Whipple endocarditis without overt gastrointestinal disease: Report of four cases

Background: Cardiac manifestations of Whipple disease are rarely diagnosed before death. Objective: To describe four patients with endocarditis caused by Tropheryma whippelii who did not have overt gastrointestinal disease. Design: Case series. Setting: Five hospitals in eastern Switzerland. Patients: Three men and one woman undergoing replacement of insufficient he art valves. Measurements: Histologic characteristics of heart valves and intestinal bio psy; broad-range and specific polymerase chain reaction for T. whippelii. Results: Tropheryma whippelii was found in the heart valves (three aortic v alves and one mitral valve) of four patients with culture-negative endocard itis necessitating valve replacement. All patients had arthralgia for diffe rent lengths of time. Only one patient had mild gastrointestinal symptoms. Histologic characteristics of intestinal mucosa were normal in all patients , and polymerase chain reaction on intestinal biopsy was positive for T. wh ippelii in only one patient, who did not have diarrhea, In all patients, ar thralgia resolved promptly after institution of antibiotic therapy. Disease did not recur in any patient after prolonged antibiotic therapy with cotri moxazole. Conclusion: In patients with culture-negative endocarditis, the absence of clinical, microscopic, or microbiological evidence of gastrointestinal dise ase did not rule out T, whippelii.