Clinical SummaryEdit
This 21-year-old black male with sickle cell anemia had recurrent attacks of acute rheumatic fever beginning at age 14. Mitral insufficiency and stenosis were present by age 16. On prophylactic antibiotics, the patient had no evidence of recurrence until three weeks before his final admission, when an upper respiratory infection developed. A few weeks later he developed acute migratory polyarthritis. This was associated with rapid deterioration of cardiac function and death.
Autopsy FindingsEdit
At autopsy, the heart was enlarged--weighing 675 grams--especially the left atrium. Both the aortic and mitral valves showed fibrosis as well as the fresh, tiny verrucae characteristic of acute rheumatic fever.
ImagesEdit
This is a gross photograph of mitral valve demonstrating marked thickening and fibrosis of the valve leaflet. There are also numerous foci of fibrinoid necrosis within the cusps and friable vegetations (verrucae) along the lines of closure (arrows). These irregular, warty projections are found at sites of erosion on the inflamed endocardial surface. The verrucae probably result from the precipitation of fibrin where the leaflets impinge on each other.
This high-power photomicrograph of myocardium shows the cellular detail of an Aschoff body. Aschoff bodies are foci of fibrinoid necrosis surrounded by lymphocytes, macrophages, an occasional plasma cell, and plump “activated” histiocytes called Anitschkow cells or Aschoff cells (arrows). These distinctive cells have abundant amphophilic cytoplasm and central round-to-ovoid nuclei in which the chromatin is disposed in a central, slender, wavy ribbon resembling a caterpillar (hence the designation “caterpillar cells”).
Aortic insufficiency refers to the inability of the aortic valve to close properly, thus allowing regurgitation of blood into the left ventricle during diastole -- i.e., volume overload. Disease of the aortic valve leaflets is a common cause of insufficiency.
A normal heart weighs 300 grams (range: 270 to 360 grams).
Friable material is easily crumbled.