Acute rheumatic fever Chronic Rheumatic heart disease Mitral stenosis Mitral regurgitation Mitral valve prolapse Aortic stenosis Aortic regurgitation

1. 1
Valvular Heart Diseases

2. 2
Valvular Heart Diseases
1. Acute rheumatic fever
2. Chronic Rheumatic heart
disease
3. Mitral stenosis
4. Mitral regurgitation
5. Mitral valve prolapse
6. Aortic stenosis
7. Aortic regurgitation
7. Tricuspid regurgitation
8. Carcinoid heart disease
9. Infective endocarditis
10.Libman-Sack
endocarditis
11.Nonbacterial
thrombotic endocarditis

3. 3
Acute Rheumatic Fever
• Is a multisystem immunologic disorder.
– Follows streptococcal infection
– Characterized by inflammatory reactions
involving:
• Heart, joints, basal ganglia and other
tissues.
• Epidemiology:
– Occurs at 5-15 years of ages
– Develops 1 to 5 weeks after group A beta
hemolytic streptococcal pharyngitis or scarlet
fever
– Possible relapse may occur leading to chronic
rheumatic heart disease.

4. 4
Acute Rheumatic Fever
• Pathogenesis:
– Immune mediated disease that follows group A
streptococcal infection
• Blood cultures are negative
• It is an immunologic reaction and not a
septicemia.
– Antibodies develop against group A
streptococcal M proteins
• Antibodies cross react with similar proteins
in human tissue (molecular mimicry).

5. 5
Acute Rheumatic Fever
Clinical findings and Diagnosis
• Jone’s criteria for ARF:
– Major criteria
1. Migratory asymmetric Polyarthritis
2. Carditis
3. Sydenham’s chorea
4. Subcutaneous nodules
5. Erythema marginatum
– Minor criteria:
• Fever , Arthralgia
• Leukocytosis, elevated ESR ,C-reactive protein
(CRP) and antistreptolysin O (ASO titers).
• Previous rheumatic fever or rheumatic heart
disease

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Acute Rheumatic Fever
• Migratory asymmetric Polyarthritis:
– MC (~ 75%) initial presentation of ARF
– Occurs in large joints (Knees) and small joints
(wrists)
– No permanent joint damage

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Endocarditis Myocarditis Pericarditis

8. 8
Acute Rheumatic Fever
• Carditis:
– Is a Pancarditis (involving all layers)
– Inflammation of
1. Pericardium (pericarditis)
2. Myocardium (myocarditis)
3. Endocardium (endocarditis)
1. Fibrinous pericarditis:
– Fibrin deposits on pericardium
– Precordial chest pain with friction rub

9. 9

10. 10
Acute Rheumatic Fever
2. Myocarditis: MC COD in acute disease*
– Aschoff Body:
• Is the pathognomonic lesion of RF
• Consists of Central area of fibrinoid
necrosis surrounded by
• Aschoff cells (aka Anitschkow cell)
– Are reactive histiocytes
– Fuse and form Aschoff multinucleated
giant cells.

11. 11
Aschoff bodies

12. 12
Aschoff body
Aschoff giant cell Aschoff cell

13. 13
Acute Rheumatic fever
• Endocarditis:
– Characterized by inflammation of valve surfaces
– Leading to development of
• Sterile verrucoid appearing vegetations (platelet
and fibrin clots) along the line of closure of the
valve
• Most commonly involves
– Mitral valve ( then aortic valve)
– Vegetations in RF:
• Do not embolize
• Can cause Mitral valve regurgitation or Aortic
valve regurgitation  May result in CHF.

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Vegetations at the margin of valve

15. 15
Acute Rheumatic fever
• Subcutaneous nodules:
– Occur on extensor
surface
– Fibrinoid necrosis
surrounded by
histiocytes
– Histologically same as
that seen in
rheumatoid arthritis

16. 16
Acute Rheumatic fever
• Erythema
marginatum
– Circular ring of
erythema with
central clearing.

17. 17
Acute Rheumatic fever
• Sydenham’s chorea:
– Reversible rapid, involuntary
movements affecting all muscles
– Late manifestation of ARF

18. 18
Acute Rheumatic fever
• Diagnosis of ARF
– Requires Presence of (Jone’s criteria)
• two major or
• One major and two minor
• Prognosis:
– Mortality very low
– MC COD is heart failure
– Recurrent attacks of ARF can lead to
Chronic rheumatic heart disease*

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Chronic Rheumatic Heart Disease
• Occurs if
– First attack of ARF is severe or
– With recurrent attacks of ARF
• Chronically damaged valves show:
– Fibrous thickening , fusion of commissures and
calcification, generating fishmouth or
buttonhole deformity.
• Most common valves involved are MV and AV.
– Resulting in mitral and aortic stenosis

20. 20
Button Hole Deformity

21. 21
Fused commisures
Narrowed orifice

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Complications of Chronic Rheumatic
Heart Disease
• Bacterial endocarditis:
– Damaged valves easily seeded by bacteria
• Mural thrombi:
– Complication of mitral stenosis
– Usually form in the left atrium
– Can give rise to thromboemboli  infarcts
• Congestive hear failure:
– Due to Aortic stenosis

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Mitral Stenosis

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Mitral Stenosis
• Etiology:
– Most often caused by recurrent attacks of
rheumatic fever
• Pathophysiology:
– Narrowing of the mitral valve orifice
– Volume overload in left atrium and lungs
– Left atrium becomes dilated and hypertrophied
• Due to increased work in filling the ventricle
in diastole

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Aortic stenosis
LVH
Mitral stenosis
Dilated LA

