RHEUMATIC HEART DISEASE

Mr. ALFRIN ANTONY

Asst. Lecturer
DEPARTMENT OF PATHOLOGY
Rheumatic Fever

DEFINITION:-Rheumatic fever is a
systemic, post-streptococal,
non-suppurative inflamatory
disease, principally affecting
the heat, joints, central
nervous system, skin and
subcutaneous tissues.
(Harh Mohan)
 INCIDENCE
 Acute rheumatic fever appear most often in children
between the age 5 and 15 years,
 About 20% of 1st attacks occur in middle to later
stage of life.
 Prognosis for the primary attack is generally good
 1% of patients die from rheumatic fever.
 Increased vulnerability to reactivation of the disease
with subsequent pharyngeal infections.
 Carditis is likely to worsen with each recurrence and
damage is cumulative.
 99% of cases of mitral stenosis is due to RHD.
 Mitral valve alone-65 to 70% of the cases.
 Mitral and aortic valve -25% .
Rheumatic Fever
 A sequelae of rheumatic fever, can be acute
or chronic.
 Rheumatic fever is an acute
immunologically mediated multi system
Inflammatory disease.
 It occurs 10 days to 6 weeks after an
episode of group A (Beta-hemolytic)
streptococcal (pharyngitis) and often
involves the heart.
 Diagnosed by Jones Criteria:
 Either two of the major manifestations or
one major and two minor manifestations.
JONES CRITERIA
 MAJOR CRITERIA  MINOR CRITEIA
1.Carditis 1.Fever
2.Poly arthritis 2.Arthralgia
3.Chorea:- a neurologic 3.Previous History of RF
disorder with 4.Increased
involuntary a.E.S.R
purposeless rapid b.C-Reactive Protein
movements.
c.Leucocytosis
4.Erythema
5.Prlonged PR intravel
Marginatum
5.Subcutaneous
Nodules
ANTOBODIES AGAINST
Beeta-Haemolytic streptococci group A
 Anti-streptolysin O (ASO)
 Anti-streptokinase
 Anti-streptohyaluronidase
 Anti DNA ase B
Rheumatic Heart Disease
DEFINITION
Rheumatic heart disease
is a chronic condition
charectorised by scaring
and fibrosis of valves and
layers of the heart
secondary to rheumatic
fever
Pathology of RHD
 Pathogenesis
Hypersensitivity reaction.
Autoimmune mech. has been proposed
Antibodies directed against the M proteins of certain
strains of streptococci cross-react with tissue
glycoprotein in the heart, joints and other tissues.

Progressive fibrosis of both healing of the acute

inflammatory lesion.

Formation of chronic sequelae

And the turbulence induced by ongoing valvular
deformities.
Aschoff’s Bodies
 Aschoff’s bodies are nodules formed
by a reaction to inflammation with
accompanying swelling and
fragmentation of collagen fibers.
 As they become age, they become
more fibrous, and scar tissue is
formed in the myocardium
 Evolution of Aschoff bodies
The aschoff bodies development involves three
stages

1.Early stage (Exudative or

Degenerative)

2.Intermediate stage (proliferative or

granulomatous)
3.Late stage (healing or fibrous)
 1.Early(Exudative) Stage:-4th week

Edema of connective tissue + increased acid

mucopolysaccharide in the ground
substance.

Accumulation of ground substance.

Separation of collagen fibers.

Collagen fibers become fragmented and

disintegrated.
 2.Intermediative (Proliferative) Stage

4th to 13th week

Proliferation of cells (lymphocytes, plasma

cells, a few neutrophils, cardiac histocytes
(anitschkow cells) at the margin of the
lesion

Anitschkow cells present in small number

in normal but it is increased in the
ANITSCHKOW CELL

Caterpillar-like 
(longitudnal
section)

Owl’s eye
(cross section)
Aschoff cells
3.Late (Fibrous o Healing) Stage
Aschoff nodules (12 to 16 weeks)

Anitschkow cells nodule becomes spindle shaped

with diminished cytoplasm.

