5: Rheumatic valvular heart disease Flashcards

1
Q

purpose of valves, and how damage results

A
  • 4 cardiac valves:
    • Tricuspid
    • Mitral
    • Pulmonic
    • Aortic
  • Maintain unidirectional blood flow
  • Suffer from high levels of repetitive mechanical stress
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2
Q

which valve is described as following?

  • 3 thin, delicate cusps
  • Coronary artery orifices above it
  • Smooth, shiny
A

AORTIC VALVE

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3
Q

which valve is described as following?

  • 3 thin, delicate leaflets
  • Thin chordae tendineae attaching leaflet to papillary muscles of ventricular wall
A

TRICUSPID VALVE

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4
Q

2 types of valvular disease?

A

STENOSIS or INSUFFICIENCY

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5
Q

Type of valvular disease associated w/ the following?

  • Failure to open completely
  • Obstructs forward flow
  • Due to primary cuspal abnormality from a chronic process
  • Leads to pressure overload cardiac hypertrophy
A

STENOSIS

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6
Q

Type of valvular disease associated w/ the following?

  • Failure to close completely
  • Regurgitation of blood
  • Due to intrinsic disease of cusps (endocarditis) or secondary (disruption of supporting structures)
  • Leads to volume overload
A

INSUFFICIENCY

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7
Q

pathophys of mitral stenosis?

A

postinflammatory scarring (rheumatic heart disease)

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8
Q

pathophys of mitral regurgitation?

A
  • abnormalities of leaflets and commissures
    • postinflammatory scarring
    • ineffective endocarditis
    • mitral valve prolapse
    • “Fen-phen”- induced valvular fibrosis
  • abnormalities of tensor apparatus
    • rupture of papillary muscle
    • papillary muscle dysfunction (fibrosis)
    • rupture of chordae tendineae
  • abnormalities of left ventricular cavity and/or annulus
    • left ventricular enlargement
    • myocarditis, dilated cardiomyopathy
    • calcification of mitral ring
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9
Q

pathophys of aortic stenosis?

A
  • postinflammatory scarring (rheumatic heart disease)
  • senile calcific aortic stenosis
  • calcification of congenitally deformed valve
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10
Q

pathophys of aortic regurgitation?

A
  • intrinsic valvular disease
    • postinflammatory scarring (rheumatic heart disease)
    • infective endocarditis
  • aortic disease
    • degenerative aortic dilation
    • syphilitic aortitis
    • anklylosing spondylitis
    • rheumatoid arthritis
    • marfan syndrome
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11
Q

what accounts for 2/3 of all valvular disease?

A

acquired stenosis of Aortic valve and Mitral valve

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12
Q

what infection causes rheumatic fever?

A
  • acute, immunologically mediated multisystem inflammatory disease occurs after group A β-hemolytic streptococcal infection (usually pharyngitis)
  • Incidence declined in the Western world
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13
Q

what is rheumatic heart disease (RHD)?

what is the characteristic lesion associated?

A

cardiac manifestation of rheumatic fever

  • Acute & chronic
  • Characteristic lesion – fibrotic valvular disease (MV)
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14
Q

what is the pathogenesis of rheumatic fever and heart disease?

A
  • results from a hypersensitivity reaction –> antibodies directed against M proteins of certain Strep strains cross-react w/ host myocardial antigens
  • Antibody binding –> activates complement, recruits macrophages, & neutrophils, cytokine production by T-cells leads to macrophage activation

Genetic susceptibility (~3% will develop RF)

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15
Q

the hypersensitivity reaction of Rheumatic fever/ heart disease causes pancarditis of which valves?

A
  • Mitral valve alone - 70% of cases
  • Mitral valve + aortic valve - 25% of cases
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16
Q

what are the key morphological features of acute rheumatic fever?

A
  • Anitschkow cells - (pathognomonic for RF); plump, elongated macrophage with slender wavy central, condensed chromatin (caterpillar cells)
  • Aschoff bodies -
    • collections of primarily T lymphocytes, plasma cells, and plump activated macrophages
    • aka mononuclear cells, histiocytes w/ abundant cytoplasm, central nucleoli, can be binucleated (image)
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17
Q

what are the features of pancarditis associated w/ Acute Rheumatic Fever?

