Vascular Pathology I Flashcards

1
Q

Both arteries and veins are composed how?

A

from inside to out, both arteries and veins are composed of an intima, a media, and an anventitia

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2
Q

what level is blood pressure controlled?

A

at the level of the arteriole

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3
Q

how does a vein’s media differ from an artery’s?

A

since veins are low pressure, they only have a thin walled media without significant elastic fibers

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4
Q

how do capillaries differ from veins/arteries?

A

capillaries only have a simple endothelial lining surrounded by pericytes but no media

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5
Q

what is an arteriovenous malformation?

A

a direct connection of arteries to veins, bypassing capillaries

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6
Q

What do large or multiple AVMs lead to?

A

they shunt blood from arterial to venous circulation, forcing the heart to pump additional volume leading to high-output cardiac failure

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7
Q

why would an AVM be surgically created?

A

it provides vascular access for hemodialysis or chemotherapy agents

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8
Q

how might a patient with a superficial AVM of the brain present?

A

seizure and intracerebral hemorrhage

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9
Q

What is a berry (saccular) aneurysm?

A

a focal abnormal dilation of an artery due to underlying defect in media

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10
Q

what are the risk factors for berry (saccular) aneurysm?

A

HTN and smoking

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11
Q

Berry (saccular) aneurysms are associated with what other diseases?

A

AD polycystic kidney disease, Marfan syndrome, and Ehlers-Danlos syndrome

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12
Q

berry aneurysms can lead to what?

A

subarrachnoid hemorrhage if they rupture

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13
Q

rupture of a berry aneurysm is associated with that?

A

acute increases in intracranial pressure like straining with stool or sexual orgasm

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14
Q

what is a mycotic aneurysm?

A

a dilation of an artery due to damage of the vessel wall by an infectious process

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15
Q

what is fibromuscular dysplasia?

A

an irregular thickening in muscular arteries caused by medial and intimal hyperplasia

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16
Q

how does fibromuscular dysplasia arise?

A

thought to be a developmental abnormality, as it has increased incidence in first degree relatives

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17
Q

how does fibromuscular dysplasia appear on angiography?

A

string of beads appearance

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18
Q

which arteries are most commonly affected by fibromuscular dysplasia and what does this lead to?

A

the renal arteries–> leads to renal artery stenosis; the renal artery stenosis causes low volume of blood flow to the kidney which activates the renin, angiotensis, aldosterone system

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19
Q

what kidney diseases could lead to secondary HTN?

A

renovascular disease, renal artery stenosis (caused by fibromuscular dysplasia or atherosclerosis) or polycystic kidney disease

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20
Q

what endocrine disease could lead to secondary HTN?

A

primary aldosteronism, cushing syndrome, or pheochromocytoma

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21
Q

what cardiovascular diseases could lead to secondary HTN?

A

coarctation of the aorta

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22
Q

how does primary aldosteronism present?

A

HTN and hypokalemia

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23
Q

if a patient is hypokalemic, how might they present?

A

weakness, muscle cramps, paresthesias, and visual disturbances

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24
Q

what is usually the cause of primary hyperaldosteronism?

A

bilateral nodular hyperplasia of the adrenal gland

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25
Q

what effect does aldosterone have on the body?

A

it reabsorbs sodium and excretes potassium leading to HTN and hypokalemia

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26
Q

what causes cushing syndrome?

A

increased cortisol production by the adrenal gland

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27
Q

what is a pheochromacytoma?

A

a tumor of chromaffin cells of the adrenal medulla

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28
Q

what does a pheochromocytoma secrete?

A

catecholamines including epinephrine and norepinephrine

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29
Q

besides HTN, what are the other associated features of a pheochromocytoma?

A

it can induce paroxysms of elevated BP associated with tachycardia, palpitation, HA, diaphoresis, tremor

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30
Q

what are pheochromocytomas associated with?

A

MEN 2 syndromes

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31
Q

how would you test for a pheochromocytoma?

A

test urinary or plasma metanephrines (they’ll be elevated)

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32
Q

what is the adult form of coarctation of the aorta?

A

there’s a congenital narrowing or infolding of the aorta opposite the ligamentum arteriosum

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33
Q

what effect does coarctation of the aorta have on the body?

A

HTN in the UEs with weak pulses and hypotension in the LEs

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34
Q

what is coarctation of the aorta associated with?

A

bicuspid aortic valve

35
Q

what effect does mild to moderate chronic hypertensive disease have on the small arterioles?

A

hyaline arteriolosclerosis

36
Q

what is hyaline arteriolosclerosis?

A

there is arteriolar narrowing- the hyaline material is compromised of precipitated plasma proteins

37
Q

what happens in hyaline nephrosclerosis?

A

there is narrowing–> impairment of the renal blood supply and ischemic glomerulosclerosis

38
Q

what is the definition of a hypertensive crisis?

A

a rapid increase in BP: systolic >180-200 and diastolic >120

39
Q

what is a hypertensive emergency?

A

a hypertensive crisis with end organ damage

40
Q

what is hyperplastic arteriolosclerosis?

A

occurs in severe HTN, the pressure induces the artery to produce lamellated, thickening of the walls–> smooth muscle forms concentric lamellations showing an “onion skinning” pattern

41
Q

the gross findings of a kidney in long standing HTN correspond with what?

A

hyaline arteriolosclerosis with characteristic pink amorphous change

42
Q

what are the gross findings in a kidney in a case of rapidly increasing BP?

A

numerous petechial hemorrhages–> hyperplastic arteriolosclerosis

43
Q

what is myocardial vessel vasospasm?

