Rheumatic heart disease

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Rheumatic heart disease

• Dr. Gehan Mohammed Dr. Abdelaty Shawky

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Endocarditis
Types (1). Non-infective endocarditis A.
Rheumatic endocarditis. B. Verrucous (libman
sack) endocarditis with SLE. C. Non-Bacterial
thrombotic endocarditis. (2). Infective
endocarditis A. Acute Infective endocarditis B.
Sub-acute Infective endocarditis
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Rheumatic endocarditis
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RHEUMATIC HEART DISEASE

• Rheumatic fever is a post-streptococcal
  immune-mediated inflammatory disease affect heart
  and extra-cardiac sites e.g. joints, skin,
  brain.
• The incidence and mortality of rheumatic fever
  has declined over the past 30 years (due to
  improved socioeconomic condition and rapid
  diagnosis and treatment of strep. pharyngitis).

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Pathogenesis

• An acute attack of streptococcal pharyngitis by
  group A beta-hemolytic streptococci.
• Within 2-4 weeks after this attack
  anti-streptococcal antibodies are formed and
  attack the heart and the extra-cardiac sites.
• The mechanism of this immune reaction is not yet
  understood, however, the most accepted hypothesis
  is antigenic similarity hypothesis.

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Strep throat
Antibody production
Antibody cross-reaction with heart
vegetations
Aschoff body
pericarditis
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Pathological features of Rheumatic Heart
disease

• The characteristic lesion of acute rheumatic
  fever is the Aschoff body, consisting of a focus
  of necrosis (representing the site of antigen
  antibody reaction) surrounded by activated
  histiocytes and lymphocytes. The histiocytes may
  be mononuclear or multinuclear, and are referred
  to as Anitschkow's or Aschoff cells.

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• These foci may be found in the pericardium, the
  myocardium, or uncommonly in the valves.
• They ultimately "heal" by fibrosis.

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• - The disease passes into two phases
• A. Acute phase
• ? acute rheumatic pancarditis (inflammation of
  endocardium, myocardium and pericardium)
• Myocarditis.
• Pericarditis "bread and butter", due to
  fibrinous inflammation
• Endocarditis edema, inflammation and fibrin
  deposits on valve leaflets (vegetations) along
  lines of closure. Mostly mitral and aortic
  valves. Aschoff nodules are uncommon in the
  valves.

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• B. Chronic phase
• Acute changes may resolve completely or progress
  to scarring and development of chronic valvular
  deformities many years after the acute disease.

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Aschoffs body
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Rheumatic vegetations
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Aortic valve stenosis
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Extra-cardiac lesions of rheumatic fever

• These lesions are acute and resolve completely
  without disability.
• 1. Migratory polyarthritis It causes "fleeting
  arthritis" in the large joints, self limited, no
  chronic deformities.
• 2. Skin skin rheumatic nodules, erythema
  marginatum.
• 3. Sydenham chorea a neurologic disorder with
  involuntary purposeless, rapid movements.

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Clinical features of Acute Rheumatic Fever

• Occurs 10 days to 6 weeks after pharyngitis
• Peak incidence 5-15 years.
• Cardiac manifestations pericardial friction
  rubs, weak heart sounds, tachycardia and
  arrhythmias.
• Extra-cardiac fever, migratory polyarthritis of
  large joints, arthralgia, skin lesions, chorea.
• Pharyngeal culture may be negative, but anti
  streptolysin O (ASO) titer will be high.

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Jones criteria

• A. Major criteria
• Carditis.
• Polyarthritis
• Sydenhams chorea.
• Erythema marginatum.
• Subcutaneous nodules.
• B. Minor criteria
• Previous history of rheumatic fever.
• Arthralgia.
• Fever.
• Lab tests indicative of inflammation ESR
  (erythrocyte sedimentation rate), CRP (C-Reactive
  protein), leukocytosis.
• ECG changes.

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Diagnosis of rheumatic fever

• Need 2 major criteria or 1 major and 2 minor
  criteria.

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CHRONIC RHEUMATIC HEART DISEASE

• - Endocarditis heals by progressive fibrosis.
  Chronic scarring of the valves constitutes the
  most important long-term sequelae of rheumatic
  fever, and usually becomes clinically manifest
  decades after the acute process.
• Left sided valves (mitral and aortic) are more
  commonly involved than the right.
• Fibrosis of valve leaflets --gt stenosis.

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• Fibrosis of chordae tendonae --gt regurgitation
  (improper closure).
• Other cardiac complications
• Subacute bacterial endocarditis.
• Arrhythmia.
• Chronic heart failure.

