Atlas of CardioVascular Diseases

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Rheumatic Fever

RHEUMATIC HEART DISEASE:

ACUTE RHEUMATIC FEVER: A hypersensitivity disease typically caused by an abnormal immune response to Strep-A antigens.

• PATHOGENESIS: Immune mechanism involves both B and Tcells.

• CLINICAL: Patient must have at least one of the major symptoms, and two of the minor symptoms of the JONES'S CRITERIA:

• MAJOR JONES'S SYMPTOMS:
  • Carditis: Found in 35% of patients.
  • Polyarthritis: found in 75% of patients.
  • Chorea: Rapid jerky, dyskinetic involuntary movements.
  • Erythema Marginatum
  • Subcutaneous nodules

• MINOR JONES'S SYMPTOMS:

• Fever
• Arthralgia
• History of Rheumatic Fever or Rheumatic Heart Disease
• Acute-Phase Reactants: high sed-rate, or positive C-Reactive Protein, which are evidence of inflammation.
• Prolonged PR interval by ECG.

• RISK-FACTORS: The more severe the initial Streptococcal infection, the more likely it is for patients to develop Rheumatic Fever.

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CHRONIC RHEUMATIC HEART DISEASE:

• PANCARDITIS: Rheumatic heart disease is typically a pancarditis, affecting all three layers of the heart.

• VALVULITIS: Endocarditis affecting Mitral and Aortic Valves.
  • MITRAL VALVEis most common: Chordae Tendineae get fused together, resulting inMITRAL STENOSIS. Rheumatic Fever is the most common cause of mitral stenosis.
    • Stenosis has a fishmouth appearance.
    • You may also at the same time seemitral insufficiency in the same patient.
  • AORTIC VALVE is second most common, which can lead to AORTIC STENOSIS.

• PERICARDITIS:Often seen, but described asBread-and-Butter Pericarditis. This is an inflammatory process, but it is not Constrictive Pericarditis.

• HISTOPATHOLOGY: It is aseptic so no bacteria are present.
  • ASCHOFF BODYis the characteristic finding. A granuloma composed of a central triangular area of fibrinoid necrosis surrounded by histocytes. They heal by scarring.
  • ANITCHKOFF CELLS: Found around the perimeter of the Aschoff bodies. They are also calledCaterpillar cells. Round-to-ovoid nuclei have chromatin disposed in a wavy ribbon resembling a caterpillar.
  
  
  
  

[angelic_doc]

Here is an Aschoff nodule at high magnification. The most characteristic component is the Aschoff giant cell. Several appear here as large cells with two or more nuclei that have prominent nucleoli. Scattered inflammatory cells accompany them and can be mononuclears or occasionally neutrophils.

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[angelic_doc]

Subcutaneous nodule



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Subcutaneous nodule on the extensor surface of elbow of a patient with acute RF

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Boys Afflicted with Chorea

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Sydenham's Chorea

primarily affecting: Face, Tongue & Upper Extremities

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Erythema Marginatum




Erythema marginatumon the trunk, showing erythematous lesions with pale centers and rounded or serpiginous margins


Closer view of erythema marginatum in the same patient

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Aortic valve showing active valvulitis. The valve is slightly thickened and displays small vegetations – "verrucae"

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Myocardial Aschoff body – the cells are large, elongated, with large nuclei; some are multinucleate

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Figure 5a: Two-dimensional color flow Doppler image of the left ventricular inflow of a patient with mitral regurgitation in the four-chamber view (top panel) and two-dimensional parasternal long-axis view (lower panel), showing lack of apposition of the leaflets of the mitral valve during systole (arrow)



Figure 5b: Color flow Doppler study of a patient with aortic regurgitation, as viewed from the parasternal long-axis view (top panel) and two-dimensional four-chamber view, showing hypertrophy and dilatation of the left ventricle (lower panel). LV = left ventricle; LA = left atrium; RV = right ventricle; RA = right atrium; AO = aorta



Figure 6a: Two-dimensional color flow Doppler study of the left ventricular inflow of a patient with mitral stenosis



Figure 6b: Pressure gradient half-time and mitral area is calculated from the diastolic mitral orifice Doppler velocities signals (lower panel). LV = left ventricle; LA = left atrium; RV = right ventricle; RA = right atrium



Figure 7: Two-dimensional parasternal long-axis view of a patient with mitral stenosis, showing thickened valve cusps (arrow), with poor leaflet separation in diastole. Left atrium is enlarged, with a thrombus in the posterior aspect of it. Aortic valve is also stenotic



Figure 8: Stenotic mitral valve seen from left atrium. Both commissures are fused; the cusps are severely thickened. The left atrium is huge. The valve is both incompetent and stenotic



Figure 9: Opened stenotic mitral valve showing thickening distorted cusps, adherent commissures with calcification and thrombus deposition, and thickening, fusion and shortening of chordae tendinae



Figure 10: Stenotic mitral valve seen from left atrium, showing fusion of commissures, thickening and calcification of the cusps

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Chest radiograph of an 8 year old patient with acute carditis before treatment



Same patient after 4 weeks