Whipple Disease

Patients with Whipple disease have been reported to have symptoms of cardiovascular

Figure 2.10. Gross photograph of excised anterior mitral leaflet with large bulky fungal vegetation. This was present on both sides of the leaflet (the back is shown).The infecting organism was Aspergillus. Ruler = 1 cm.

disease in 58% of cases. However, at autopsy 79% have gross evidence of cardiac involvement, and of these 53% have valvular disease [30,31]. The mitral valve is the most common valve affected, with the aortic and tricuspid valves also reported to be involved at times. There are periodic acid Schiff reaction (PAS)-positive macrophages on light microscopic examination and bacilliform organisms on electron microscopy. Polymerase chain reaction performed on blood may be helpful for diagnosis [22]. The organism is a Gram-positive actin-omycete, Tropheryma whippelii [31]. The infection may lead to fibrosis and chronic inflammation giving rise to a valve with similar appearance to a post-rheumatic one. The deposits may be nodular and are often not calcified. Similar pathological changes are found in the myocardium, endocardium, and pericardium [32]. History of gastrointestinal disorder should be questioned for, as the diagnosis is usually made by small intestinal biopsy.

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