CV lecture 3- Cardiac Valvular Disease and Vasculitis Flashcards

1
Q

Acquired mitral valve stenosis is usually caused by what?

A

usually due to chronic (recurrent) rheumatic valvular disease

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2
Q

what is mitral valve stenosis?

A

failure of a valve to open completely, obstructing forward flow

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3
Q

what strain of bacteria is usually responsible for acquired stenosis?

A

Group A β-hemolytic Streptoccocal pharyngitis

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4
Q

mitral valve stenosis usually effects what group of people?

A

children

only 20% of cases are in adults

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5
Q

what are the clinical features of Acute rheumatic fever (ARF)?

A

arthritis (often polyarthritis)
carditis
erythema marginatum (skin rash)
subcutaneous nodules

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6
Q

The myocarditis caused by ARF is characterized microscopically by _______ bodies

A

Aschoff

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7
Q

what are the 3 types of Rheumatic Carditis?

A

Pericarditis – fibrinous

Endocarditis – sterile vegetations

Myocarditis – Aschoff bodies

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8
Q

what are the microscopic characteristics of Aschoff bodies?

A
  • mononuclear cells and fibroblasts

- form of granulomatous inflammation

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9
Q

Recurrent bouts of acute rheumatic fever lead to __________ and ____________

A

fibrosis

and

mitral valve stenosis

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10
Q

______________ refers to a valve that fails to close completely, allowing backflow of blood

A

Regurgitation (insufficiency)

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11
Q

what is Mitral valve prolapse?

A

a condition in which the leaflets balloon into the left atrium during left ventricular contraction (systole)

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12
Q

what are the THREE causes of mitral valve regurgitation?

A

1) Ischemic heart disease
2) Infective endocarditis
3) Floppy mitral valve (severe MV prolapse)

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13
Q

floppy mitral valve (sever MV prolapse) can be isolated or part of ___________ syndrome

A

Marfan syndrome

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14
Q

what are the characteristics of floppy mitral valve (Sever MV prolapse)?

A
  • the valve cusps are large and microscopically show fragmentation
  • separation and loss of collagen
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15
Q

_____________ degeneration is the name given when mitral valves separate and lose collagen

A

myxomatous degeneration

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16
Q

what are the possible complications of floppy mitral valve syndrome?

A
  • MV regurgitation
  • endocarditis
  • thromboemboli
  • sudden death (rare)
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17
Q

_____________ Stenosis is caused by fibrosis and calcification

A

Aortic Valve Stenosis

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18
Q

what is the effect on the heart from fibrosis and calcification during aortic valve stenosis?

A

reduce the valve cusp mobility

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19
Q

what are the 3 types/causes of Aortic valve stenosis?

A
  • Chronic rheumatic valvular disease
  • Degenerative (senile)
  • Congenital bicuspid valve
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20
Q

which form of Aortic valve stenosis is more common in younger people?

A

Congenital bicuspid valve

  • much younger initial onset (40-50 years)
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21
Q

what are the 3 causes of Aortic Valve Regurgitation?

A
  • Valve cusp destruction
  • Weakened valve cusps
  • Dilation of the aortic root
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22
Q

what 2 conditions can cause valve cusp destruction (and lead to Aortic valve regurgitation)?

A

infectious endocarditis

rheumatic heart disease

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23
Q

myxomatous degeneration (from Marfan syndrome) will lead to the weakening of what structure?

(causes aortic valve regurgitation)

A

Weakened valve cusps

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24
Q

A Dilation of the ________ can occur as a result of degeneration of the media of the aorta

A

dilation of the aortic root

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25
Q

name the predisposing factors for infective endocarditis:

A
  • intracardiac shunts
  • valvular disease
  • prosthetic valves (10-20% of all IE cases)
  • IV drug abuse
  • immune suppression
  • diabetes mellitus
26
Q

what 3 factors have been identified in the pathogenesis of infective endocarditis?

A

1) endocardial/endothelial injury (blood flow)
2) fibrin thrombi
3) organisms in the blood (sepsis)

27
Q

list the clinical signs of infective endocarditis:

A

Fever

Fatigue

Anemia

Myalgia/arthralgia

Splinter hemorrhages; Roth spots (retinal hemorrhages)

Heart murmur

28
Q

where are “splinter hemorrhages” found in patients with infective endocarditis?

A

in the nail beds

29
Q

which form of infective endocarditis affects previously normal heart valves?

which form effects previously abnormal valves?

A

normal valves- ACUTE infective endocarditis

previously abnormal valves- SUBacute infective endocarditis

30
Q

what are some complications of infective endocarditis?

A

1) valvular regurgitation
2) rupture of chordae tendineae
3) contiguous spread of infection
4) thromboembolism – “splinter hemorrhages” of fingernails
5) septic emboli with abscesses

31
Q

what are the causes of vasculitis?

A
***Infection***
Immunologic mechanisms
Radiation
Trauma
Caustic substances
Unknown
32
Q

what are the 2 diseases that lead to “large vessel” vasculitis?

A
Giant cell (temporal)
Takayasu arteritis
33
Q

_________________ and ____________ are both diseases that cause “medium vessel” vasculitis

A

Polyarteritis nodosa

and

Kawasaki disease

34
Q

name the 2 diseases that result in small vessel vasculitis

A

Microscopic polyarteritis

Wegener granulomatosis

35
Q

what are the 4 factors involved in the pathogenesis of vasculitis?

