L21- CVS Pathology III

Question 1

Valvular disease can be (1) or (2) in nature / development. Valvular disease results in either (3) or (4).

Answer 1

1/2- congenital, acquired
3- stenosis: failure to open completely, impeding forward flow
4- incompetence, regurgitation, insufficiency: failure of valve to close completely, allows reverse flow

Question 2

acquired valve stenosis is the consequence of (1) and leads to (2) then (3)

Answer 2

1- chronic injury
2- fibrosis
3- calcification

Question 3

acquired valve regurgitation is the consequence of (1) or (2) and can exist in a(n) (3) or (4) duration

Answer 3

1- intrinsic valve disease
2- damage to supporting structures
3/4- acute or chronic

Question 4

list the signs and symptoms of mitral stenosis

Answer 4

Sxs: dyspnea (pulmonary edema), fatigue, hemoptysis (blood in sputum)

Signs: late low pitch diastolic murmur, crepitations in lung

Question 5

list the signs and symptoms of mitral regurgitation

Answer 5

Sxs: dyspnea (pulmonary edema), palpitations, fatigue

Signs: pansystolic murmur radiating to axilla

Question 6

list the signs and symptoms of aortic stenosis

Answer 6

Sxs: angina, syncope, CHF

Signs: ejection systolic murmur loudest at base and radiates to neck after S1

Question 7

list the signs and symptoms of aortic regurgitation

Answer 7

Sxs: volume overload LHF

Signs: bounding pulses, early diastolic murmur, displaced apex beat

Question 8

list the 5 common acquired valvular diseases

Answer 8

• rheumatic heart disease
• calcific aortic stenosis
• mitral valve disease
• endocarditis
• prosthetic valve disease

Question 9

Rheumatic fever occurs as a result of (1) and mostly in (2) countries.

Answer 9

1- GAS infection (pharyngitis), about 3 wks after (3% of strep patients)
2- developing countries, economically depressed areas

Question 10

In rheumatic fever, Igs are produced against (1) which will cross react with (2) areas in the body leading to (3) in those areas.
-(4) list the evidence that supports this hypothesis

Answer 10

1- M proteins (on GAS)
2- heart, joints, other tissues
3- inflammation
4- elevated ASO, anti-DNAase titers, Strep absent from lesions, Sxs develop 2-3 wks post-infection

Question 11

list the 3 morphologies of acute rheumatic fever in the heart

Answer 11

Pancarditis: inflammation in all three layers of the heart

1) myocarditis
2) endocarditis (verrucous)
3) pericarditis (fibrinous pericarditis)

Question 12

myocarditis is mostly evident by the presence of the following histologically….

Answer 12

• Paravascular Aschoff Bodies (in all 3 layers of heart): central zone of eosinophilic matrix infiltrated by T-cells, plasma cells, activated macrophages w/in CT of heart
• Anitschkow cells: wavy ribbon like chromatin (caterpillar cells)

Question 13

endocarditis is mostly evident by the presence of the following histologically….

Answer 13

(verrucous)

• edematous and thickened valves with foci of fibrinoid necrosis
• multiple tiny 1-2 mm wart-like vegetations along lines of mitral valve closure (no effect on function)

Question 14

describe acute rheumatic fever morphology in other places besides the heart

Answer 14

-Joints: chronic inflammatory infiltrate, edema in joints and periarticular soft tissues
-Erythema Marginatum: maculopapular rash
-Skin Nodules
(chorea)

(JONES: joints, ocular, nodules (skin), erythema marginatum, Sydenham chorea)

Question 15

chronic rheumatoid fever usually involves (1) valves more than (2) valves

Answer 15

1- mitral, aortic

2- tricuspid, pulmonary

Question 16

chronic mitral valvulitis is mostly evident by the presence of the following histologically….

Answer 16

(more frequent)

• irregular fibrous thickening and calcification of leaflets
• fusion of commissures and shortening of chordae tendinae => fixed narrow opening
• mitral stenosis and regurgitation

Question 17

chronic aortic valvulitis is mostly evident by the presence of the following histologically….

