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Y2S1-CVS-Pathology > Valvular heart diseases > Flashcards

Valvular heart diseases Flashcards

1
Q

What are the two types of valvular disease?

A

1) Stenosis

2) Regurgitation/Incompetence

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2
Q

What is meant by valvular stenosis?

A

It is the failure of a valve to open completely, impeding the blood flow

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3
Q

What is meant by valvular insufficiency?

A

It is the failure of a valve to close completely, allowing the reversed flow

  • Mainly occurs if there was an intrinsic cusp disease (like endocarditis) or disruption in the supporting system (disrupting the aorta, chorda tendea, papillary muscles)
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4
Q

What is meant by functional regurgitation?

A
  • It is a type of regurgitation that occurs but is not due to the valve, it is caused by:

1) Dilation of the ventricles, which leads to the pull-down and outward of the papillary muscles

2) Dilation of the aortic or pulmonary artery, pulling the valve commissures apart, preventing the closure of the aortic or the pulmonary valve cusps

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5
Q

What are the main causes of Valvular heart disease?

A

1) Congenital disorders

2) Acquired

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6
Q

What are the main causes of acquired valvular heart disease?

A

1) Degeneration (calcific aortic stenosis, Mitral annular calcification)

2) Immunologic inflammation (rheumatic heart disease)

3) Infection (IE)

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7
Q

What is meant by aortic stenosis?

A

It is the calcification of the normal aortic valve

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8
Q

What is the main cause of aortic stenosis?

A

1) Aging (mainly calcific)
- Senile calcific aortic stenosis
- Calcification of congenitally deformed valve
- Occurs due to wear and tear
- Calcific aortic stenosis is the most common valvular abnormality
- It can also be due to postinflammatory scarring (rheumatic heart disease)

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9
Q

What is meant by Mitral stenosis?

A

Calcification of the mitral valves

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10
Q

What is the main cause of mitral stenosis?

A

Postinflammatory scarring (rheumatic heart disease 99%) is the main cause of Mitral valve stenosis “unless proven otherwise”

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11
Q

What is meant by aortic insufficiency?

A

It is the dilation of the ascending aorta, mainly due to hypertension and aging

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12
Q

What is meant by degenerative valve disease, and what are its causes?

A
  • It is a term that describes the changes which affect the integrity of the valvular ECM

1) Calcifications:

  • Cuspal in the aortic valve
  • Annular in the mitral valve

2) Alterations in the ECM

  • Increased proteoglycan, & diminished fibrillar collagen and elastin (myxomatous degeneration)
  • In some cases, the valve becomes fibrotic and scarred

3) Changes in the production of the matrix metalloproteinases or their inhibitors

4) Aging (due to the repeated mechanical stress)

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13
Q

Describe the morphology of calcific aortic stenosis

A
  • Heaped-up nodular, rigid subendothelial masses on the outflow side of the cusps, causing cuspal thickening and immobility, impeding the aortic outflow
  • A raphe (site where the mitral valve fuses together) can occur in bicuspid valve (mitral)
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14
Q

What are the clinical features of calcific aortic stenosis?

A
  • The LV flow is obstructed = increased LV pressure = Concentric LV hypertrophy (exhaustion of the compensatory cardiac hyperfunction with a poor prognosis if not treated by surgery (50% mortality rate within 2 years)

1) Hypertrophic myocardium tends to be ISCHEMIC (ANGINA can develop)

2) SYNCOPE may develop due to the poor perfusion to the brain

3) Systolic and diastolic dysfunction which can lead to CHF

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15
Q

What are the mitral valve disease?

A

1) Mitral stenosis (incomplete opening of the mitral valve):
- 99% due to rheumatic heart disease
- Can also be due to calcification

2) Mitral insufficiency/regurgitation:

  • Mainly due to myxomatous degeneration (mitral valve prolapse)

2a) Acute:
- IE
-Ischemic heart disease
- Mitral valve prolapse

2b) Chronic:
- Ischemic heart disease
- Mitral valve prolapse
- IE
- Rheumatic
- Prosthetic
- Mitral annular calcification
- Cardiomyopathy

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16
Q

Describe the pathophysiology of mitral stenosis

A

1) Mitral Stenosis

Then

2) The LA- to LV diastolic gradient is increased

3) The left atrial pressure is increased

4a) Pulmonary hypertension (Secondary right-heart changes (RV dysfunction; tricuspid regurgitation)

5a) Right heart failure

4b) Left atrial Enlargement

5b) Atrial fibrillation and LA thrombus, having a high risk of thromboembolism

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17
Q

Describe the morphology of mitral valve stenosis

A

1) Leaflet thickening

2) Commissural fusion

3) Shortening, thickening and fusion of the tendinous cord

18
Q

What are the clinical features of the Mitral stenosis?

