Brief overview of the clinical features, epidemiology, pathophysiology, etiology, morphology, diagnosis, complications & medical management of rheumatic heart disease Enjoy learning!

1. RHEUMATIC
HEART DISEASE
- BY ROSHAN ASATI
SECOND YEAR MBBS
MGM MEDICAL COLLEGE

2. INTRODUCTION
❏ Rheumatic Heart Disease (RHD) is the most common
acquired heart disease, especially in developing
countries.
❏ RHD is a chronic heart condition caused by recurrent
episodes of Rheumatic Fever which can be prevented and
controlled.

3. WHAT IS RHEUMATIC FEVER?

4. Rheumatic Fever
❏ Rheumatic Fever (RF) is a systemic, post-streptococcal, non-
suppurative inflammatory disease, principally affecting the heart,
joints, central nervous system, skin and subcutaneous tissues.
❏ It is an immunologically mediated inflammatory disorder, which
occurs as a sequel to Group A beta hemolytic Streptococcal
pharyngeal infection.
❏ Rheumatic Fever - Not a communicable disease but results from a
communicable disease (streptococcal pharyngitis).

5. CLINICAL FEATURES OF RF
● Streptococcal sore throat
○ Tender lymph node
○ Strawberry tongue
○ Excoriated nares (crusted lesions) in infants
○ Tonsillar exudate in older children
○ Abdominal pain
● Recurrence of fever with manifestations of Acute Rheumatic Fever
● Shortness of breath

6. RHEUMATIC HEART DISEASE
❏ Rheumatic heart disease is an immunologic disease characterized by
valvular damage or dysfunction followed by one or more episodes of
rheumatic fever caused by pharyngeal infection with GAB
Hemolytic Streptococci.
❏ RHD is the permanent heart valve damage resulting from one or
more attacks of ARF.
❏ Acute rheumatic carditis is a common manifestation of active RF and
may progress over time to chronic Rheumatic Heart Disease (RHD).
RF RHD
“Rheumatism licks the joint, but bites the whole heart” - William Boyd

7. Rheumatic Heart Disease
❏ The extent of the damage depends
on the heart area that the disease strikes.
❏ The order of frequency of involvement
depends on the hemodynamic stress
Placed on the various chambers.
❏ Thus the order is Mitral > Aortic > Tricuspid > Pulmonic
65-70% 20-25% 10% Rarely

8. EPIDEMIOLOGY
❏ The disease appears most commonly in children between the age of
5 to 15 years when the streptococcal infection is most frequent and
intense.
❏ The geographic distribution, incidence and severity of RF and RHD
are generally related to frequency and severity of streptococcal
pharyngeal infection.
❏ The disease is seen more commonly in
poor socio-economic strata of the society
living in damp and overcrowded place.

9. Epidemiology contd...
❏ The incidence of RF and RHD have declined in the developed
countries as a result of improved living conditions and early use of
antibiotics in streptococcal infection.
❏ But it is still common in developing countries (India, Pakistan,
Bangladesh, Nepal, Afghanistan) particularly Indian subcontinent,
some Pacific countries, sub-Saharan Africa and Latin America.
❏ In India, RHD and RF continue to be a major public health problem.
❏ In a multicentric survey in school-going children by the Indian
Council of Medical Research (ICMR), an incidence of 1-5.5/1000
children has been reported.

10. PATHOPHYSIOLOGY
❏ Based on current evidences, RF is caused by Group A Streptococcal
(GAS) pharyngeal infection.
❏ It is also evident that series of preceding streptococcal infections are
needed to prime the immune system prior to final infection that
directly causes the disease.
❏ The chronic stage of RF involves all the layers of heart (pancarditis)
causing major cardiac sequelae i.e RHD.
❏ Group A Streptococcal pharyngeal infection
Precedes clinical manifestations of ARF
by 2-6 weeks.