A

1) Immune complexes; circulating or may form in-situ
2) Antineutrophilic cytoplasmic antibodies (ANCA)
3) Anti-endothelial cell antibodies
4) Cell-mediated immune mechanisms

36
Q

which Antineutrophilic cytoplasmic antibody (ANCA) is associated with microscopic polyarteritis?

A

Anti-myeloperoxidase (anti-MPO)

  • perinuclear localization
37
Q

which Antineutrophilic cytoplasmic antibody (ANCA) is associated with Wegener granulomatosis?

A

Anti-proteinase-3 (anti-PR3)

  • diffuse cytoplasmic distribution
38
Q

anti-endothelial cell antibodies are important in the pathogenesis of what disease?

A

Kawasaki disease

a medium vessel vasculitis

39
Q

what are the clinical characteristics of Giant Cell (temporal) arteriosis?

A
rare before age 50
fever
weight loss
headache
visual disturbances

pain and tenderness over temporal artery

polymyalgia rheumatica

40
Q

what are the pathological characteristics of Giant cell arteriosis?

A

Granulomatous inflammation

Intimal proliferation / fibrosis

41
Q

T/F: the etiology (cause) of both Giant cell, and Takayasu arteriosis have both been identified

A

FALSE

the etiology of both is unknown

42
Q

________________ is also called the “pulseless disease”…. what causes this?

A

Takayasu Arteritis

  • patients have weak arm pulses
43
Q

what is the pathology of Takayasu arteritis?

A

1) Involves aortic arch and branches
2) Intimal fibrosis
3) Granulomatous inflammation with fibrosis

(NOTE: numbers 2 & 3 are the same as giant cell vasculitis)

44
Q

what are the clinical manifestations of Polyarteritis Nodosa?

A

1) acute-relapsing-chronic progression
2) fever
3) weight loss
4) hematuria
5) renal failure
6) hypertension
7) abdominal pain
8) melena

45
Q

describe the pathology for Polyarteritis Nodosa

A
  • Haphazard and segmental involvement of medium and small muscular arteries
  • Kidney > liver > heart > GI
  • Fibrinoid necrosis, PMN’s
  • Thrombosis, aneurysms
  • Heal by fibrosis
46
Q

what sites in the body are usually involved with Polyarteritis Nodosa?

A

Usual sites of involvement are:

kidneys (85%),
heart (75%),
liver (65%),
GI tract (50%).

47
Q

________________ is also known as mucocutaneous lymph node syndrome

A

Kawasaki disease

48
Q

what is the etiology of Kawasaki disease?

A

anti-endothelial antibody triggered by viral infection

It is suspected that a viral infection triggers a hypersensitivity reaction. AKA: mucocutaneous lymph node syndrome

49
Q

what age group is effected by Kawasaki disease?

A

Infants & young children

50
Q

name the clinical features of Kawasaki disease:

A

fever
mucous membrane erythema (eyes/mouth)
skin rash
cervical lymphadenopathy

usually self-limited

51
Q

1-2% of patients with Kawasaki disease will die due to __________________

A

coronary artery vasculitis

52
Q

what is the etiology/cause of Microscopic Polyangiitis?

A

antigen-antibody complexes

Ag-Ab complexes/MPO-ANCA

53
Q

what are the clinical signs for microscopic polyangiitis?

A

skin rash, other organs

54
Q

microscopic polyangiitis may be caused by what “precipitating” conditions?

A

May be precipitated by:
drugs
microorganisms
foreign proteins or tumor proteins.

55
Q

what is the pathology of microscopic polyangiitis?

A

Involves arterioles, capillaries, venules.

Fibrinoid necrosis

  • small vessel vasculitis WITHOUT necrotizing granulomatous inflammation
56
Q

what is the etiology, and the clinical signs, for Wegener’s Granulomatosis?

A

Etiology: neutrophil-related endothelial damage mediated by PR3-ANCA

Clinical – sinusitis, pneumonitis, renal failure, glomerulonephritis

57
Q

what is the pathology of Wegener’s Granulomatosis?

A
  • Affects kidneys, upper and lower respiratory tract
  • Necrotizing granulomas
  • Vasculitis with fibrinoid necrosis
58
Q

give the etiology and clinical signs for Thromboangiitis Obliterans (Buerger Disease):

A

Etiology – endothelial injury from substance in cigarette smoke

Clinical – cigarette smoking, < 35 years, pain of extremities, ischemic ulcers, gangrene

59
Q

what are the pathological characteristics of Thromboangiitis Obliterans (Buerger Disease)?

A

Vasculitis with thrombosis

60
Q

what are the pathological characteristics for Dissecting Aortic Hematomas?

A

A) intimal tear - split between mid & outer third of the
media

B) media may be normal or have degeneration

61
Q

what are the complications AND predisposing factors for Dissecting aortic hematomas?

A

Complications:

1) rupture – hemorrhage
2) branch obstruction

Predisposing factors:

1) hypertension
2) connective tissue disorders (Marfan’s)

62
Q

which form of vasculitits is MUCH more common in women than in men?

A

Takayasu arteriosis

  • most common in women under 40 yrs old