Patho: Valvular Heart Diseases

Question 1

Determine if the following affect the function of the semilunar valves or the AV valves:

1) leaflets
2) cusps
3) cuspal attachments
4) tendinous cords
5) papillary muscles

Answer 1

1) AV
2) semilunar
3) semilunar
4) AV
5) AV

Question 2

Histology

Layer at the outflow: ___
Proteoglycan rich: ___
Rich in elastin for prompt valve closure: ___

Answer 2

Fibrosa - dense, collagenous, maintain structure
Spongiosa - lightly-staining
Ventricularis/Atrialis - inflow; has collagen for mechanical integrity

Question 3

Identify this cell:

Most abundant cell type in valves
Synthesize ECM
Produce matrix-degrading enzymes
Produce inhibitors

Answer 3

Interstitial cells

Note: blood supply through diffusion since valves are thin

Question 4

Diseases and their type of acquired valvular diseases

1) rheumatic heart disease: ___
2) calcific aortic stenosis: ___
3) mitral valve prolapse

Answer 4

1) fibrotic thickening
2) nodular calcification
3) damage to collagen

Question 5

Identify which type of nodular calcification:

1) progressive calcification, has a midline raphe
2) most common; etiologic cause is age-associated wear-and-tear; deposition of hydroxyapatite
3) degenerative calcific deposits at fibrous annulus

Answer 5

1) Calcific Stenosis of Congenitally Bicuspid Aortic Valve (BAV) - predisposed to other cardiac conditions e.g. aortic dilation & dissections, infective endocarditis, aortic insufficiency
2) Calcific Aortic Stenosis - mounded calcified nodules on the outflow surface; commissural fissure not seen
3) Mitral Annular Calcification - complications: nodules can provide site of thrombus formation; arrhythmia

Question 6

Most frequent cause of pure, isolated mitral regurgitation

Answer 6

Mitral Valve Prolapse/Myxomatous Degeneration of the Mitral Valve - leaflet/s ballon back during systole

Question 7

T/F: In mitral valve prolapse, there is an audible click associated with late systolic murmur.

Answer 7

True: mid-systolic, non-ejection click

Question 8

Involves myxomatous degeneration of the spongiosa layer

A) calcified aortic stenosis
B) rheumatic heart disease
C) mitral stenosis
D) mitral valve prolapse

Answer 8

Answer is D.
increased deposition of highly sulfated hydrophilic matrix → associated marked thickening of the spongiosa layer → associated attenuation of the collagenous fibrosa layer → compromised structural integrity

Question 9

T/F: MVP is not a predisposing factor for thrombus formation and infective endocarditis.

Answer 9

False

Question 10

Acute, immunologically mediated, multisystem inflammatory disease involving fibrotic thickening. Usually occurs after a GAS pharyngitis episode.

Answer 10

Rheumatic fever - pathogenesis involves antibody binding and T-cell mediated reaction

Question 11

JONES criteria: _____

Need 2 major manifestation os 1 major and 2 minor (fever, arthralgia, elevated levels of acute phase reactants)

Answer 11

J - joints: migratory polyarthritis
O - looks like the heart -> pancarditis
N - (subcutaneous) nodules
E - erythema marginatum of the skin
S - Syndenham chorea (involuntary, rapid, purposeless movement

Question 12

Acute rheumatic carditis: Identify which carditis (pan-, endo-, etc.)

1) inflammation in all layers
2) involves pericardial effusion
3) involves Aschoff nodules
4) fibrinoid necrosis within cusps & tendinous chords
5) can cause cardiac dilation
6) usually involves left-sided valves

Answer 12

1) pan-
2) peri- (with fibrinoid pericarditis)
3) myo-
4) endo-
5) myo-
6) endo-

Question 13

Number one cause of rheumatic mitral stenosis; “fish mouth” or “buttonhole” stenosis

A) acute rheumatic carditis
B) fibrinoid pericarditis
C) chronic rheumatic carditis
D) rheumatic fever

Answer 13

Answer is C.

Chronic rheumatic carditis: clinically manifests years or decades after initial episode of rheumatic fever

Question 14

Microbial infection of the heart/mural endocardium leading to vegetation; often associated with destruction of underlying cardiac tissues

Answer 14

Infective endocarditis

Note: Usually for IE, there’s an infection somewhere else in your body and then the microorganisms get into the heart and adhere to the thrombus

Question 15

T/F: The usual underlying condition prior to aortic stenosis is heart diseases that manifests with turbulence, trauma, and stasis.

Answer 15

F. Aortic stenosis -> infective endocarditis

Question 16

Traditional classification of IE:

1) insidious infection of a structurally abnormal valve by low virulent organism
2) infection from a previously healthy individual, through a highly virulent organism

Answer 16

1) subacute IE

2) acute IE - rapidly produces necrotizing destructive lesions

Question 17

Most common agent in prosthetic valve IE:
A) Streptococcus viridans
B) Staphylococcus epidermidis
C) Eikenella
D) Enterococci

Answer 17

Answer is B.

Question 18

What is EHACEK?

Answer 18

Drugs. Enterococci, hemophilus, actinobacillus, cardiobacterium, eikenella, kingella, gram-negative bacilli, fungi

Question 19

Hallmark feature of IE (gross morphology): ___

Answer 19

vegetations on heart valves

Question 20

Role of lab diagnostic tests

1) Determines proper antibiotic therapy, confirms presence of microorganism in the circulation
2) For monitoring response to treatment

Answer 20

1) Blood culture

2) Inflammatory markers

Question 21

Mnemonic for systemic complications of IE: FROM JANE

Answer 21

Fever, roth spots, osler nodes, murmur, Janeway lesions, anemia, nail-bed hemorrhage, emboli

Question 22

No associated tissue destruction & resulting complications: which type of IE?

Answer 22

Nonbacterial thrombotic endocarditis (NBTE)

IE involves tissue destruction

Question 23

Which type of endocarditis: immune complex deposition in the valves results in complement activation and recruitment of FC-receptor-bearing cells (neutrophils, macrophages)

Answer 23

Libman-Sacks Disease (Endocarditis of SLE)

Question 24

Syndrom marked by flushing, diarrhea, dermatitis, bronchoconstriction

Typically occur when there is a massive hepatic metastatic burden

Answer 24

Carcinoid syndrome - caused by bioactive compounds released by carcinoid tumors

Carcinoid heart disease

Question 25

Based on gross morphology/histology, identify type of valvular disease:

1) Glistening, white intimal plaque-like thickening of endocardial surfaces
2) Bland thrombus; thrombotic vegetations
3) Organized inflammation; post-inflammatory neovascularization

Answer 25

1) carcinoid heart disease
2) nonbacterial thrombotic endocarditis
3) chronic rheumatic carditis