Answer 17

• thickened, firm cusps adherent to each other

- valve orifice is reduced to rigid, triangular channel

Question 18

describe the clinical features of acute rheumatic fever (besides timing of onset with GAS pharyngitis)

Answer 18

• Arthritis: migratory, large joints
• Carditis, pericardial friction rub, weak heart sounds, CHF
• Chorea: involuntary, purposeless, rapid movements
• Skin Changes

Question 19

describe the clinical features of chronic rheumatic fever

Answer 19

Chronic Rheumatic Carditis:

• valvulitis, M > A > T > P murmurs
• cardiac hypertrophy and dilation
• CHF
• arrhythmias
• infective endocarditis

Question 20

Jones Criteria for Rheumatic Fever diagnosis

Answer 20

(2 major OR 1 major, 2 minor)
Major: i) pancarditis, ii) subcutaneous nodules, iii) sydenham chorea, iv) migratory polyarthritis, v) erythema marginatum

Minor: fever, arthralgia, elevated ESR

AND preceding Strep, infection

Question 21

Ca deposition will affect the (1) valve by depositing in the (2) of the valve, causing (3). It usually occurs in one of the following three situations: (4), (5), (6)

Answer 21

1- aortic
2- cusps, valve ring
3- stenosis / narrowing of aortic valve lumen

4- elderly, degenerative process (90% > 65 y/o)
5- congenital bicuspid aortic valve (1% of population, 40-50 y/o)
6- scarred result from rheumatic fever

Question 22

Calcific aortic stenosis is the result of (dystrophic/metastatic) calcification. The leaflets will appear (2). The deposits are evident behind (3) and extending to (4). There is sometimes (5), a predisposition, but (6) is also usually evident as a result.

Answer 22

1- dystrophic
2- rigid, deformed (irregular calcified masses)
3- valve cusps
4- into sinus of Valslava –> coronary ischemia
5- congenital bicuspid aortic valve
6- marked LVH

Question 23

describe the clinical features of calcific aortic stenosis

Answer 23

• Angina pectoris: inc requirements of hypertrophic myocardium
• Syncope: poor cerebral perfusion
• Death via CHF or arrhythmia

Question 24

Mitral valve prolapse is usually discovered in (1) age group and is more common in (males/females).
There exists an intrinsic defect in (3) and can arise from as a complication from (4) hereditary disorder.

Answer 24

1- 20-40 y/o
2- females (7x)
3- connective tissue
4- Marfan Syndrome

Question 25

In mitral valve prolapse the cusps will appear (1) and the valve leaflet will (2). The (3) are usually elongated and fragile, leaving (4) as a possibility. (5) may be dilated allowing (6). The tricuspid valve is involved in (7)% of cases.

Answer 25

``` 1- soft, puffed up, rubbery 2- balloon into LA during systole 3- chordae tendinae 4- rupturing (severe cases) 5- mitral annulus 6- regurgitation 7- 20-40% ```

Question 26

describe histological appearance of mitral valve prolapse

Answer 26

- thinning of fibrosa layer of valve + expansion of spongiosa layer - excessive loose, edematous, faintly basophilic ground substance w/in middle layer of valve leaflets and chordae (myxomatous degeneration)

Question 27

describe the clinical presentation of mitral valve prolapse

Answer 27

* most are asymptomatic - palpitations, fatigue / atypical chest pain - midsystolic click (abrupt tension on leaflets and chordae as valve closes) - Severe complications (3%): mitral regurgitation, CHF, infective endocarditis, ventricular arrhythmia (=> SCD), thromboemboli => stroke - Rarely scoliosis, high arched palate

Question 28

Endocarditis in an infection of (1) or (2) resulting in formation of (3) termed (4) that will (5) the underlying cardiac tissue.

Answer 28

``` 1- cardiac valves 2- mural surface 3- adherent, bulky mass of thrombotic debris and organisms 4- vegetations 5- destroy ```

Question 29

Acute Endocarditis: (1) original valve status (2) extent of destruction (3) causational organisms (4) mortality

Answer 29

1- normal valve 2- highly destructive, fulminent (severe, sudden) 3- highly virulent organisms 4- high mortality even with antibiotics or surgery

Question 30

Subacute Endocarditis: (1) original valve status (2) extent of destruction (3) causational organisms (4) mortality

Answer 30

1- abnormal valve 2- low destruction 3- low virulent organisms 4- most recover with appropriate therapy

