection lead to ischemia of the gut?

A

If the dissection extends to the abdomen, it can occlude the superior mesenteric artery. The parts of the gut supplied by this artery and its branches will die.

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7
Q

Why is aortic regurgitation a possible consequence of aortic dissections?

A

Dissection can go proximally towards the heart, and can modify the cusps in various ways. For instance, it can pull the cusps apart, it can make it flail, can break it off, etc.

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8
Q

What is the role of collagen in tunica media of aorta?

A

Provides strength that enables aorta to withstand large amounts of pressure loads.

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9
Q

Loeys-Dietz syndrome

A

Mutation of TGF-beta receptor

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10
Q

What are possible consequences when blood from media ruptures through the adventitia?

A

1) Extend into pericardial sac, which can lead to cardiac tamponade
2) Etend into pleural or peritoneal cavities leading to massive hemorrhage

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11
Q

Why does atherosclerosis make abdominal aorta more susceptible to aneurysms, especially abdominal aortic aneurysms?

A

Below the renal arteries, there is no vasa vasorum, so abdominal aorta has to rely on diffusion for getting oxygen and nutrients. Atherosclerosis increases the diffusion barrier to the media, creating atrophy and weakness of vessel wall.

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12
Q

What is associated with Turner’s syndrome, coarctation of aorta, cardial septal defects, and abnormalities of the coronary circulation?

A

Bicuspid aortic valves

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13
Q

On gross examination, what does an aorta with aneurysm look like?

A

“tree bark” appearance

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14
Q

Difference between MI pain and Acute dissection pain (Type of pain, onset, and radiation)

A

MI pain: Heaviness/pressure-like pain as if something is sitting on chest. Pain starts low and increases in severity as heart becomes ischemic. Pain radiates to the left arm, shoulder/neck

Acute Dissection pain: Sudden onset with 10/10 at onset; ripping/tearing type of pain. Pain radiates to back or scapula.

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15
Q

What types of murmurs are associated with coarctation?

A

Aortic stenosis murmur: Systolic ejection murmur crescendo-decrescendo with over Left infra-clavicular area of under left scapula

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16
Q

Ehlers-Danlos syndrome

A

mutations encoding type III pro-collagen

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17
Q

Pathophysiology of aortic dissection

A

Occurs when there is tear in the intima, allowing blood to gain access to a diseased media. The media has underlying damage (due to chronic HTN, aging, cystic medial degeneration) and allows blood to travel both proximally and distally within the blood vessel, called the false lumen.

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18
Q

Degenerative calcific aortic stenosis and how this differs from bicuspid aortic stenosis

A

Degenerative calcific aortic stenosis is the most common type of aortic stenosis. This is due to overuse/ atherosclerosis/ HTN which causes degeneration of the valve. However, this occurs in older patients (>70 years). In contrast, bicuspid aortic stenosis occurs in young and middle-aged adults.

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19
Q

What antibiotic should be included in tx for thoracic aortic aneurysms?

A

PCN for tertiary syphillus

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20
Q

How can pulse deficits occur with aortic dissections? What manifestations can occur as a result of pulse deficits?

A

The dissection can go up to the brachiocephalic trunk, occluding the right common carotid and right subclavian. It can also go to the left common carotid artery and left subclavian artery, obstructing flow there as well. This obstruction can result in upper limb ischemia and/or stroke symptoms.

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21
Q

What is a lamellar unit of the aorta and what is the function?

A

A lamellar unit is a concentric layer of aorta consisting of smooth muscle cell that is embedded in matrix of elastin and collagen. These lamellar units allow aorta to withstand high pressures, allow distensiblity, and for the pressure to evenly distribute the flow to the extremities.

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22
Q

3 main risk factors of aortic dissection

A

1) HTN- major risk factor
2) Congenital CT disorders (e.g. Marfan syndrome)
3) Bicuspid aortic valve

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23
Q

Why do 1 in 4 individuals have variation in aortic arch anatomy?

A

During embryological development of aortic arch, there are errors in programming during rearranging/reabsorbing

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24
Q

What is the most common presenting symptom of thoracic aortic aneurysm?

A

They are asymptomatic until they rupture or are impending rupture. Most common presenting sx is chest pain.

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25
Q

What is associated with a continuous machine-like murmur?

A

Patent Ductus Arteriosus

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26
Q

In general, what happens to murmurs during valsalva and why?

A

Murmur becomes softer. This is because during valsalva, person increases intra-abdominal pressure → decrease return to the heart → decrease amount of blood that’s flowing over problematic valve → softer murmur.

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27
Q

How does familial thoracic aortic aneurysm predispose pt to aortic dissection?

A

Familial thoracic aortic aneurysm leads to cystic medial degeneration; a breakdown of collagen, elastin, and smooth muscle in the media of aortic wall.

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28
Q

What is the most common presentation of bicuspid aortic valve?

A

Aortic stenosis in a young- middle aged patient (e.g. 30 year old)

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29
Q

How is tertiary syphilis infection associated with thoracic aortic aneurysms?