11. Pathophysiology
❏ Body produces antibodies against Group A Streptococci.
❏ These antibodies cross react with human tissues / host proteins
because of the antigenic similarity between streptococcal
components and human connective tissues (Molecular Mimicry).
[There is ceratin amino acid sequence - “M” protein that is similar
between GAS and human tissue]
❏ There is immunologically mediated inflammation & damage
(autoimmune) to human tissues which have antigenic similarity
streptococcal M proteins - like heart, joints, brain.

12. ❏ In particular, antibodies and CD4+T cells directed against streptococcal
M proteins can also in some cases recognize cardiac self-antigens.
❏ Cytokine production by the stimulated T cells leads to macrophage
activation (e.g., within Aschoff bodies)
❏ Damage to heart tissue may thus be caused by a combination of
antibody and T cell-mediated reactions.

15. ETIOLOGY

16. Etiology
The infection can also spread from :
● Everyday oral activities - Activities such as brushing your teeth or
chewing food can allow bacteria to enter your bloodstream.
● An infection or other medical condition - From an infected area,
such as skin sore OR Gum disease, a sexually transmitted disease.
● Weakened immune system
● Certain dental procedures - Some dental
Procedures can cut your gums and cause
The bacteria to enter the bloodstream.

17. MORPHOLOGICAL FINDINGS
➢ CARDIAC LESIONS
○ Involvement of the interstitial tissue of all the three layers of
heart, pancarditis happens in RF.
○ The characteristic feature of pancarditis in RF is the
presence of Aschoff nodules or Aschoff bodies.

19. ASCHOFF BODIES OR NODULES
❏ Aschoff bodies or nodules are spheroidal or fusiform distinct tiny
structures or granulomas, 1-2 mm in size, occuring in the
interstitium of the heart in RF and maybe visible to naked eyes.
❏ Especially found in the vicinity of small blood vessels in the
myocardium and endocardium and occasionally in the pericardium.
❏ Lesions similar to Aschoff nodules may be found in the extracardiac
tissues.

21. HOW ARE ASCHOFF BODIES FORMED?
● Formed in 3 stages :
1) Early (Exudative or Degenerative) stage
- Initially, there is oedema of the connective tissue and increase in
acid mucopolysaccharide in the ground substance. This results in
separation of collagen fibres by accumulating ground substance.
Eventually, the collagen fibres are fragmented and disintegrated,
that takes the stain of fibrin (hence called fibrinoid degeneration).
2) Intermediate (Proliferative or Granulomatous) stage
- This stage involves infiltration by lymphocytes (mostly T cells),
plasma cells, a few neutrophils and the characteristic cardiac
histiocytes (Anitschkow cells) at the margin of the lesion.

22. Cardiac Histiocytes or Anitschkow Cells
● These are large mononuclear cells having central round nuclei and
contain moderate amount of amphophilic cytoplasm.
● The nuclei are vesicular and contain prominent central chromatin
mass which in longitudinal section appears serrated or caterpillar-
like, while in cross-section the chromatin mass appears as a small
rounded body in the centre of vesicular nucleus, just like an owl’s
eye.
● Some of these modified cardiac histiocytes become multinucleate
cells containing 1 to 4 nuclei and are called Aschoff giant cells and
are characteristic of RHD.

24. Aschoff Giant Cells

25. How are Aschoff Bodies formed?
3) Late (Healing or Fibrous) stage
- This is a stage of healing by fibrosis of the Aschoff nodule and occurs
in about 12 to 16 weeks after the illness resulting in fibrous scar.
- The nodule becomes oval or fusiform in shape.
- The Anitschkow cells in the nodule become spindle-shaped with
diminished cytoplasm.
- These cells tend to be arranged in a palisaded manner.
- With passage of time, Aschoff body becomes less cellular and
collagenous tissue is increased. Later it is replaced by a
fibrocollagenous scar.

