18318109 rheumatic heart disease alfrin

7/29/2019 18318109 Rheumatic Heart Disease Alfrin

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Saranghaeyo Lyrics(Sassy Girl Chun-hyang OST)


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RHEUMATIC HEART DISEASE

Mr. ALFRIN ANTONY

Asst. Lecturer

DEPARTMENT OF PATHOLOGY


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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)


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INCIDENCE

Acute rheumatic fever appear most often in childrenbetween the age 5 and 15 years,

About 20% of 1st attacks occur in middle to laterstage 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% .


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Rheumatic Fever

A sequelae of rheumatic fever, can be acuteor chronic.

Rheumatic fever is an acuteimmunologically mediated multi systemInflammatory disease.

It occurs 10 days to 6 weeks after anepisode of group A (Beta-hemolytic)streptococcal (pharyngitis) and ofteninvolves the heart.

Diagnosed by Jones Criteria: Either two of the major manifestations or

one major and two minor manifestations.


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JONES CRITERIA

MAJOR CRITERIA

1.Carditis

2.Poly arthritis

3.Chorea:-aneurologicdisorder with involuntary

purposeless rapid movements.

4.Erythema

Marginatum5.Subcutaneous

Nodules

MINOR CRITEIA

1.Fever

2.Arthralgia

3.Previous History of RF4.Increased

a.E.S.R

b.C-Reactive Protein

c.Leucocytosis5.Prlonged PR intravel


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ANTOBODIES AGAINSTBeeta-Haemolytic streptococci group A

Anti-streptolysin O (ASO)

Anti-streptokinase

Anti-streptohyaluronidase Anti DNA ase B


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Rheumatic Heart DiseaseDEFINITION

Rheumatic heart disease is a chroniccondition charectorised by scaring andfibrosis of valves and layers of the heartsecondary to rheumatic fever


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Pathology of RHD


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PathogenesisHypersensitivity reaction.Autoimmune mech. has been proposed

Antibodies directed against the M proteins of certainstrains of streptococci cross-react with tissue

glycoprotein in the heart, joints and other tissues.

Progressive fibrosis of both healing of the acuteinflammatory lesion.

Formation of chronic sequelae

And the turbulence induced by ongoing valvulardeformities.


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Aschoffs Bodies

Aschoffs bodies are nodules formedby a reaction to inflammation withaccompanying swelling andfragmentation of collagen fibers.

As they become age, they becomemore fibrous, and scar tissue is

formed in the myocardium


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Evolution of Aschoff bodies

The aschoff bodies development involves threestages

1.Early stage (Exudative or Degenerative)2.Intermediate stage (proliferative or

granulomatous)3.Late stage (healing or fibrous)


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1.Early(Exudative) Stage:-4th week

Edema of connective tissue + increased acidmucopolysaccharide in the ground

substance.

Accumulation of ground substance.

Separation of collagen fibers.

Collagen fibers become fragmented and

disintegrated.


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2.Intermediative (Proliferative) Stage

4th to 13th week

Proliferation of cells (lymphocytes, plasmacells, 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 aschoff bodies


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ANITSCHKOW CELL

Caterpillar-like

(longitudnal

section)

Owls eye

(cross section)


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Aschoff cells


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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 collagenoustissue is increased

Fibro collagenous scar


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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 plumpmacrophages called anitschkow cells

Pathgnomonic for rheumatic fever or caterpillarcells

+/- aschoff giant cells - multinucleated cells.


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RHEUMATIC PANCARDITIS

1.Rheumatic endocarditis

(a).Rheumatic valvulitis

(b).Rheumatic mural endocarditis2.Rheumatic myocarditis

3.Rheumatic pericarditis


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1(a).Rheumatic Valvulitis

GrosslyAcute

Thickening and loss oftransulency of thevalve leaflets

Gray brown, wateryvegetations

Chronic

Permanent deformityof on one or morevalves (mitral or

aortic) Fish mouth or

button hole Thickening,

shortening and fusionof chordae tendinae

MicroscopicallyAcute

Edema Cellular infiltration

Vegetations of fibrinChronic Thicken by fibrous

tissue withhyalinization(Calcification rarely)

Thickened bloodvessels with narrowedlumina


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Endocardium

Small (1-2mm)irregularvegetations

verrucae alongthe lines of closureof the valves.

Leaflet Thickening

and Fusion of TheTendinous Cords


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Fish Mouth or Button HoleStenosis.


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1(b).Rheumatic mural endocarditis

GrosslyMacCallums patch:-

Lesions of

endocardial surfacein the posteriorwall of the leftatrium just above

posterior leaflet ofthe mitral valve

MicroscopicallyMacCallums patch:-

Edema

Fibrinoid changes incollagen

Cellular infiltrate oflymphocytes

Plasma cells Macrphages

Anitschkow cells


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2.Rheumatic Myocarditis

GrosslyAcute

Left ventricular myocardiumsoft and flabby

Intermediate stage

Interstitial tissue of themyocardium shows smallfoci of necrosis

Late stageFoci of aschoff bodies are

visible

MicroscopicallyAcute

Aschoff nodules arescattered (inter

venticular septum, leftventricle and left atrium)

Intermediate stage

In Aschoff:- Granuloma withcentral fibrinoid necrosis

and surrounded byanitschkow cells

Late stage

Aschoffs bodies are replacedby small fibrous scars


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Myocardium

Shows scattered aschoff bodies withinthe interstitial connective tissue

often perivascular


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3.Rheumatic Pericarditis

GrosslyDeposition of fibrous

exudates (Loss of normalshiny pericardial surface)

Accumulation of fibrousexudates in thepericardial sac

(Bread and butterappearance)

Chronic adhesive pericarditis

Microscopically Fibrosis aschoff

bodies on thesurfaces

Infiltrated subserosal connectivetissue

Adhesions between

visceral and parietalsurfaces


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Pericardium

Fibrinouspericardialexudate

Bread andbutterpericarditis

Generally

resolves withoutsequelae.


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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% .


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Extracardiac Lesions

Polyarthritis

Subcutaneous nodules

Erythema maginatum Chorea minor


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18318109 rheumatic heart disease alfrin

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