A

excessive vasoconstriction of myocardial arteries or arterioles which could cause ischemia or infarct

44
Q

what are 3 causes of myocardial vascular contraction (vasospasm)?

A

1) high levels of vasoactive mediators 2) elevated thyroid hormones 3) autoantibodies and T cells in scleroderma

45
Q

what are 3 examples of vasoactive mediators that can cause myocardial vascular contraction (vasospasm)?

A

cocaine, epinephrine, and norepinephrine

46
Q

what is Takotsubo cardiomyopathy?

A

“broken heart syndrome”- severe emotional distress can cause an overwhelming release of catecholamines stimulating increased contractility and constriction of coronary arteries–> can lead to ischemic cardiomyopathy

47
Q

what are varicose veins?

A

abnormally dilated, twisted veins which occur from prolonged increased intraluminal pressure leading to incompetence of venous valves and dilation of the veins

48
Q

what can varicose veins lead to?

A

stasis, congestion, thrombus, edema, pain, and ischemia of overlying skin (stasis dermatitis)

49
Q

what does portal hypertension lead to?

A

esophageal varices, splenomegaly, hemorrhoids, and caput medusae

50
Q

what is the most common cause of superior vena cava syndrome?

A

intrathoracic malignancy–> bronchogenic lung carcinoma and lymphoma

51
Q

how does superior vena cava syndrome present?

A

marked dilation of veins of head, neck, chest wall, arms with cyanosis; facial swelling, neurologic manifestations, respiratory distress

52
Q

what is the most common cause of inferior vena cava syndrome?

A

neoplasms- especially hepatocellular carcinoma and renal cell carcinoma

53
Q

how does inferior vena cava syndrome present?

A

lower extremity edema and distention of the superficial collateral veins of the lower abdomen

54
Q

what is thrombophelitis?

A

a venous thrombosis and inflammation

55
Q

what cases are paradoxical emboli seen?

A

ventricular septal defect, PDA, and AV malformations

56
Q

lymphangitis is usually caused by what?

A

beta-hemolytic streptococcus

57
Q

what is a cause of primary lymphedema?

A

congenital defect- familial milroy disease (lymphatic agenesis)

58
Q

what is peau d’orange?

A

in breast cancer, when draining lymphatics fill with tumor, one can see distinctive pitting or dimpling texture

59
Q

What is vascular ectasias? and what are 2 examples?

A

local dilation of a blood vessel; nevus simplex and port-wine stain

60
Q

what is sturge-weber syndrome?

A

rare disorder characterized by a facial port wine stain with associated additional capillary venous malformations that affect the brain and the eye

61
Q

what is a prototypical clinical presentation of sturge-weber syndrome?

A

a large facial telangiectasia in a child with mental deficiency

62
Q

what causes sturge-weber syndrome?

A

somatic mutations in fetal ectodermal tissue causing inappropriate maturation of blood vessel formation- so its not heritable

63
Q

when are spider telangiectasias commonly seen?

A

in cases of increased circulating estrogen–> pregnancy or liver disease

64
Q

what is osler-weber-rendu syndrome?

A

an autosomal dominant disorder in which numerous telangiectasias and AV malformations form throughout the body

65
Q

what mutation is associated with osler-weber-rendu disease?

A

mutation in TGF-beta signaling pathway

66
Q

what occurs when the TGF-beta pathway is mutated?

A

abnormal blood vessel formation in the skin, mucous membranes, and organs

67
Q

what is a hemangioma?

A

common, benign vascular tumor of children and adults

68
Q

when internal involvement of hemangiomas is present, where are they likely found?

A

liver

69
Q

what is a cavernous hemangioma?

A

an irregular dilated vascular channel making a lesion with an indistinct border; more likely to involve deep tissue and more likely to bleed

70
Q

what is the histologic appearance of a cavernous hemangioma?

A

it has enlarged vascular spaces filled with blood

71
Q

what is a glomus tumor?

A

a mesenchymal tumor composed of modified smooth muscle arising from glomus body responsible for thermoregulation

72
Q

what are the characteristics of a glomus tumor?

A

bluish tumor with a predilection for the subungal location under the fingernail; painful and gets worse with temperature changes

73
Q

what are the 2 principal types of lymphangioma?

A

1) simple (capillary) lymphangioma and 2) cavernous lymphangioma

74
Q

what is the key difference between hemangiomas and capillary lymphangiomas?

A

in capillary lymphangiomas, the dilated spaces do not contain RBCs

75
Q

what is a cavernous lymphangioma?

A

thought to arise from a congenital malformation where the lymphatics fail to communicate with the venous system

76
Q

what are cavernous lymphangiomas associated with?

A

turner syndrome

77
Q

What is bacillary angiomatosis?

A

a reactive vascular proliferation to gram negative bartonella bacilli- occurs in immunocompromised

78
Q

what are the microscopic features of bacillary angiomatosis?

A

lobular proliferation of capillaries and ectatic vessels lined by endothelial cells typically with background inflammatory cells; bacteria on silver stain

79
Q

what is kaposi sarcoma?

A

an angioproliferative disorder caused by HHV 8

80
Q

what are the 4 types of kaposi sarcoma?

A

classic, endemic (africa), iatrogenic, and AIDS

81
Q

all 4 subtypes of kaposi sarcoma can show 3 distinct stages on the skin- what are they?

A

1) patch 2) plaque 3) tumor

82
Q

what are 3 important risk factors for angiosarcoma?

A

1) liver angiosarcoma: arsenic, thortrast, and PVC production 2) lymphedema- s/p axillary lymph node dissection–> lymphgosarcoma 3) radiation for carcinoma

83
Q

what do angiosarcomas stain for?

A

CD31 or CD34