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• In valve stenosis
• Leaflets are thickened, fibrotic, shrunken with
  fusion
• Dilatation and hypertrophy of left atrium.
• Secondary deposition of Ca
• fish mouth (button hole) stenosis - i.e. the
  stenosed valve looks like a fish's mouth
• Lungs are firm and heavy (chronic passive
  congestion).

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• Pulmonary hypertension
• Right side of the heart may be affected later
  (right ventricular hypertrophy).
• In valve incompetence (regurgitation)
• Retracted leaflets.
• Left ventricular hypertrophy and dilatation.

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Mitral valve stenosis

• Leads to left atrial dilatation and failure,
  chronic venous congestion of the lung, lung
  fibrosis, pulmonary hypertension and chronic
  right sided heart failure.

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Mitral stenosis with commissural fusion
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Mitral valve incompetence

• Leads to left ventricular dilatation and failure,
  left atrial dilatation and failure, chronic
  pulmonary congestion, lung fibrosis, pulmonary
  hypertension and chronic right sided heart
  failure.

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Nonbacterial Thrombotic EndocarditisNBTE
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• Characterized by the deposition of small masses
  of fibrin, platelets, and other blood components
  on the leaflets of the cardiac valves. There is
  no infective organism (sterile).
• Aortic valve is most common site.
• Clinically asymptomatic, if large may embolize.
• Pathogenesis/ association
• Subtle endothelial abnormalities.
• Hypercoagulability.
• Association with malignancy (50).

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Verrucous (Libman-Sacks) endocarditis
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• Less common, non-infective endocarditis
  attributable to elevated levels of circulating
  immune complexes may occur in patients with
  systemic lupus erythematosus

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Infective Endocarditis
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• Definition infection of the cardiac valves or
  mural surface of the endocardium, resulting in
  the formation septic vegetations (thrombi).
• Divided into
• a. Acute infective endocarditis.
• b. Subacute infective endocarditis.

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Acute infective endocarditis

• Etiology
• Acute suppurative inflammation that affects
  healthy valves.
• Organisms Highly virulent as staph. Aureus,
  strept.hemolyticus and gonococci.

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Lesions

• Mitral aortic valves are most commonly
  affected. Tricuspid is affected in IV drug
  abusers.
• The mural endocardium may be also affected.
• The affected valve and mural endocardium show
  acute suppurative inflammation vegetations.

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• Vegetations are
• N/E multiple, large, yellowish, friable found
  anywhere on the cusps.
• M/P the vegetations consist of platelet,
  fibrin, bacteria, numerous neutrophils pus
  cells.
• Myocardial shows microabscesses.
• The pericardial sac is filled with pus.

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Complications

• 1. Embolic complications
• Detached septic vegetations leads to systemic
  pyemia.
• 2. Toxemic complications
• Severe toxemia

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Prognosis

• Rapidly fatal due to
• Severe toxemia (septicemia).
• Cusp perforation (acute heart failure).

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Subacute infective endocarditis

• Etiology
• Subacute inflammation that affects abnormal
  valves in
• Rheumatic valvulitis
• Congenitally abnormal valves.
• Prosthetic valves.
• Caused by Less virulent bacteria as
  strept.viridans.

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Lesions

• Mitral aortic valves are commonly affected.
•   The mural endocardium may be also affected
• The affected valve and the mural endocardium
  show the lesion of the corresponding disease
  (e.g. rheumatic, congenital) vegetations.

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• Vegetations are
• N/E multiple, large, gray, friable found
  anywhere on the cusps.
• M/P the vegetations consist of platelets,
  fibrin, bacteria and some inflammatory cells
  mainly histiocytes.
• The Myocardium shows degenerative changes.

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Complications

• 1. Embolic complications
• Infarctions in kidney, spleen and brain, retina,
  heart.
• Mycotic aneurysms mainly in cerebral and
  mesenteric.
• Petechial hemorrhage in skin, mucous membranes
  and serous membranes.
• Oslers nodules small. tender, intracutaneous
  nodules in pulps of fingers toes.

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• 2. Toxemic complications
• Moderate toxemia fever, anemia, clubbing of
  fingers, splenomegaly, petechial hemorrhage and
  focal glomerulonephritis (flea bitten kidney)

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Prognosis

• Heal by fibrosis leads to valve lesion either
  stenosis or incompetence.

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Endocarditis of the mitral valve (subacute,
caused by Streptococcus viridens). vegetations
are denoted by arrows.
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C. Histologic appearance of vegetation of
endocarditis with extensive acute inflammatory
cells and fibrin. Bacterial organisms were
demonstrated by tissue Gram stain.
Slide 13.42
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References Robbins and Cotrans Pathologic
Basis of Disease. Seventh edition.
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Thanks