26. RHEUMATIC VALVULITIS
● The valves in acute RF show thickening and loss of translucency of
the valve leaflets or cusps. This is followed by the formation of
characteristic, small (1 to 3 mm in diameter), multiple, warty
vegetations or verrucae. Chiefly along the line of closure of the
leaflets and cusps. These tiny vegetations are almost continuous so
that the free margin of the cusps or leaflets appear as a rough and
irregular ridge.
● The vegetations in RF appear grey-brown, translucent and are
firmly attached so that they are not likely to get detached to form
emboli, unlike the friable vegetations of infective endocarditis.

27. Rheumatic Valvulitis
Though all the four heart valves are
affected, their frequency and severity of
involvement varies: mitral valve alone
being most common site, followed in
decreasing order of frequency, by
combined mitral and aortic valve. The
tricuspid and pulmonary valve usually
show infrequent and slight involvement.
The higher incidence of vegetations on
left side of heart is possibly because of
the greater mechanical stresses on the
walls of the left heart, especially along
the line of closure of the valve cusps.

28. ● Due to vegetations and
thickening the mitral valve may
look like ‘fish mouth’ or
‘buttonhole’. Mitral stenosis and
insufficiency are commonly
combined in chronic RHD.
Calcific aortic stenosis may also
be found.

29. ● Thickening, shortening and fusion of the chordae tendineae further
contribute to the chronic valvular lesions.

30. RHEUMATIC MURAL ENDOCARDITIS
❏ Mural endocardium may also show features of rheumatic carditis
though the changes are less conspicuous as compared to valvular
changes.
❏ Grossly, the lesions most commonly seen in MacCallum’s patch which is
the region of endocardial surface in the posterior wall of left atrium just
above the posterior leaflet of the mitral valve. MacCallum’s patch
appears as a map-like area of thickened, roughened and wrinkled part
of the endocardium.
❏ Microscopically, the appearance of MacCallum’s patch is similar to that
seen in rheumatic valvulitis. The affected area shows oedema, fibrinoid
change in the collagen, and cellular infiltrate of lymphocytes, plasma
cells and macrophages with many Anitschkow cells.

32. RHEUMATIC MYOCARDITIS
❏ Grossly in early (acute) stage, the myocardium especially of the left
ventricle, is soft and flabby. In the intermediate stage, the interstitial
tissue of the myocardium shows small foci of necrosis. Later, tiny
pale foci of the Aschoff bodies may be visible throughout the
myocardium.
❏ Microscopically, the most characteristic feature of rheumatic
myocarditis is the presence of distinctive Aschoff bodies.
❏ These nodules are scattered throughout the interstitial tissue of the
myocardium and most frequent in the interventricular septum, left
ventricle and left atrium.

33. RHEUMATIC PERICARDITIS
❏ Grossly, the usual findings is fibrous pericarditis in which there is
loss of normal shiny pericardial surface due to deposition of fibrin
on its surface and accumulation of exudate in the pericardial sac. If
the parietal pericardium is pulled off from the visceral pericardium,
the two separated surfaces are shaggy due to thick fibrin covering
them.
❏ The appearance is often likened to ‘bread and butter appearance’
that is resembling the buttered surface of two slices in a sandwich
when they are gently pulled apart. If fibrinous pericarditis fails to
resolve and instead, undergoes organisation, the two layers of the
pericardium form fibrous adhesions resulting in chronic adhesive

35. ➢EXTRACARDIAC LESIONS
POLYARTHRITIS
Acute and painful inflammation of the
synovial membranes of some of the
joints, especially the larger joints of the
limbs, is seen in about 90% cases of RF in
adults and less often in children. As pain
and swelling subsides in one joint, others
tend to get involved, producing the
characteristic ‘migratory polyarthritis’
involving two or more joints at a time.
Histologically, the synovial membrane
show hyperaemia, oedema, fibrinoid
change and neutrophilic infiltration.

