Cardiac Valvular Disease and Vasculitis Flashcards by Rachel Eifert

What are the five discussed valvular heart diseases?

Mitral Valve Stenosis
Mitral Valve Regurgitation
Aortic Valve Stenosis
Aortic Valve Regurgitation
Infective Endocarditis

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What is Mitral Valve Stenosis?

failure of a valve to open completely, obstructing forward flow

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Mitral valve stenosis is usually due to ________.

Chronic (recurrent) Rheumatic Valvular Disease

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What is Acute Rheumatic Fever?

a systemic disease, usually in children, which follows a group A beta-hemolytic streptococcal pharyngitis

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True or False: Rheumatic Fever (RF) produces myocarditis, pericarditis, arthralgia, or arthritis.

True

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Myocarditis associated with RF is characterized microscopically by ______ bodies which are collections of _________ and ________.

Aschoff
mononuclear inflammatory cells
fibroblasts

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What will recurrent bouts of RF eventually lead to?

severe fibrosis and calcification of the mitral valve (and possibly other valves)

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True or False: RF is thought to be due to the production of antibodies against the streptococcal bacteria that cross react with various antigens in the heart, joints and other sites.

True

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What is “regurgitation?”

insufficiency

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What is valve regurgitation?

a valve that fails to close completely, allowing backflow of blow

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Mitral Valve Regurgitation may be caused by a variety of conditions including ______ and ______.

IHD

endocarditis

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What is Mitral Valve Prolapse?

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

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Mild prolapse occurs in _____ % of the general population.

5-10

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True or False: Mild Mitral Valve Prolapse is very common.

True

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True or False: Mitral Valve Prolapse usually progresses to valvular regurgitation.

False, it usually does not. “Severe Prolapse” may be associated with regurgitation

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True or False: Some Mitral Valve patients also experience chest pain and palpitations.

True

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Severe Mitral Valve Prolapse is also called _______

Floppy Mitral Valve

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Describe the valve cusps in severe mitral valve prolapse.

-the valve cusps are large and microscopically show fragmentation, separation and loss of collagen (Myxomatous Degeneration)

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Floppy mitral valve may be part of a systemic connective tissue disorder, such as ______.

Marfan Syndrome

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In Aortic Valve Stenosis, _____ and ______ reduce the valve cusp mobility.

fibrosis

calcification

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Bicuspid aortic valve is a common congenital malformation and these valves are predisposed to calcification and fibrosis, starting at about ______ of age.

40 years

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What are the mechanisms of Aortic Valve Regurgitation?

valve cusp destruction (endocarditis)
myxomatous degeneration
dilation of the aortic root

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Infective endocarditis is usually caused by _____.

a BACTERIAL infection of the heart valve

*may also be fungus or unusual infections

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What are predisposing factors of infective endocarditis?

abnormal heart valves
prosthetic valves
intravenous drug use
intracardiac shunts
diabetes
immunosuppression

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What are the three factors that have been identified as having importance in the pathogenesis?

1. endocardial or endothelial injury due to abnormalities in blood flow 2. fibrin thrombi 3. organisms in the blood

What are the clinical manifestations of Infective endocarditis?

``` fever heart murmur fatigue anemia arthralgia myalgia splinter hemorrhages Roth spots ```

What are Roth Spots?

retinal hemorrhages (not absolutely specific to IE)

True or False: Chordae tendineae could rupture due to complications with Infective Endocarditis.

True

What is the difference between acute and subacute endocarditis?

Acute: short duration, virulant organism, large vegetations, previously normal valve, prominent tissue destruction Subacute: Longer duration, low virulence, small vegetations, previously abnormal valve, less tissue destruction

Vasculitis may be caused by ________, mechanical trauma, toxins, caustic substances, radiation, or immune complexes.

infection

What are the three classifications of vasculitis?

Large vessel medium vessel small vessel

What are the two forms of Large Vessel Vasculitis?

``` Giant Cell (temporal) Arteritis Takayasu Arteritis ```

What are the two forms of Medium Vessel Vasculitis?

Polyarteritis Nodosa | Kawasaki Disease

What are the two forms of Small Vessel Vasculitis?

Microscopic Polyangiitis | Wegener Granulomatosis

True or False: The etiology of Giant Cell Arteritis is unknown.

True

Giant Cell is rare at what age?

before age 50

What are the two key characteristics regarding the pathology of Giant Cell?

1. Granulomatous Inflammation with giant cells 2. Intimal Proliferation and fibrosis (destruction of elastic fibers) =narrowing of the lumen

True or False: Takayasu Arteritis affects large vessels and is more common in females.

