1. Rheumatic heart disease
Ali Al Khader, M.D.
Faculty of Medicine
Al-Balqa’ Applied University

2. What will we discuss today?
Etiology and epidemiology of rheumatic heart disease
Pathogenesis of rheumatic heart disease
Morphological changes in rheumatic heart disease
Clinical manifestations

3. Etiology & overview Rheumatic fever (RF) …acute
Few weeks
Rheumatic fever (RF)
…acute
…immunologically mediated
…multisystem disease
Pharyngitis (mainly but not only)
Group A streptococcus
…a common manifestation of active RF is:
…acute rheumatic carditis
The main cardiac abnormalities in this condition are in ………..???
may progress to chronic rheumatic heart disease… = RHD

4. Epidemiology
Mortality & incidence of RF and RHD have significantly declined in many parts of the world….Why?

5. Pathogenesis Acute RF results from: immune reaction…cross-reactivity
Antibodies and CD4+ T cells…damage heart tissue
Antigens: M protein and cardiac self antigens (cross-reactivity)

6. Pathogenesis, cont’d Activation of complement T cells (mainly CD4+)
They recognize streptococcal M proteins (cross-react with cardiac self antigens) by T cell receptors (TCRs)
Activation of complement
stimulate B cells to secrete:
T cells (mainly CD4+)
Antibodies
Opsonization
-Cytokine release
-Activation of macrophages
in Aschoff bodies

7. Morphology In acute rheumatic fever:
…foci of inflammation in various tissues
…especially in the heart…we find: Aschoff bodies
= T cells + occasional plasma cells + activated macrophages
Pathognomonic for RF
= Anitschkow cells
-plump
-abundant cytoplasm
-central round-to-ovoid nuclei…occasionally binucleate
-chromatin condenses into a central, slender, wavy ribbon…so they are called: caterpillar cells
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8. Morphology of acute RF, cont’d
Diffuse inflammation and Aschoff bodies may be found in any of the three layers of the heart
…pericarditis, myocarditis or endocarditis
…pancarditis
little disturbance in cardiac function
Mainly: scattered Aschoff bodies in the interstitium
Mainly: fibrinous exudate
…self-limited
= valvular involvement: fibrinoid necrosis and fibrin
deposition along the lines of closure…verrucae (vegetations)

9. Acute on top of chronic RHD…an example
Small vegetations (1-2 mm)
Robbins basic pathology 9th edition

10. Morphology of chronic rheumatic heart disease
Organization…scarring
Aschoff bodies are replaced by fibrous scars…also there is calcification
Valve cusps & leaflets…permanently thickened and retracted
…especially: mitral valve
Robbins basic pathology 9th edition
(E, From Schoen FJ, St John-Sutton M: Contemporary issues in the pathology of valvular heart disease. Hum Pathol 18:568, 1967.)

11. Mitral stenosis in chronic RHD

12. Clinical notes, acute RF
Mainly children…carditis
20%: in adults…arthritis more
In all ages: 2-3 weeks after streptococcal infection:
…fever + migratory polyarthritis…then spontaneous resolution
…then: pericarditis, myocarditis or endocarditis…or pancarditis
< 1% mortality
Pericardial friction rub
Can be so severe to cause mitral insufficiency and congestive heart failure
arrhythmias

13. Clinical notes, acute RF…cont’d
Cultures are negative
Serum antibodies against one or more streptococcal antigens (streptolysin O, DNAse..etc.) are positive
For diagnosis:
serologic evidence of previous streptococcal infection +
2 or more of the major Jones criteria
(1) carditis
(2) migratory polyarthritis of large joints
(3) subcutaneous nodules
(4) erythema marginatum skin rashes
(5) Sydenham chorea (also called St. Vitus dance)
*Minor criteria:
-fever
-arthralgias
-ECG changes
-elevated acute phase reactants

14. Clinical notes, chronic RHD
Valvular stenosis and regurgitation…predominantly stenosis
…70%: mitral alone
…25%: mitral + aortic
…tricuspid…much less frequently
…pulmonic…almost always spared
…with time…left atrial dilation and atrial fibrillation…mural thrombus
…with time…a picture similar to left sided heart failure…followed by rt.
sided failure
…What about left ventricle??
Murmurs

15. Clinical notes, chronic RHD
Recurrent attacks with recurrent streptococcal infections
…cumulative damage
Chronic disease…after years/decades
Scarred and deformed valves are more susceptible to infective endocarditis

16. Thank You