36. SUBCUTANEOUS NODULES
These nodules are small (0.5 to 2 cm in
diameter), spherical or ovoid and
painless. They are attached to deeper
structures like tendons, ligaments, fascia
or periosteum and therefore often
remain unnoticed by patients.Occur
mostly at extensor surfaces of wrists,
elbows,ankles and knees.
Histologically, subcutaneous nodules are
representative of giant Aschoff bodies of
the heart , with 3 distinct zones : central
area(fibrinoid changes), zone of
histiocytes & fibroblasts, outermost
zone(CT with inflammatory cells)

37. ERYTHEMA MARGINATUM
This non-pruritic erythematous rash
is characteristic of RF. The lesions
occur mainly on the trunk and
proximal parts of the extremities.
The erythematous area develops
central clearing and has slightly
elevated red margins. The erythema
is transient and migratory.

38. RHEUMATIC ARTERITIS
Arteritis in RF involves not only the
coronary arteries and the aorta but
also occurs in arteries of various
other organs such as renal,
mesenteric and cerebral arteries.
Lesions are seen mostly in
intramyocardial branches.
Histologically, lesions may be like
those of hypersensitivity angiitis or
sometimes may resemble
polyarteritis nodosa.

39. CHOREA MINOR
Also called as Sydenham’s chorea or
Saint Vitus’ dance is a delayed
manifestation of RF due to
involvement of CNS. The condition is
characterised by disordered and
involuntary jerky movements of the
trunk and the extremities
accompanied by some degree of
emotional instability.
Histologically, the lesions are
located in the cerebral hemispheres,
brainstem and the basal ganglia.

40. DIAGNOSIS

42. COMPLICATIONS
❏ Congestive Heart failure
❏ Infective Endocarditis
❏ Arrhythmias mainly atrial fibrillation
❏ Embolic episodes
❏ Cardiomegaly

43. MEDICAL MANAGEMENT
1) ERADICATE INFECTION
❏ Preventive and prophylactic therapy is indicated after rheumatic fever
and acute rheumatic heart disease to prevent further damage to valves.
❏ Primary Prophylaxis (initial course of antibiotics administered to
eradicate the streptococcal infection) also serves as the first course of
secondary prophylaxis (prevention of recurrent rheumatic fever and
rheumatic heart disease).
❏ An injection of 0.6 to 1.2 million units of benzathine penicillin G
intramuscularly every 4 weeks is the recommended regimen.
❏ Administer the same dosage every 3 weeks in areas where rheumatic
fever is endemic, in patients with residual carditis, and in high-risk
patients.

44. ❏ Continue antibiotic prophylaxis indefinitely for patients at high risk
(eg, health care workers, teachers, daycare workers) for recurrent
GABHS infection.
❏ Patients of rheumatic fever with carditis and valve disease should
receive antibiotics for at least 10 years or until age of 40 years.
❏ Patients with rheumatic heart disease and valve damage require a
single dose of antibiotics 1 hour before surgical and dental
procedures to help prevent bacterial endocarditis.
❏ Patients who had rheumatic fever without valve damage do not need
endocarditis prophylaxis.

45. ❏ Do not use penicillin, ampicillin or amoxicillin for endocarditis
prophylaxis in patients already receiving penicillin for secondary
rheumatic fever prophylaxis (relative resistance of PO streptococci
to penicillin and aminopenicillins.
❏ Alternate drugs recommended by the American Heart Association
for these patients include PO clindamycin (20 mg/kg in children, 600
mg in adults) and PO azithromycin or clarithromycin (15 mg/kg in
children, 500 mg in adults).

46. 2) MAXIMIZE CARDIAC OUTPUT
❏ Corticosteroids are used to treat carditis, especially if heart failure is
evident.
❏ If heart failure develops, treatment including ACE inhibitors, Beta
blockers and Diuretics is effective.
3) PROMOTE COMFORT
❏ Patients with arthritic manifestations obtain relief with salicylates.
❏ Bed rest is usually prescribed to reduce cardiac effort until evidence
of inflammation has subsided.

47. SURGICAL MANAGEMENT
BALLOON VALVULOPLASTY MITRAL COMMISSUROTOMY

49. VALVE REPLACEMENT