A

1) Pulmonary congestion

2) Atrial enlargement, and fibrillation

3) Peripheral embolization (anticoagulation is often needed)

4) Chest pain

5) Shortness of breath

6) Dizziness

7) Fatigue

8) Pedal edema

19
Q

What is meant by rheumatic fever?

A
  • The main cause of mitral stenosis
  • It is an immunologically mediated multisystem inflammatory disease, caused by a group A streptococcal pharyngitis
20
Q

Which bacterial group causes rheumatic fever?

A

Group A streptococcus pharyngitis (in immunology she said S.pyogens)

21
Q

Describe the pathogenesis of rheumatic fever

A

1) Acute

  • Occurs in children mainly (5-15 years)
  • It is a type of hypersensitivity reaction due to antibodies directed against the group-A streptococcal molecule that cross-reacts with the host antigen in the heart, joints, and other tissues
  • The principal clinical manifestation is carditis (symptoms begin 2-3 weeks after strep. infection accompanied by fever and polyarthritis

2) Chronic

  • It is when the extracardiac involvement in acute RF is resolved and scars replace the Aschoff bodies, progressively damaging the left valves mainly, forming a button-hole stenosis (fusion of the commissures of the mitral valve, accompanied by the shortening and thickening of the chorda tendinea)
22
Q

Describe the morphology of acute rheumatic fever

A
  • Pancarditis

1) Fibrinous pericarditis (bread and butter appearance)

2) Aschoff’s nodules (myocardial inflammatory lesions) in the myocardium

3) Endocardium:
- Fibrinoid necrosis within the cusps/along the tendinous cords
- Small 1-2mm vegetation along its internal lines

23
Q

What are the Aschoff bodies?

A
  • Swollen eosinophilic collagen
  • Collection of lymphatic infiltration (T-cells mainly)
  • Anitschkow Macrophages with abundant cytoplasm with centrally dense nuclei this cell looks like a caterpillar
  • Anitschkow cells can be found in all heart layers including the valves
24
Q

How to diagnose Acute RF?

A
  • Major:

1) JOINTS Polyarthritis

2) Carditis

3) Subacute Nodules

4) Erythema Marginatum of the skin (rounded pale pink lesions with raised red border on the trunk and limbs)

5) Sydenham chorea (neurologic disorder with involuntary purposeless movements)

  • Minor

1) Arthralgia

2) Prolonged PR interval

3) Fever

4) Elevated ESR/CRP

  • Evidence of streptococcal infection (Culture or Serology-anti-streptolysin O)
25
Q

What is meant by rheumatic aortic stenosis?

A

The stenosis of the aortic valve due to rheumatic heart disease, the mitral valve is nearly always involved to

  • Its morphology is the fusion of the commissures, thickening and distortion of the cusps
26
Q

What is meant by Myxomatous Mitral valve (Mitral Valve Prolapse)?

A
  • Non-inflammatory progressive disarray of the valve structure due to the altered synthesis/remodeling by type 6 collagen
  • One or both mitral valve leaflets are floppy and prolapse, they balloon back into the LA during systole

1) Primary

  • One of the most common forms of valvular disease affecting women 7 folds more

2) Secondary

  • Mitral regurgitation is caused by another underlying cause
27
Q

Describe the morphology of Myximatous Mitral Valve

A
  • One or both leaflets are floppy and prolapsed
  • Affected leaflet is usually enlarged, redundant, thick and rubbery
  • It is often accompanied by the elongation, attenuation, or rupture of the chordae tendineae
  • Concomitant involvement of the tricuspid valve is usually present in 20-40% of cases and the aortic/pulmonic valve might also be affected
  • You will see hooding of the thrombotic plaques in the valve leaflet
28
Q

Describe the structural changes that occur in myxomatous mitral valve

A
  • Deepest

1) Thinning of the fibrosa layer (“first-layer” structural integrity of the leaflet depends on it), with a disorganized collagen

2) Expansion of the middle spongiosa layer with the deposition of the myxomatous material, proteoglycans are deposited into it

3) The collagenous structure of the cord is attenuated

4) Elastin in the Atrialis is disorganized

  • Outermost
29
Q

What are the clinical features of myxomatous Mitral valve

A
  • Usually asymptomatic and discovered via routine examination

1) Mid-systolic click

2) If regurgitation is present, there will be a later holosystolic murmur

3) 3% develops (1. IE, 2. Mitral Insufficiency, 3. Arrhythmias, 4. Sudden death)

30
Q

What is meant by mitral annular calcification?