Question 31

list the predisposing factors for infective endocarditis

Answer 31

- pre-existing cardiac abnormality: rhematic valvular disease, mitral prolapse, congenital defects, degeneratice calcific aortic stenosis - prosthetic heart valves (10-20% of cases) - IV drug abuse (tricuspid valve) - transient bacteremia (dental procedures, urinary cath., endoscopy

Question 32

(1) is the most common causative organism of endocarditis (2) is seen IV drug abuse (3) is associated with colon cancer (4) is another popular group

Answer 32

1- Strep. Viridans 2- S. aureus 3- Strep. Bovis 4- HACEK (hemophilus, actinobacillus, cardiobacterium, eikenella, kingella)

Question 33

list the Hemodynamic factors and Adherence properties of organisms involved in infective endocarditis

Answer 33

Hemo: abnormal blood flow across damaged valve --> endothelial injury --> focal deposition of platelets, fibrin Adherence: fibronectin, adhesion factors like polysaccharides

Question 34

Infective endocarditis usually affects (1) valves, and (2) valves in IV drug abuse.

Answer 34

1- mitral, aortic | 2- tricuspid

Question 35

(1) are the hallmarks of infective endocarditis, they are described as (2) lesions containing (3). (1) are prone to (4), which can lead to (5).

Answer 35

1- vegetations 2- friable, bulky, potentially destructive lesions 3- fibrin, inflammatory cells, bacteria 4- embolization 5- septic infarcts, abscesses, mycontic aneurysms

Question 36

describe the gross and microscopic appearance of subacute endocarditis

Answer 36

Gross: vegetations are less friable, less valve destruction, ring abscesses are uncommon Micro: granulation tissue, fibrosis, calcification, chronic inflammatory infiltrate

Question 37

describe the clinical appearance of infective endocarditis

Answer 37

- fever (PUO- pyrexia of unknown origin) - clubbing of fingers, splinter hemorrhages under nail beds - Osler nodes: tender subcutaneous nodules - Janeway lesions: nontender maculae on palms/soles - Roth spots: retinal hemorrhages w/ clear center -systemic embolism: stroke, distal organ infarcts

Question 38

compare acute and subacute bacterial endocarditis

Answer 38

Acute: - high grade fever, chills - new cardiac murmur - features of septicemia Subacute: - low grade fever, malaise - changing cardiac murmur - weight loss - splenomegaly

Question 39

diagnostic requirements for infective endocarditis

Answer 39

- duke criteria - repeated blood cultures (aerobic, anaerobic organisms) - echocardiography

Question 40

describe treatment for infective endocarditis

Answer 40

difficult because valves are avascular, but longterm, IV antibacterial therapy is required

Question 41

(1) endocarditis occurs in SLE patients and is characterized by (2) that result from (3). (4) and (5) are other special features.

Answer 41

1- Libman-Sacks 2- sterile vegetations 3- IC (immune complex) deposition 4- special predilection for lines of valve closure 5- able to extend towards ostia (opening) of coronary arteries

Question 42

(1) endocarditis occurs in debilitated patients, and has (2) as an alternate name. It is characterized by (3) and is associated with (4). It will present as (5) and (6) are the possible complications.

Answer 42

1- nonbacterial thrombotic endocarditis (NBTE) 2- marantic endocarditis 3- sterile thrombi on leaflets (previously normal valves) 4- endothelial abnormalities, hypercoagulable states, adenocarcinomas 5- asymptomatic 6- embolism, infective endocarditis

Question 43

describe the gross and microscopic appearance of NBTE

Answer 43

(nonbacterial thrombotic endocarditis) Gross: multiple 1-5 mm nodules along closure line, non-invasive and destructive vegetations, commonly Mitral Valve Micro: eosinophilic (fibrin) nodules with delicate layer of aggregated plateletsloosely attached to the cusp, no inflammation, no fibrosis

Question 44

Describe the complications of prosthetic valve disease

Answer 44

Mechanical valve: thrombo-embolism, life long anticoagulation / bleeding, infective endocarditis, hemolysis, inadequate healing (paravalvular leak) Tissue valve / Bioprostheses (porcine, bovine, human; no anticoagulation needed): less durable => matrix deterioration, rigidity, calcification --> stenosis; infective endocarditis, inadequate healing