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RHEUMATIC HEART DISEASE

Mr. ALFRIN ANTONY Asst. Lecturer DEPARTMENT OF PATHOLOGY

Rheumatic Fever
DEFINITION:-Rheumatic fever is a

systemic, post-streptococal, nonsuppurative 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.Fever 2.Arthralgia 3.Previous History of RF 4.Increased a.E.S.R b.C-Reactive Protein c.Leucocytosis 5.Prlonged PR intravel

1.Carditis 2.Poly arthritis 3.Chorea:- a neurologic

disorder with involuntary purposeless rapid movements.

4.Erythema 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.

Aschoffs Bodies
Aschoffs 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 aschoff bodies

ANITSCHKOW CELL
Caterpillar-like (longitudnal section)

Owls 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 Acute Thickening and loss of transulency of the valve leaflets Gray brown, watery vegetations Chronic Permanent deformity of on one or more valves (mitral or aortic) Fish mouth or button hole Thickening, shortening and fusion of chordae tendinae Microscopically Acute Edema Cellular infiltration Vegetations of fibrin Chronic Thicken by fibrous tissue with hyalinization (Calcification rarely) Thickened blood vessels with narrowed lumina

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
MacCallums patch:-

Microscopically
MacCallums patch: Edema Fibrinoid changes in collagen Cellular infiltrate of lymphocytes Plasma cells Macrphages Anitschkow cells

Lesions of endocardial surface in the posterior wall of the left atrium just above posterior leaflet of the mitral valve

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
Deposition of fibrous exudates (Loss of normal shiny pericardial surface)
Accumulation of fibrous exudates in the pericardial sac (Bread and butter appearance) Chronic adhesive pericarditis

Microscopically Fibrosis aschoff bodies on the surfaces Infiltrated sub serosal connective tissue Adhesions between visceral and parietal surfaces

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