A

Syphilis causes endarteritis of vasa vasorum of ascending and transverse portions of aortic arch → luminal narrowing → decreased flow → atrophy of vessel wall

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30
Q

Turner syndrome

A

XO

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31
Q

Why does systolic blood pressure rise as a consequence of aging?

A

The elastic component of the aorta in media degenerates, and collagen > elastin. As a result, the arteries stiffen and systemic blood pressure rises.

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32
Q

How does damage to media occur before aortic dissection?

A

Cystic medial degeneration- HTN + atherosclerosis lead to necrosis and fibrosis of the media. As a result, when the blood enters media after intima rips, there is no structural support and cannot provide resistance to the blood flow.

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33
Q

what are the ways in which thoracic aortic aneurysms can be acquired? (3)

A

1) Degenerative (HTN, smoking, atherosclerosis)
2) Acquired infection (syphilis)
3) Inflammatory conditions

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34
Q

What is the major complication of abdominal aortic aneurysm and how does it present?

A

Rupture; present as triad of hypotension, pulsatile abdominal mass, flank pain usually to the left side.

35
Q

Two fates of dissection once blood enters media

A

1) Extend within the media proximally toward the heart and/or distally toward the lower extremities
2) Rupture through the adventitia into the: Pericardial sac can lead to cardiac tamponade (most common cause of death in aortic dissection) or pleural or peritoneal cavities leading to massive hemorrhage

36
Q

Key examination findings for acute dissection (3)

A

1) HTN- almost always
2) Murmur of aortic regurgitation
3) Pulse deficits

37
Q

What finding in CT Angiography indicates aortic dissection?

A

False lumen or intimal flap

38
Q

What are the 3 possible catastrophic dissections?

A

1) Free rupture- false lumen ruptures and blood enters chest → death
2) Occlusion of coronary ostia: via dissection; person gets MI
3) Proximal rupture into pericardium: Dissection goes to pericardium → pericardial tamponade → death

39
Q

How do majority of adult patients get dx with coarctation?

A

First, incidental HTN but cannot be controlled with meds → think coarctation.

40
Q

Saccular aneurysm

A

Spherical out-pouching involving only a portion of the blood vessel

41
Q

Cystic medial degeneration

A

necrosis and fibrosis of media due to HTN + atherosclerosis or congenital CT defects (e.g. Ehlers-Danlos or Marfan’s syndrome)

42
Q

Physical exam finding of AAA

A

Pulsatile abdominal mass. However, this can be hard for some pts due to their high BMIs.

43
Q

How does diameter change from thoracic to abdominal/infra-renal portions of aorta?

A

Diameter decreases

44
Q

What are the two main sx assocaited with a ruptured AAA? What are some other sx?

A

Abdominal or back pain + Hypotension are the two most common signs. Other sx include cold, clammy extremities.

45
Q

What determines clinical consequence of how sick a pt is with aortic coarctation?

A

Depends on how obstructed the coarctation causes the aorta to become; if completely obstructed, baby looks very sick. If there is a partial obstruction, patient can present as an older child (e.g. 11-12 y-o) or even as an adult.

46
Q

Fibrillin-1

A

Glycoprotein that tethers vascular smooth muscle cells to matrix of elastin and collagen, allowing aortic wall and valves to have structural integrity

47
Q

How does adult coarctation of aorta present?

A

HTN in upper extremities and hypotension with weak pulses in lower extremities

48
Q

Most common place or aortic aneurysms

A

Infrarenal abdominal aorta

49
Q

Famillial thoracic aortic aneurysm

A

Autosomal dominant condition that leads to cystic medial degeneration.

50
Q

Marfan syndrome

A

Mutation of fibrillin-1, impairing formation of elastin

51
Q

Reasons why Stanford A aortic dissections are more dangerous than Stanford B

A

They can lead to:

1) Occlusion of coronary arteries leading to MI
2) Occlusion of carotid artery leading to stroke
3) Involve pericardial sac resulting in cardiac tamponade.

52
Q

What is the classic finding of an CXR of a pt with aortic dissection?

A

Widening of mediastinum

53
Q

How do infantile coarctation of aorta present? (2 ways)

A

1) CHF (tachycardia, poor feeding, shock): if ductus arteriosus is closed and aorta is completed obstructed, LV tries to work against obstructed aorta and baby has complete CHF.
2) Differential cyanosis- cyanosis in lower half of body. This occurs when infant has PDA; blood from pulmonary trunk will go into aorta distal to coarctation due to reduced pressure and feed the lower half of the body deoxygenated blood.

54
Q

What population is most commonly affected by abdominal aortic aneurysms?

A

HTN male smokers that are older than 60

55
Q

Fusiform aneurysm

A

Diffuse and circumferential dilation of a long segment of vessel

56
Q

Where do coarctations of the aorta usually occur?

A

At the aortic isthmus, aortic narrowing near insertion of ductus arteriosus (“juxtaductal”)

57
Q

How do elastin and collagen content change from thoracic to abdominal/infra-renal aorta?