True and True!

True or False: Takayasu may be the same disease as Giant Cell but in a younger patient.

True (think of a Kayak (taKAYAsu)...you need a BIG river to paddle down, so it is going to occur in big vessels like the aorta)

What is another name given to Takayasu? Why?

"Pulseless Disease" | causes weak pulses in the arms due to reduced bloodflow from major aortic branches

__________ is a medium vessel vasculitis that was once associated with Hepatitis B because _____% of patients had both conditions

Polyarteritis Nodosa | 30

Why would clinical presentation of Polyarteritis Nodosa be confusing?

due to involvement of multiple organ systems (Known this) ``` *sites most commonly involved: Kidneys 85% Heart 75% Liver 65% GI Tract 50% ```

__________ Disease is a medium vessel vasculitis that is also known as Mucocutatneous Lymph Node Syndrome.

Kawasaki

______% of patients with Kawasaki Disease are under the age of 4 yrs.

A child with Kawasaki will usually present with what symptoms?

skin rash* fever mucous membrane erythema

What is the etiology of kawasaki disease?

anti-endothelial antibody (?) triggered by viral infection

What are the two small vessel vasculitis diseases?

Microscopic Polyangiitis | Wegener Granulomatosis

Etiology of Microscopic Polyangiitis is mediated by ________ Ag-Ab Complex; whereas, Wegener is mediated by __________.

MPO- ANCA (M-icroscopic PO-lyarteritis "MPO") | PR3- ANCA ("PR3" stands for proteinase 3)

True or False: Microscopic Polyangiitis involves fibrinoid necrosis and leukocytoclastic vasculitis.

True

What is leukocytoclastic vasculitits?

breakdown of PMN nuclei

Wegener Granulomatosis affects _________ and kidneys.

upper/lower respiratory tract

Damage associated with Wegener is due to an abnormal expression of ______ on endothelial cell surfaces which bind to ANCA and activates _____ which will cause endothelial damage.

proteinase 3 | neutrophils

What is the pathology of Wegener Granulomatosis?

(Known this) * Necrotizing Granulomas * Vasculitis with fibrinoid necrosis (this is how it affects the respiratory tract...the air space fills with fibinoid tissue)

What is a distinguishing oral characteristic/sign of Wegener Granulomatosis?

Strawberry Gingivitis

________ is an endothelial injury from a substance in cigarette smoke.

Thromboangiitis Obliterans (Buerger Disease)

True or False: Thromboangiitis Obliterans (Buerger Disease) usually begins before age 65.

False, BEFORE AGE 35!

Thromboangiitis Obliterans causes segmental acute and chronic vasculitis mainly in _______ and with ______. What is the other name for this condition?

extremities thrombosis "Buerger Disease)

________ syndrome is a connective tissue disorder that is related to Mitral Valve Prolapse and Dissecting Aortic Hematoma.

Marfan

What is a Dissecting Aortic Hematoma?

aneurysm -longitudinal tear of the aortic media which begins in the ascending aorta and extends a variable distance proximal (toward the heart) and distal to the descending aorta

What are complications associated with Dissecting Aortic Hematoma?

severe hemorrhage from rupture (death) | branch obstruction and organ ischemia (luminal compression by the expanding hematoma)

Serious complications of Dissecting Aortic Hematomas predominately occur in _____ dissections, which therefore mandate surgical intervention.

Type A

What is the difference between Type A and Type B dissections?

Type A = proximal, ascending aorta | Type B = distal, after take-off of the great arteries

What is the difference between Pericarditis, Endocarditis, and Myocarditis?

``` Peri = fibrinous Endo = sterile vegetations Myo = Aschoff bodies (mononuclear cells and fibroblasts) ```

What is the difference between degenerative and congenital aortic valve stenosis?

Degenerative (senile) = advanced age | Congenital Bicuspid Valve = much younger onset, ~40

Mitral Valve fibrosis and calcification with valvular stenosis could be a result of __________ with Group A beta-hemolytic ________.

acute rheumatic fever (ARF) | streptococcal pharyngitis

What is the histopathological feature common with Mitral Valve Prolapse?

myxomatous degeneration

Aortic valve stenosis is caused by _____ and _____.

fibrosis and calcification

Dissecting Aortic Hematoma occurs in which layer of the vessel wall?

between the middle and outer third of the MEDIA

What does ANCA stand for?

antineutrophilic cytoplasmic antibodies