Valvular Disease

Question 1

Stenosis (valve)

Answer 1

Narrowing of a valve

Question 2

Regurgitation (valve)

Answer 2

Insufficiency of a valve causes back flow of blood

Question 3

Chorea

Answer 3

Chorea is characterized by involuntary brief, semi-directed, irregular movements that are not repetitive or rhythmic, but appear to flow from one muscle to the next.

Question 4

What is the cell origin of angiosarcoma?

Answer 4

Arise from endothelial cells

Question 5

What is the general structure of a valve?

Answer 5

Valves are lined by endothelium on both in-flow and out-flow surfaces and there is a collagenous core (3 parts – spongiosa, fibrosa, elastosa) of connective tissue

Question 6

What are the semilunar valves?

Answer 6

Aortic and Pulmonic

Question 7

What are the atrioventricular valves?

Answer 7

Tricuspid and Mitral

Question 8

What is bicuspid aortic valve?

Answer 8

Congenital malformation where the aortic valve has 2 cusps instead of 3 and the cusps are usually of unequal size.

Question 9

What may develop later on as a complication of bicuspid aortic valve?

Answer 9

Aortic stenosis may develop at age 50’s and 60’s

Question 10

What are people with bicuspid aortic valves predisposed to?

Answer 10

There is a predisposition to infective endocarditis

Question 11

Valvular Degeneration Caused by Calcification

Answer 11

Valves are subject to high repetitive mechanical stresses which leads to cumulative damage to the delicate valves and deposition of calcium phosphate minerals

Question 12

What patients are at increased risk of valve degeneration via calcification?

Answer 12

Chronic injury due to hyperlipidemia,
hypertension, inflammation may have a role in and may precede the characteristic calcification - similar to atherosclerosis but is a distinct process

Question 13

What is calcific aortic stenosis?

Answer 13

Heaped up calcified masses within the aortic cusps protrude through outflow surfaces and prevent the opening of cusps leading to stenosis.

Question 14

When does calcific aortic stenosis manifest in patients with bicuspid aortic valves? And when does it occur for those with normal valves?

Answer 14

Stenosis manifests clinically in 50’s and 60’s (stenosis may manifest at an earlier age since bicuspid valves incur greater mechanical stress than normal tricuspid valves)

If calcification affects structurally normal valves -stenosis usually manifests clinically in age 70’s and 80’s

Question 15

What is Myxomatous Degeneration of the Mitral Valve also known as?

Answer 15

Mitral Valve Prolapse

Question 16

Who is most commonly affected with mitral valve prolapse?

Answer 16

Young women

Question 17

What is the pathology of mitral valve prolapse?

Answer 17

One or both mitral valve leaflets are enlarged, redundant, rubbery - Floppy valves balloon back (prolapse) into left atrium during systole

Question 18

What happens to the mitral valve histologically in mitral valve prolapse?

Answer 18

Histologically the valve leaflets have deposition of mucoid/myxomatous material

Question 19

What is the presentation of mitral valve prolapse?

Answer 19

Majority of patients are asymptomatic. There are rare serious complications – development of infective
endocarditis, development of mitral valve insufficiency

Question 20

What is infective endocarditis?

Answer 20

Destructive inflammation of cardiac valves and endocardium - vegetations can often be seen

Question 21

What is the most common cause of infective endocarditis?

Answer 21

Bacterial infection of a valve is the most common cause

Question 22

Acute Infective Endocarditis

Answer 22

Caused by highly virulent organisms
-Staphylococcus aureus
Results in a rapid clinical course with severe destruction of valves - Affected valves are often previously normal

Question 23

Subacute Infective Endocarditis

Answer 23

Caused by low virulence organisms - alpha-hemolytic (viridans) Streptococci
Affects previously deformed valves with less valvular destruction

Question 24

What are vegetations?

Answer 24

Composed of thrombotic debris, fibrin, inflammatory cells,

microorganisms - friable which leads to embolisms

Question 25

What are some risk factors for the development of infective endocarditis?

Answer 25

i. Preexisting valvular disease
ii. Prosthetic valves
iii. Immune deficiency
iv. Diabetes mellitus
v. Intravenous drug abuse
vi. Alcoholism

Question 26

What are some major complications of infective endocarditis?

Answer 26

i. Cardiac
- Valvular insufficiency (or stenosis)
- Abscess formation
- Prosthetic valve dehiscence or paravalvular leak
ii. Embolic complications

Question 27

What is Nonbacterial Thrombotic Endocarditis also known as?

Answer 27

Marantic Endocarditis

Question 28

What are the characteristics of Marantic Endocarditis?

Answer 28

Characterized by small (1-5mm), sterile vegetations on cardiac valves.

• Vegetations are composed of fibrin, platelets
• Vegetations are non-destructive but loosely attached to valves

Question 29

What is Endocarditis of Systemic Lupus Erythematosus also known as?

Answer 29

Liebman-Sacks Endocarditis

Question 30

What are the characteristics of Liebman-Sacks Endocarditis?

Answer 30

Small (1-4mm) sterile vegetations in patients with Systemic Lupus Erythematosus

• Consequence of immune complex deposition leading to valvulitis
• Most commonly seen on mitral and tricuspid valve undersurfaces
• May be associated with marked inflammation and necrosis of valve

Question 31

What is the pathogenesis of acute rheumatic fever?

Answer 31

Acute, immunologically mediated, multisystem inflammatory disease and occurs 6-10 days after Group A (B-hemolytic) streptococcal pharyngitis due to cross reaction of antibodies.
- Antibodies directed against M proteins of strep cross-react with self-antigens in the heart

Question 32

Who is most commonly affected by acute rheumatic fever?

Answer 32

Most commonly seen in children 5-15 years old

Question 33

What is CHRONIC rheumatic fever?

Answer 33

Clinical manifestations develop years to decades after episode of acute rheumatic fever
-Manifests as valvular disease – most commonly mitral stenosis

Question 34

What are the major criteria for acute rheumatic fever?

Answer 34

• Joints with migratory polyarthritis of large joints
• ❤Carditis
• Subcutaneous nodules
• Erythema marginatum of skin
• Sydenham chorea

Question 35

What are the minor criteria for acute rheumatic fever?

Answer 35

• Fever
• Arthralgias
• Elevated acute-phase reactants

Question 36

What is a common finding with chronic rheumatic fever?

Answer 36

-Valve leaflets become thickened, fused and the leaflet thickening and fusion can results in a classic “fish
mouth” or “ buttonhole” stenosis

Question 37

What are Aschoff bodies comprised of?

Answer 37

– T lymphocytes 
– plasma cells
– macrophages
• Anitschkowcells (caterpillar cells)
– multinucleated cells

Question 38

What is pancarditis comprised of?

Answer 38

• Pericarditis
• Myocarditis
• Endocarditis

Question 39

What is carcinoid heart disease?

Answer 39

Endocrine tumors most commonly involving the gastrointestinal tract and lung which secrete bioactive products – serotonin (5-hydroxytryptamine),
kallikrein, bradykinin, histamine, prostaglandins, tachykinins that affect the heart

Question 40

How is the heart affected by carcinoid tumors and what is the most commonly affected part of the heart?

Answer 40

Affects the right side of the heart.

The endocardium and valves develop a plaque-like thickening and the pathogenesis is not entirely understood but there is a correlation of serotonin levels with the development of right sided heart disease