A

Elastin and collagen content dec

58
Q

Genetic syndromes associated with aortic dissections (4)

A

1) Marfan syndrome
2) Ehler-Danlos Syndrome
3) Turner syndrome
4) Bicuspid aortic valve

59
Q

Syndromes associated with aortic aneurysms for pts under the age of 65 (5)

A

All of the syndromes have connective tissue disorders:

1) Marfan syndrome
2) Ehlers-Danlos syndrome
3) Loeys-Dietz Syndrome
4) Turner’s syndrome
5) Impaired copper metabolism

60
Q

Most common cause of death in aortic dissection

A

Cardiac tamponade

61
Q

What does it mean in regards to obstruction by coarctation when an older child or adult presents? What are the classic findings in these patients? (3)

A

Means that obstruction is partial and did not cause them to be sick as a baby (not a complete obstruction).

Classic findings:

1) Systolic HTN in upper extremities
2) Reduced systolic BP in lower extremities (>20 mmHg when compared to UE)
3) Radial artery to femoral pulse delay

62
Q

How do medial elastin layers of aorta change going from proximal to distal (thoracic → abdominal/infra-renal)

A

Decline in medial elastin layers

63
Q

What is a consequence of deficiency of fibrillin-1?

A

Marfan syndrome; leads to smooth muscle cell detachment from elastin and collagen, inducing apoptosis and loss of ECM structural integrity within the aortic wall.

64
Q

3 possible pathophysiology of aortic aneurysms over age 65

A

1) Degradation of medial connective tissue by increased matrix metalloproteinase (MMP) activity
2) Ischemic injury to the media: related to atherosclerosis/damage to vasa vasorum
3) Poor quality vascular connective tissue due to nutritional disorders: activation of proteases breaks down wall of aorta → pressure results in expansion/thinning of wall.

65
Q

Pathophysiology of notching of ribs and HTN due to juxtaductal coarctation of aorta

A

Increased afterload due to obstruction of aorta→ increased LV wall stress and compensatory LV hypertrophy → development of aortic collaterals to intercostal arteries →notching of ribs

*HTN (don’t know why)

66
Q

Common causes of aortic dissection

A

Chronic arterial HTN

Smoking

Cocaine

Trauma

67
Q

What is the function of elastic fibers in tunica media of the aorta

A

Elastin allows distensibility of the aorta, allowing them to recoil under pressure.

68
Q

How do infants present with coarctation?

A

1) Congestive heart failure (tachycardia/poor feeding/shock)- in severe coarctations, aorta is obstructed; LV tries to work against completely obstructed aorta.
2) Differential cyanosis- cyanotic in lower extremities; if DA is still open, blood is perfusing the lower extremities from pulmonary artery and not the lungs.

69
Q

How does juxtaductal coarctation affect a fetus in utero?

A

It DOES NOT cause any hemodynamic problems in utero because CO flows through PDA into the descending aorta, bypassing the coarctation at the isthmus.

70
Q

Classifications of Aortic Dissection and how they’re different in regards to location and treatment.

A

Stanford A (proximal): must involve the ascending aorta, and may extend to involve the aortic arch and descending aorta. Treatment is usually surgical.

Stanford B (distal): Does not involve ascending aorta and therefore is confied to descending thoracic/ abdominal aorta. Usually managed medically unless it is leaking or ruptured.

71
Q

When is risk of AAA rupture increased?

A

When the aneurysm is greater than 5 cm

72
Q

What medications are used to treat Stanford B aortic dissections?

A

Beta blockers and subsequent vasodilators (e.g. sodium nitroprusside)

73
Q

Most common cause of abdomianl aortic aneurysms

A

Atherosclerosis

74
Q

What pathology is associated wiht other congenital heart defects such as VSD, canal defects, and especially bicuspid aortic valve , as well as Turner’s syndrome? a

A

Coarctation of the aorta.

75
Q

What is the most common presenting symptom of AAA?

A

They are asymptomatic until exapnsion or rupture. Symptoms include back, flank, abdominal, or groin pain, including compressive symptoms.

76
Q

How is aortic aneurysms managed before surgical intervention is necessary?

A

Beta blockers, ARBs

77
Q

Why may patients with aortic dissection have unequal pulses in their extremities

A

Due to partial occlusion of left subclavian artery or brachiocephalic trunk

78
Q

What are the ranges of presentations in bicuspid aortic valves?

A

Severe aortic stenosis in childhood to asymptomatic until old age.

79
Q

Causes of aortic dissections in older adults (40-60 years of age) compared to younger adults

A

Adults: most commonly due to hypertension

Younger adults: most commonly due to connective tissue disorders.

80
Q

What is the ostea and how can this be affected in aortic dissection?

A

Once blood enters media, can propagate circumferentially and create a “case” around normal lumen → obstruction of flow → obstruction of ostea (vessels coming off aorta)

81
Q

Branches coming off from aorta from proximal to distal

A

1) R + L coronary arteries
2) Brachiocephalic artery → Right subclavian artery + right common carotid artery
3) Left common carotid artery
4) Left subclavian artery

82
Q

Where do most aortic dissections occur?

A

Ascending aorta (65%), aortic arch (5%), descending aorta (20%).

83
Q

What type of coarctation (infantile or adult) is associated with Turner syndrome?

A

Infantile coarctation