A
  • Pericarditis – fibrinous exudate–> friction rub
  • Myocarditis – scattered Aschoff bodies –> arrhythmias, heart failure
  • Valvular disease – verrucous vegetations (1-2 mm) along the lines of closure
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18
Q

what are caterpillar cells?

A

Anitschkow cells: plump, elongated macrophage with slender wavy central, condensed chromatin (caterpillar cells)

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19
Q

what are the morphological changes of chronic rheumatic fever?

A
  • Replace acute lesions with scarring
    • leaflet thickening
    • commissural fusion and shortening
    • thickening and fusion of chordae tendineae
  • MV virtually always involved –> mitral stenosis –> “fish mouth” appearance
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20
Q

what is this gross anatomical finding associated with?

A

fibrous thickening, commissural fusion (fishmouth);

associated w/ CHRONIC Rheumatic Fever

21
Q

what is this gross anatomical finding associated with?

A

Neovascularization of valve, thickened, fused chordae;

associated w/ Chronic Rheumatic Fever

22
Q

clinical features of acute rheumatic fever

A
  • Timing: appears 10 days to 6 weeks after infxn in 3% of patients
  • Clinical sxs:
    • Pharyngeal cultures are usually negative
    • Antibodies against strep are elevated (streptolysin O or DNAase)
  • After 1st attack, patient has increased vulnerability to reactivation of disease with subsequent pharyngeal infection
23
Q

what is the following auscultation finding? what condition is it associated with?

“opening snap followed by diastolic rumble”

A
  • mitral stenosis
  • associated w/ acute rheumatic fever
24
Q

To diagnose: serologic evidence of a previous Strep infection + 2/- Jones Criteria

(Jones Criteria includes what?)

A
  • Carditis
  • Migratory polyarthritis of large joints
  • Subcutaneous nodules
  • Erythema marginatum
  • Sydenham chorea
25
Q

what are the minor criteria associated with Rheumatic Fever?

A
  • fever,
  • arthralgias,
  • EKG changes,
  • elevated acute phase reactants
26
Q

what is the most common cause of aortic stenosis?

what causes this?

A
  • Calcific Aortic Stenosis;
  • Result of recurrent chronic injury (hyperlipidemia, hypertension, inflammation) , due to age-related “wear and tear”
27
Q

Calcific Aortic Stenosis:

epidemiology

A
  • Incidence increases with age (70s-80s, but 40s-50s in those with bicuspid AV)
  • Bicuspid aortic valve – 1-2% of all live births, 2 cusps of unequal size (NOTCH1 mutations)
28
Q

what are the complications of aortic stenosis?

A
  • Concentric LV hypertrophy - may progress to HF
  • Angina and syncope with exercise - limited ability to increase blood flow across stenotic valve
  • Microangiopathic hemolytic anemia - RBCs damaged while crossing the calcified valve
29
Q

describe the pathology of mitral valve prolapse (MVP)?

A
  • Myxomatous Degeneration –> One/both leaflets are “floppy” and prolapse
  • Etiology is unclear (likely disorders of connective tissue - Marfan syndrome)
    • Primary MVP: F >>> M
    • Secondary MVP: M=F
30
Q

mitral valve prolapse (MVP) - morphology

A

Ballooning of the leaflets

  • Chordae can be elongated, thinned, and may rupture
  • Valve leaflets undergo fibrotic thickening (<–rubbing against each other)
  • LV endocardial surface undergoes linear fibrous thickening (<–long cords rub against surface)
  • Mural endocardium of LV/LA thickens
  • Atrial surfaces of the leaflets/ atrial walls develop thrombi
31
Q

which histo slide is normal?

what is the characteristic sign of a myxomatous mitral valve?

A
  • Left side is normal
  • Right side is myxomatous mitral valve:
    • collagen in the fibrosa is loose & disorganized
    • **proteoglycan deposition (mucoid/myxoid material) in the spongiosa is
      markedly expanded
    • elastin in the atrialis is disorganized
32
Q

what are the clinical features of mitral valve prolapse associated w/ Myxomatous degeneration?

A
  • Most are asymptomatic
  • Palpitations, dyspnea, chest pain
  • *Midsystolic click* on ausculation
  • 3% of patients will develop complications
    • Infective endocarditis
    • Mitral insufficiency with chordal rupture
    • Stroke
33
Q

what is: Microbial infection of the heart valves or endocardium that leads to formation of vegetations often assoc. with destruction of underlying cardiac tissue

A

Infective endocarditis

34
Q

which bacteria is associated w/ Acute Infective Endocarditis?

