Suggested Readings

1. FeldmanT. Rheumatic heart disease. Curr Opin Cardiol. 1996; 11:126-30.

2. Shulman ST. Complications of streptococcal pharyngitis. Pediatr Infect Dis J. 1994; 13(1 Suppl l):S70-4; discussion S78-9.

3. Veasy LG, Hill HR. Immunologic and clinical correlations in rheumatic fever and rheumatic heart disease. Pediatr Infect Dis J. 1997; 16:400-7.

4. Rose AG. Etiology of valvular heart disease. Curr Opin Cardiol. 1996; 11:98-113.

5. BaxleyWA. Aortic valve disease. Curr Opin Cardiol. 1994; 9:152-7.

6. Stollerman GH. Rheumatogenic streptococci and autoimmunity. Clin Immunol Immunopathol. 1991; 61:131-42.

7. Adler Y, Fink N, Spector D, Wiser I, Sagie A. Mitral annulus calcification- a window to diffuse atherosclerosis of the vascular system. Atherosclerosis. 2001; 155:1-8.



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Figure 29. Cross section of the heart revealing severe left ventricular hypertrophy due to aortic valve stenosis.

Figure 30. Rheumatic heart disease involving the aortic valve (viewedfrom the aorta) with a superimposed fresh thrombus.
Figure 31. Rheumatic heart disease. Resected mitral valve with thickened chordae and
Figure 32. Rheumatic heart disease. In this example the mitral valve shows thickened and
Figure 33. Rheumatic heart disease, histologic section. Aschoffbody (center)(Hematoxylin
Figure 34. Rheumatic heart disease with Anitschkow cells. These cells have oval nuclei and centrally condensed chromatin. Due to the pattern of nuclear chromatin, these cells are also known as caterpillar cells in longitudinal section and owls' eye in cross section (Hematoxylin and Eosin, 100X)

Clinical Summary

This is the case of a 60 year old Hispanic man who presented to the hospital with complaints of shortness of breath. A few months earlier, a mid-systolic click and a late systolic murmur III/VI, radiating to the axilla, were discovered on a routine physical exam. An echocardiogram was done revealing significant mitral regurgitation. Catheterization showed severe mitral valve regurgitation with flaring posterior leaflet, normal ejection fraction and 50% right coronary artery stenosis. Mitral valvuloplasty was performed with the post-surgical complication of atrial fibrillation and flutter for which the patient was started on the anticoagulant coumadin. Ten days post-surgery, the patient was found at home unconscious with agonal respiration. CPR was performed to no avail and the patient was pronounced dead on arrival to the emergency room.

Gross Description

The patient measured 176 cm and weighed 81 kg. A healing 14 cm long sternotomy scar was seen on the right sternal border. The heart weighed 575 g. The pericardial surfaces had a fibrinous exudate consistent with pericarditis, and the sac contained 500 cc of sero-sanguineous fluid. Both atria showed well healed surgical incisions. The left atrium was dilated. The right atrium was of normal size. Mitral valve annuloplasty was evident, and the cusps and leaflets showed fibromyxomatous degeneration. Excess tissue was present, and the chordae tendineae were thickened and elongated (Figure 35). The remaining valves were unremarkable. The left ventricle was mildly hypertrophied (wall thickness 1.6 cm). The right ventricle had a normal chamber size. The lungs weighed 900 g combined, and were congested. Intimal plaques were noted in the pulmonary vasculature. The other significant post-mortem finding was the presence of multiple subacute and remote infarcts affecting cerebral cortex, subcortical white matter and thalamus.

Microscopic Description

Sections from the pericardium revealed extensive granulation tissue with thick deposits of fibrin, focal hemorrhage and giant cell proliferation. Myocyte hypertrophy with interstitial fibrosis and adipose tissue infiltration was noted in the myocardium. The mitral valve showed fibromyxomatous changes (Figure 36). Coronary arteriosclerosis was moderate with no evidence of plaque, hemorrhage or thrombotic occlusions. The aorta exhibited thickening of the wall with proliferation of the intima and extensive deposition of cholesterol with focal ulceration in the areas of calcification. Sections from the central nervous system confirmed the gross impression of multiple infarcts.

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