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Cardiovascular Block: Week 6 > Cardiac Valvular Diseases: Pathology > Flashcards

Cardiac Valvular Diseases: Pathology Flashcards

1
Q

Which side of the heart is more likely to get valvular disease?

A

Left side

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2
Q

What are the three main causes of valvular disease?

A
  1. Calcified valves
  2. Valve degeneration
  3. Annular dilatation
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3
Q

What can calcification of the valves be described as?

A

“Atherosclerosis of the valve”

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4
Q

What can cause valve degeneration?

A
  1. Structural components - connective tissue diseases (Marfan syndrome)
  2. Micro-organisms (directly or through immune reaction)
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5
Q

What can lead to annular dilatation?

A
  1. Aortic aneurysm

2. Rupture of papillary muscle

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6
Q

What is stenosis?

A

Failure of valve to open completely, impedes forward flow

= “tight valve”

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7
Q

What is insufficiency / regurgitation?

A

Failure of valve to close completely, allowing reverse flow

= “floppy valve”

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8
Q

In what situations may stenosis / regurgitation occur?

A

May be:

  • alone or coexist
  • in a single valve or in > 1 valve at a time
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9
Q

What does clinical presentation in valvular disease depend on?

A
  1. Valve involved
  2. Degree of impairment
  3. How fast it develops
  4. Compensatory mechanisms
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10
Q

Which two valvular disorders are classified as calcification?

A
  1. Calcific aortic stenosis

2. Calcific stenosis of congenitally bicuspid aortic valve

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11
Q

What types of valvular degeneration are there?

A
  1. Structural:
    - Mitral valve prolapse (myxomatous degeneration of the mitral valve)
    - Connective tissue diseases
  2. Micro-organisms:
    - Rheumatic heart disease
    - Infective endocarditis
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12
Q

How does calcific valvular disease develop?

A
  • Heart valves are subject to high levels of repetitive mechanical stress (especially left side of the heart)
  • Cumulative damage and calcification that lead to clinical dysfunction
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13
Q

What builds up in calcific valvular disease?

A

Similar to atherosclerosis: build up of calcium, fat and cholesterol

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14
Q

What is calcific aortic stenosis?

A

Age associated “wear and tear” (to normal or bicuspid valves)
- due to recurrent chronic injury (hyperlipidemia, hypertension, inflammation)

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15
Q

What are the risk factors for calcific aortic stenosis?

A

Same risk factors are coronary artery disease

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16
Q

What is the long term outcome of calcific aortic stenosis?

A

Calcified masses within the aortic cusps which ultimately protrude through outflow surfaces and prevent cuspal opening
- Eventually get outflow obstruction with LV pressure overload, LVH
= Need valve replacement

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17
Q

What effect does calcific aortic stenosis have on the mitral valve?

A

The mitral valve is usually normal

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18
Q

What is a congenital bicuspid aortic valve?

A

Developmental abnormality present in 1% of the population
= 2 functional cusps; unequal size
- 2 cusps doing the work of 3 therefore damaged more easily

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19
Q

What are patients with a congenital bicuspid aortic valve predisposed to?

A
  1. Calcific stenosis of congenital bicuspid aortic valve

2. Infective endocarditis

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20
Q

How else can the aortic valve become bicuspid (other than due to congenital causes)?

A

Valves can also become bicuspid secondary to rheumatic valve disease

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21
Q

What is mitral annular calcification?

A

Typically develops in fibrous annulus (ring supporting aortic valve)
= irregular, hard, occasionally ulcerated nodules at the base of the leaflet
(usually does not affect valvular function)

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22
Q

What are patients with mitral annular calcification at risk of developing?

A
  1. May develop thrombi, risk for embolisation

2. Nidus for infective endocarditis

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23
Q

What are the two causes of valvular degeneration?

A
  1. Connective tissue disorders: mitral valve prolapse

2. Micro-organisms (RHD / IE)

24
Q

What is mitral valve prolapse?

A

Myxomatous degeneration of the mitral valve - mitral valve bulges into the LA
- one or both of the mitral valve leaflets are floppy and balloon back into LA during systole

25
Q

What is the etiology of mitral valve prolapse?

A 
Etiology unknown (idiopathic) 
- may be associated with connective tissue disorders e.g. Marfan syndrome
26
Q

Mitral valve prolapse: what is the appearance of the affected leaflets?

A

Affected leaflets are enlarged, redundant, thick and rubbery

27
Q

Mitral valve prolapse: what is the appearance of the Tendinous chords?

A

Tendinous chords may be elongated, thinned or ruptured

28
Q

Mitral valve prolapse: What histological changes are seen?

A

Myxomatous degeneration with deposition of mucoid material in valve

29
Q

Mitral valve prolapse: what may happen to the annulus?

A

Annulus may be dilated

30
Q

Mitral valve prolapse: what effect does it have on other valves?

A

Other valves may be affected

31
Q

What secondary changes occur in mitral valve prolapse?

A

Fibrous thickening of valve leaflets

32
Q

What is rheumatic fever?

A

Rheumatic fever is an acute immunologically mediated multi-system inflammatory disease
- occurs a few weeks after an episode of group A streptococcal pharyngitis (can follow after streptococcal infections at other sites e.g. skin)

33
Q

What is a common manifestation during the active phase of rheumatic fever?