27. 27
Stenotic mitral valve
Dilated LA
with
thrombus

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Mitral Stenosis: Clinical findings
• Dyspnea and hemoptysis with rust colored
sputum:
– Due to pulmonary capillary congestion and
hemorrhages into the alveoli (heart failure
cells).
• Pulmonary hypertension:
– Due to chronic backup of atrial blood into the
pulmonary vein.
– RVH eventually develops
• Atrial fibrillation:
– Due to left atrial dilation and hypertrophy
– Intra atrial thrombus develops due to stasis.
• Can embolize

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Mitral Stenosis: Clinical findings
• Dysphagia for solids:
– Left atrium is the most posteriorly located
chamber in the heart
– Dilation of the left atrium compresses the
esophagus.
• Opening snap
– Occurs when non-pliable valve gives way under
increased left atrial pressure
• OS followed by mid diastolic rumble:
– Best heard at apex ,increases with expiration.

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3
1
5
4
2

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Mitral Regurgitation
• Causes:
1. Mitral valve prolapse is the MC cause
2. Left sided heart failure  stretching of MV
ring
3. Infective endocarditis
4. Rupture of papillary muscle
• Usually right coronary artery thrombosis

32. 32
Mitral Regurgitation
• Pathophysiology:
– Reflux of blood into LA during systole
• Due to an incompetent mitral valve or
dilated mitral valve ring
– Volume overload in LV and LA
• Leads to dilatation and hypertrophy of both
chambers
• Results in LHF

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Mitral Regurgitation
• Clinical findings:
– Pansystolic murmur with radiation into the
axilla
• Heard best at apex
• Increases on expiration
– S3 heart sound: volume overload in LV
– Dyspnea and cough from LHF

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Myxomatous connective tissue

35. 35
Mitral Valve Prolapse (MVP)
• Mitral valve leaflets are enlarged and floppy
– Prolapse in left atrium during systole
• Epidemiology:
– MC indication for valve replacement
– MC valvular disease in the young population
– More common in women
– Associated with Marfan and Ehlers Danlos
syndrome

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Mitral Valve Prolapse (MVP)
• Pathophysiology:
– Leaflets prolapse into the LA during systole
like a parachute
• Anterior and posterior leaflets***
• Pathology:
– Valve leaflets are deformed and redundant
– Valves become redundant (voluminous): due to:
• Myxomatous degeneration** of leaflets
– Due to excess production of dermatan
sulfate.

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Mitral Valve Prolapse (MVP)
Clinical findings:***
• Most patients are asymptomatic
• Heart murmur:
– Mid systolic click:
• Occurs when the valve prolapses into LA and
is suddenly restrained by the chordae
tendineae.
– Click followed by a mid to late systolic
regurgitation murmur***

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Aortic Stenosis
• 3 major causes:
1. MCC is calcified congenital bicuspid
valve
2. Chronic rheumatic heart disease
3. Degenerative (senile) calcific stenosis

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Calcific aortic stenosis
of a
congenitally bicuspid aortic valve
Calcific aortic stenosis
in a
three-cuspid aortic valve

40. 40
Aortic Stenosis
• Pathophysiology:
1. Obstruction to left ventricular outflow during
systole
• Decreased SV/CO
2. Reduction in aortic valve orifice area
produces pressure overload in the LV leading
to
• Concentric LV hypertrophy

41. 41
Aortic Stenosis: Clinical findings
1. Systolic ejection murmur*
2. Angina with exercise:
• Decreased blood flow through the stenotic
valves leads to less filling of the coronary
arteries during diastole
• Subendocardium of hypertrophied heart
receives less blood.
3. Syncope with exercise:
• Due to decreased blood flow through brain
4. Hemolytic anemia with schistiocytes

42. 42
Fragmented RBC’s
( = Schistiocytes)

43. 43
Aortic Regurgitation
• Causes:
1. Infective endocarditis
• MCC of acute aortic regurgitation
2. Isolated aortic valve root dilation
• Syphlitic aortic aneurysm
• Dissecting aortic aneurysm
• Coarctation of aorta.

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Aortic Regurgitation
• Pathophysiology:
1. Retrograde blood flow into the LV
• Due to incompetent valve or dilated valve
ring
– Volume overload of the left ventricle
» Increases stroke volume

45. 45
Aortic Regurgitation
• Clinical findings:
– Early diastolic murmur
– Bounding pulse (water hammer pulse), head
nodding, pulsating uvula.
– Austin flint murmur:
• Due to regurgitant stream hitting the
anterior mitral valve leaflet
• Presence of this murmur is a indication for
replacement of valve.

46. 46
Tricuspid regurgitation
• Etiology:
1. Right sided heart failure
2. Infective endocarditis in IVDA
3. Carcinoid heart disease.
• Pathophysiology:
– Retrograde flow into the RA during systole
– Produces volume overload in RA
– Volume overload in RV.
– Can cause RHF

47. 47
Tricuspid regurgitation
• Clinical findings:
– Pansystolic murmur that increase in intensity
with inspiration
– Pulsating liver
• Blood regurgitates into venous system with
systole

48. 48
Carcinoid heart disease
• Due to metastasis of a carcinoid tumor from
intestine to liver.
• Carcinoid tumors liberate serotonin.
• Carcinoid syndrome is a triad of:
– Cutaneous flushing
– Diarrhea
– Valvular disease
• Serotonin is fibrogenic and causes:
– Fibrosis of tricuspid valve and pulmonary
valves
• Results in tricuspid valve regurgitation and
pulmonary valve stenosis (TIPS).