AFTER YEARS
Aschoff body becomes less cellular and collagenous
tissue is increased

Fibro collagenous scar

Morphology
 Typically occurs as a pancarditis.
 Diffuse inflammation and aschoff bodies may
be found in any of the three layers of the
heart.
 Aschoff bodies are foci of fibrinoid degeneration
surrounded by lymphocytes,
 Occasional plasma cells and plump
macrophages called anitschkow cells
Pathgnomonic for rheumatic fever or caterpillar
cells
 +/- aschoff giant cells - multinucleated cells.
RHEUMATIC PANCARDITIS
1.Rheumatic endocarditis
(a).Rheumatic valvulitis
(b).Rheumatic mural endocarditis
2.Rheumatic myocarditis
3.Rheumatic pericarditis
1(a).Rheumatic Valvulitis
Grossly Microscopically
Acute Acute
 Thickening and loss of  Edema
transulency of the  Cellular infiltration
valve leaflets  Vegetations of fibrin
 Gray brown, watery
vegetations Chronic
Chronic
 Thicken by fibrous
tissue with
 Permanent deformity hyalinization
of on one or more (Calcification rarely)
valves (mitral or
aortic)
 Thickened blood
vessels with narrowed
 ”Fish mouth” or lumina
“button hole”
 Thickening,
shortening and fusion
of chordae tendinae
Endocardium
 Small (1-2mm)
irregular
vegetations –
verrucae – along
the lines of closure
of the valves.
 Leaflet Thickening
and Fusion of The
Tendinous Cords
“Fish Mouth” or “Button Hole”
Stenosis.
1(b).Rheumatic mural endocarditis
Grossly Microscopically
MacCallum’s patch:- MacCallum’s patch:-
Lesions of  Edema
endocardial surface  Fibrinoid changes in
in the posterior collagen
wall of the left  Cellular infiltrate of
lymphocytes
atrium just above
 Plasma cells
posterior leaflet of
 Macrphages
the mitral valve
 Anitschkow cells
2.Rheumatic Myocarditis
Grossly
Acute
Left ventricular myocardium
soft and flabby
Intermediate stage
Interstitial tissue of the
myocardium shows small
foci of necrosis
Late stage
Foci of aschoff bodies are
visible
Microscopically
Acute
Aschoff nodules are
scattered (inter
venticular septum, left
ventricle and left atrium)
Intermediate stage
In Aschoff:- Granuloma with
central fibrinoid necrosis
and surrounded by
anitschkow cells
Late stage
Aschoffs bodies are replaced
by small fibrous scars
Myocardium
 Shows scattered aschoff bodies within
the interstitial connective tissue
 often perivascular
3.Rheumatic Pericarditis
Grossly Microscopically
Deposition of fibrous  Fibrosis aschoff
exudates (Loss of normal bodies on the
shiny pericardial surface)
surfaces
Accumulation of fibrous
 Infiltrated sub
exudates in the serosal connective
pericardial sac tissue
(“Bread and butter”  Adhesions between
appearance) visceral and parietal
surfaces
Chronic adhesive pericarditis
Pericardium
 Fibrinous
pericardial
exudate
 “Bread and
butter”
pericarditis
 Generally
resolves without
sequelae.
CLINICAL FEATURES
 Pericardial friction rubs,
 Weak heart sounds
 Tachycardia
COMPLICATIONS
 Arrhythmias
 Functional mitral valve insufficiency
 Heart failure.
INVOLVEMENT OF VALVES
99% of cases of mitral stenosis is due to RHD.
Mitral value alone-65 to 70% of the cases.
Mitral and aortic valve -25% .
Extracardiac Lesions
 Polyarthritis
 Subcutaneous nodules
 Erythema maginatum
 Chorea minor