A

Acute rheumatic carditis

34
Q

Over time what may rheumatic fever progress to?

A

Rheumatic heart disease

35
Q

What is the key feature of rheumatic heart disease?

A

Valvular abnormalities = key feature

  • Characterized by deforming fibrotic valvular disease; particularly mitral valve
  • Especially mitral stenosis (RHD virtually only cause of mitral stenosis)
36
Q

What is the morbidity and mortality of rheumatic heart disease?

A

M&M has decreased due to better socio-economic conditions and treatment of streptococcal pharyngitis with antibiotics.
- Still seen in developing countries

37
Q

Describe the pathogenesis of rheumatic fever.

A
  • Host immune response to group A streptococcal antigens that cross-react with host proteins
  • Antibodies and CD4 T-cells directed against streptococcal M proteins can recognize cardiac self-antigens
  • Antibody binding activates complement and recruits neutrophils and macrophages
  • Cytokine production stimulated by T-cells lead to macrophage activation
    = DAMAGE to heart tissue as a combination of antibody and T-cell mediated reactions
38
Q

What is said of rheumatic fever?

A

“Licks the joints, but bites the heart”

39
Q

What criteria is used in diagnosing rheumatic fever and how many of the criteria must be present for a diagnosis to be made?

A

Jones Criteria

2 major or 1 major and 2 minor criteria

40
Q

What are the major criteria in the Jones Criteria system?

A
  1. Pancarditis
  2. Migratory polyarthritis of large joints
  3. Subcutaneous nodules
  4. Skin rashes - Erythema marginatum
  5. Sydenham’s Chorea
41
Q

What is Sydenham’s Chorea?

A

Neurological disorder = involuntary rapid purposeless movements

42
Q

What are the minor criteria in the Jone’s Criteria system?

A
  1. Recent sore throat (increased serum anti-strptolysin O titres / positive throat cultures for beta hemolytic group A streptococcus)
  2. Arthralgia
  3. Fever
  4. Acute phase reactants (increased CRP, increased ESR, leukocytosis)
  5. ECG changes
43
Q

How long after a group A streptococcal infection does acute rheumatic fever occur?

A

Occurs 10 days to 6 weeks following Group A Strep infection

44
Q

What age group is most commonly affected by acute rheumatic fever?

A

Mostly children 5-15 years

45
Q

What are some of the features of acute rheumatic fever?

A
  • Carditis and arthritis
  • Pericardial friction rubs, tachycardia, arrhythmias
  • Myocarditis
46
Q

Following the initial attack, what are some of the chronic complications of rheumatic fever?

A
  1. After initial attack, increased vulnerability to reactivation of the disease with subsequent pharyngeal infections
  2. Damage to valves is cumulative
  3. Ongoing valvular deformities with fibrosis
47
Q

What are the macroscopic features of acute rheumatic fever?

A

Pancarditis (pericarditis, myocarditis, endocarditis)

48
Q

What are the microscopic features of acute rheumatic fever?

A

Distinctive lesions in the heart: Aschoff bodies (foci of T-lymphocytes, plasmal cells, activated macrophages called Anitschkow cells)

49
Q

What is “caterpillar cell” a nickname for?

A

Cardiac histiocyte

50
Q

What is the main pathological effect of the thickening of the mitral valve in chronic rheumatic heart disease?

A

The main pathological effect is the dilation of the mitral ring. Only the posterior part of the ring can dilate so the anterior leaflet cannot oppose the retracted posterior leaflet.
&
The chordae elongate due to the inflammatory oedema
= SEVERE often fatal mitral regurgitation

51
Q

What to important processes take place as the acute phase of rheumatic fever heals?

A
  1. Valvular stenosis (mitral + aortic)
  2. The chordae tendineae initially “sticky” in the acute phase tend to stick together, and as the inflammatory process heals chordae fuse together and shorten as the fibrous scar tissue contracts in healing.
    = Valvular incompetence (mitral). The valve cannot close properly as the leaflets are held down by the shortened chordae.
52
Q

What are the cardinal anatomic changes of the mitral valve in chronic rheumatic valve disease?

A

Leaflet thickening

  • Commisural fusion and shortening
  • Thickening and fusion of tendinous cords
53
Q

Which valves are usually affected in chronic rheumatic valve disease?

A

In chronic disease mitral valve is virtually always involved; or with aorta; tricuspid involvement not common; pulmonary valve uncommon

54
Q

What is the result of chronic rheumatic valve disease leading to rheumatic mitral stenosis?

A

In rheumatic mitral stenosis calcification and fibrosis bridging across the valvular commissures result in “fish-mouth” or “buttonhole” stenoses

55
Q

What is the result of tight mitral stenosis in chronic rheumatic valve disease?

A
  • LA progressively dilates = mural thrombi

- Embolisation

56
Q

What are the 3 main complications of chronic rheumatic heart disease?

A
  1. Thromboembolic complications
  2. Infective endocarditis
  3. Heart failure
57
Q

What valvular disease are seen in chronic rheumatic heart disease?

A
  1. Narrowed valve outlet = stenosis

2. Shortened, fused, and thickened chordae = mitral incompetence

Cardiovascular Block: Week 6 (4 decks)

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