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Cardiac Whipple Disease: Identification of Whipple Bacillus by Electron Microscopy in the Myocardium of a Patient before Death
- Frank E. Silvestry, MD;
- Back Kim, MD;
- Bonnie J. Pollack, MD;
- Julia E. Haimowitz, MD;
- Richard K. Murray, MD;
- Emma E. Furth, MD;
- Harvey L. Nisenbaum, MD;
- Michael L. Kochman, MD;
- Neil Freedman, MD;
- Rogelio Pine, MD; and
- Howard C. Herrmann, MD
- From the University of Pennsylvania Medical Center, Philadelphia, Pennsylvania; and Princeton Medical Center, Princeton, New Jersey. Requests for Reprints: Howard C. Herrmann, MD, Cardiovascular Division, University of Pennsylvania Medical Center, 9 Founder's Pavilion, 3400 Spruce Street, Philadelphia, PA 19104. Current Author Addresses: Drs. Silvestry and Herrmann: Cardiovascular Division, University of Pennsylvania Medical Center, 9 Founder's Pavilion, 3400 Spruce Street, Philadelphia, PA 19104.
Whipple disease was first described in 1907 in a 36-year-old patient with diarrhea, abdominal pain, weight loss, and polyarthritis [1]. The causative agent, now called Tropheryma whippelii, is closely related to the actinomycetes [2]. Whipple disease usually affects the intestinal tract but may involve other organ systems, including pulmonary and neurologic tissue [3, 4]. Macrophages that yield positive results on periodic acid-Schiff staining are characteristically seen in the lamina propria of the small bowel, and electron microscopy shows rod-shaped bacillary bodies that are believed to be the causative agent.
Although overt cardiac involvement is rare, case reports have documented constrictive pericarditis [5], endocarditis [6], and myocarditis [7]. Postmortem studies of some patients without clinical cardiac involvement have described gross deformities of the valves and pericardium [5] and macrophages that yield positive results on periodic acid-Schiff staining in the myocardium [5, 8]. Electron microscopy has been used after death to document rod-shaped bacillary bodies in the myocardium of two patients without clinical cardiac involvement who died of untreated Whipple disease. We report an atypical presentation of Whipple disease with predominant cardiac involvement and show that Whipple bacillus was evident in myocardial tissue by electron microscopy before death.
Case Report
A 44-year-old woman was referred for evaluation of heart failure. She had been healthy until 9 months before presentation, at which time she noted dyspnea on exertion, increased abdominal girth, edema of the lower extremities, and pain in the right upper quadrant after a viral illness. Echocardiography had shown biatrial enlargement and mild global dysfunction of the left ventricle, which suggested viral myocarditis. The patient's condition improved with administration of furosemide and enalapril, but she then developed additional symptoms, including anorexia; weight loss; …
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