A
  • (Staph aureus)
  • destructive infections, highly virulent organism attacking a previously normal valve
    • substantial morbidity and mortality, even with appropriate antibiotic therapy and/or surgery.
35
Q

which bacteria is associated w/ Subacute Infective Endocarditis?

A
  • (Strep viridans)
  • infections by organisms of low virulence affecting a previously abnormal valves (scarred/deformed)
    • follows a protracted course of weeks to months; most patients recover
      after appropriate antibiotic therapy.
36
Q

pathogenesis of infective endocarditis?

A
  • Starts with seeding of the blood with microbes
  • Develops on normal, damaged, or prosthetic heart valves
    • RHD, MVP, bicuspid AV, calcific valvular stenosis

10% culture negative endocarditis

37
Q

most of the damaged/defomed valves are affected by which bacteria?

A
  • 50-60% of cases affecting damaged/deformed values
  • Streptococcus viridans (part of normal oral flora)
38
Q

most of the normal/damaged valves are affected by which bacteria?

A
  • 10-20% normal/damaged valves
  • Staph. aureus
    • Major cause in IV drug users
39
Q

which causative organism causes infective endocarditis in prosthetic valve?

A

Staphylococcus epidermidis

40
Q

what are the HACEK group of organisms associated w/ infective endocarditis?

A
  • Haemophilus
  • Actinobacillus,
  • Cardiobacterium
  • Eikenella
  • Kingella
41
Q

what is the key morphology of Infective Endocarditis?

A
  • Friable, bulky, destructive vegetations that contain fibrin, inflammatory cells, microorganisms
  • Ring abscess – vegetations erode into underlying myocardium and form an abscess cavity
  • Aortic and mitral valves are most commonly infected
  • Tricuspid valve frequent target in IV drug users
42
Q

symptoms associated with Infective Endocarditis?

A
  • Fever, fatigue, weight loss, flu-like symptoms
  • Murmurs in 90% with left-sided lesions
43
Q

What are the microscopic pathological features in infective endocarditis?

what clinical presentation does this cause?

A
  • Microemboli –> petechiae, splinter hemorrhages, retinal hemorrhages (Roth spots), painless palm or sole erythematous lesions (Janeway lesions), painful fingertip nodules (Osler nodes)
  • Glomerulonephritis
  • Fatal if untreated
44
Q

two families of noninfected vegetations?

A
  1. Nonbacterial thrombotic endocarditis
  2. Endocarditis of systemic lupus erythematosus (SLE)
45
Q

Nonbacterial Thrombotic Endocarditis:

pathology

A
  • Deposition of sterile thrombi on valves (underlying hypercoagulable state)
    • Mucinous adenocarcinoma
    • Also by indwelling catheters (endocardial trauma)
46
Q

Nonbacterial Thrombotic Endocarditis:

describe the vegetations; location, etc

A
  • Vegetations are non-destructive and small (1-5 mm)
  • Located along the line of closure of leaflets/cusps
  • Usually occurs on previously normal valves
  • Vegetations can also be a nidus for bacterial colonization –> infective endocarditis
47
Q

Endocarditis of Systemic Lupus Erythematosus (SLE) aka. Libman-Sacks Endocarditis:

describe the pathology and vegetations,

A
  • Path: Immune complex deposition –> inflammation –> can lead to fibrosis and scarring, similar to chronic RHD
  • Sterile, small (1-4 mm) vegetations on valves of patients with SLE
    • Mitral and tricuspid valves
    • Located on valve undersurface, cords, endocardial surfaces
48
Q

Endocarditis of Systemic Lupus Erythematosus (SLE) aka. Libman-Sacks Endocarditis:

histo, and epidemiology

A
  • Histology – fibrinous, pink material with cellular debris, valvulitis (fibrinoid necrosis of valve substance)
  • Epi: 10% of SLE patients
49
Q

what % of pts will develop serious prosthesis-related problems w/ prosthetic valves?

A

60% will develop serious prothesis-related problems within 10 years of surgery (thromboembolism, infective endocarditis, etc)