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
6. 6
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
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.
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.
15. 15
Acute Rheumatic fever
• Subcutaneous nodules:
– Occur on extensor
surface
– Fibrinoid necrosis
surrounded by
histiocytes
– Histologically same as
that seen in
rheumatoid arthritis
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*
19. 19
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
22. 22
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
25. 25
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
28. 28
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
29. 29
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.
31. 31
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
33. 33
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
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
36. 36
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.
37. 37
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***
39. 39
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
44. 44
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).