A
  • Degenerative non-inflammatory, calcific deposits, which can develop a fibrous rin “annulus” of the mitral valve
  • Irregular, stony hard, and occasionally ulcerated nodules (2-5mm) behind the leaflets
  • It generally does not affect the valvular function
  • Increases the risk of strokes, as the calcific nodule can be a site for thrombi which can embolize
  • The calcific nodule can be the site for IE
  • Common in women >60 years, and people with myxomatous mitral valve/elevated LV pressure
31
Q

What are the major valvular lesions?

A

1) Mitral stenosis

  • Rheumatic heart disease (thickened leaflets and commissural fusion)

2) Mitral insufficiency

  • Acute: IE, Ruptured chordee or papillary muscle
  • Chronic: MVP, RHD, Dilated cardiomyopathy

3) Aortic stenosis

  • Degenerative calcific stenosis, RHD, Congenital

4) Aortic insufficiency

  • Congenital, RHD, IE, Aortic root dilation
32
Q

What is endocarditis? and what are its types?

A
  • Inflammation of the endocardium

1) Non-infective

  • Immune-mediated (Rheumatoid endocarditis, SLE)
  • Non-bacterial thrombotic endocarditis

2) Infective Endocarditis

33
Q

What is meant by infective endocarditis?

A
  • Characterized by the colonization/invasion of the heart valves or the mural endocardium (inner wall of the heart) leading to the formation of the bulky vegetation (composed of thrombotic debris and organisms), most cases are bacterial, but fungi, rickettsiae, and chlamydia can all cause endocarditis
34
Q

What are the classification of endocarditis?

A

1) Acute endocarditis

  • Destructive infection of a native valve with a highly virulent microorganism, leading to death within days to weeks in more than 50% of the patients (caused by S.aureus, B-hemolytic strep, Pneumococcus)

2) Subacute endocarditis

  • Caused by organisms of low virulence in a previously abnormal heart, (HACEK group causes it)

3) Prosthetic valve endocarditis

  • Caused mainly by S.Epidermidis in hospitalized patients within 2 months, after that it is caused by strep and enterococci
  • In 10% of the cases no organism can be found as the cause
35
Q

What are the predisposing risk factors for IE?

A

1) Neutropenia

2) Immunodeficiency

3) Malignancy

4) Therapeutic Imunosuppressors

5) Diabetes Mellitus

6) Alcohol/Drug abuse

36
Q

Describe the pathogenesis of IE

A
  • It can develop in normal valves but cardiac abnormalities can also predispose it
  • Years back RHD was the major cause, but now myxomatous mitral valve, degenerative calcific valvular stenosis, bicuspid aortic valve, and artificial valve can all be a cause
37
Q

Describe the morphology of IE

A

1) Friable, Bulky, and potentially destructive vegetations containing fibrin, inflammatory cells, and organisms are present in the heart valve

2) Aortic and Mitral valves are most common

3) The vegetation might be single or multiple and might involve more than one valve

38
Q

What are the clinical presentations of IE?

A

1) Fever (Consistent)

  • Subacute:

2) Fatigue

3) Weight loss

4) Flu-like syndrome

  • Acute:

5) Chills

6) Weakness

7) Lassitude (worrying)

8) Murmurs (consistent)

9) Glomerulonephritis (as a complication)

10) Janeway lesions (not painful)

11) Roth spots (retinal hemorrhage)

12) Osler nodes (painful)

13) Splinter hemorrhage

39
Q

What is meant by nonbacterial thrombotic endocarditis (NBTE)?

A
  • Due to the deposition of small masses of fibrin, platelets, and other blood components on the leaflets of the cardiac valve (it is sterile)
  • It often occurs in patients with cancer or sepsis and might result in the production of emboli that cause infarcts in other places
40
Q

Describe the patterns of the different vegetation

A

1) RHD:
- Small at the chordae tendinae junction
- Single, Occurs In a line along the closure of the leaflets

2) IE:
- Big >5mm
- Concentrated in one place

3) LSE:
- Libman sacks on both sides of the valve

4) NBTE:
- Sterile, Non-destructive, small 1-5mm, in a single or multiply along the closure line of the leaflet

Y2S1-CVS-Pathology (12 decks)

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