HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Todd A. Shuman John S. Pirolo Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Elkins, C. Articles by Pirolo, J. S. Search for Related Content PubMed PubMed Citation Articles by Elkins, C. Articles by Pirolo, J. S.

Ann Thorac Surg 1999;67:250-251
© 1999 The Society of Thoracic Surgeons

---

Case Reports

Cardiac Whipple’s disease without digestive symptoms

^a Cardiovascular Surgery Associates, PC, Nashville, Tennessee, USA

Accepted for publication June 28, 1998.

Address reprint requests to Dr Shuman, Cardiovascular Surgery Associates, PC, 4230 Harding Rd, Suite 501, Nashville, TN 37205

	Abstract

Top Abstract Introduction Comment References

 
Whipple’s disease is a systemic illness that can affect the heart, causing pericarditis, myocarditis, and valvular endocarditis. We describe a 43-year-old man without gastrointestinal symptoms who underwent mitral and aortic valve replacement for endocarditis, in whom a diagnosis of Whipple’s disease was made at operation.

	Introduction

Top Abstract Introduction Comment References

 
Whipple’s disease is a rare, chronic, multisystem illness caused by Tropheryma whippelii [1]. The classical presentation consists of a triad of gastrointestinal symptoms—diarrhea, weight loss, and malabsorption. Cardiac involvement as the primary clinical presentation is rare. We describe a 43-year-old man with aortic and mitral valvular endocarditis caused by Whipple’s disease in the absence of significant gastrointestinal symptoms.

The patient was in his normal state of health until 4 years before admission, when he developed a left lower extremity deep venous thrombosis and received anticoagulation. One month later, he presented with left-sided neglect, slurred speech, and left hemiparesis. A computed tomographic head scan showed no abnormalities, however, a magnetic resonance imaging scan demonstrated a right middle cerebral artery infarct, and arteriography revealed occluded posterior temporal branches. The patient’s neurologic symptoms resolved within 48 hours. Transesophageal echocardiography demonstrated only a small, 5-mm cylindric density at the tip of the anterior leaflet of the mitral valve. Further workup revealed iron deficiency anemia; subsequently, gastrointestinal workup results were negative. Upper and lower endoscopy and small bowel follow-through findings were normal.

The patient did well until 12 months before admission, when he presented with a right lower extremity deep vein thrombosis while receiving therapeutic anticoagulation. Hypercoagulation workup results were unrevealing, and an inferior vena caval filter was placed. During the 8 months before admission, the patient noted fatigue, dyspnea on exertion, and a 25-lb (11.25-kg) weight loss. In addition, he developed pleuritic chest pain, a nonproductive cough, and recurrent low-grade fevers. Outpatient laboratory data revealed renal insufficiency (creatinine 3.1 mg/dL), hypercalcemia (11.2 mg/dL), and anemia (hemoglobin, 7.3 g/dL). Vasculitis workup results were negative. He was admitted to an outside hospital for renal biopsy and was noted to be febrile. Blood culture results were negative, and renal biopsy demonstrated interstitial fibrosis, tubular atrophy, and mesangial prominence without evidence of immune complex deposition. An abdominal computed tomographic scan demonstrated periaortic lymphadenopathy. The patient was transferred to our institution for further evaluation.

On physical examination the patient was afebrile. Axillary and anterior cervical nodes were palpable. A grade II to VI systolic murmur was heard at the left sternal border with radiation to the axilla. The remainder of the examination showed normal findings. Chest X-ray film showed no abnormalities, and the electrocardiogram demonstrated normal sinus rhythm with borderline left ventricular hypertrophy. Laboratory studies revealed a blood-urea-nitrogen level of 32 mg/dL, a creatinine level of 2.8 mg/dL, a hematocrit of 31%, a white blood cell count of 10,100, and an erythrocyte sedimentation rate of 90 mm/h. Transesophageal echocardiography demonstrated 4+ mitral regurgitation and 2 to 3+ aortic insufficiency with vegetations on both the mitral and aortic valves. Blood culture results were negative. Axillary lymph node biopsy showed numerous epithelial granulomas.

The patient subsequently underwent aortic and mitral valve replacement. On opening of the pericardium, dense fibrous adhesions were encountered with numerous 1 to 2-cm nodules attached to the myocardium. Examination of the valves revealed multiple pedunculated nodules on the atrial side of the mitral valve (Fig 1, upper panel), and the ventricular side of the aortic valve (Fig 1, lower panel). Valve replacement with St. Jude prostheses was performed. The patient tolerated the procedure well. He was extubated and without inotropic support, 12 hours postoperatively. Pathologic analysis of the pericardial lesions, aortic valve, and mitral valve demonstrated macrophages containing periodic acid-Schiff–positive, diastase-resistant material. Polymerase chain reaction assay was positive for T. whippelii. By virtue of renal insufficiency and a penicillin allergy, the patient was treated with a course of minocycline followed by oral trimethoprim and sulfamethoxazole. Seven months after operation the patient reported improved endurance, less dyspnea, and a 15-pound (6.75-kg) weight gain. He has remained afebrile. His most recent laboratory data are as follows: blood-urea-nitrogen 16 mg/dL, creatinine 1.8 mg/dL, hematocrit 40%, erythrocyte sedimentation rate 8 mm/h, and calcium 9.6.

View larger version (84K): [in this window] [in a new window]   Fig 1. (Upper panel) Mitral valve with anterior leaflet at top. (Lower panel) Aortic valve with right coronary leaflet indicated by arrow.

	Comment

Top Abstract Introduction Comment References

Valvular pathologic features of Whipple’s disease are now known to be distinct from rheumatic disease and are the etiologic causes of some cases of culture-negative endocarditis. The valvular lesions most frequently involve the mitral valve but occasionally involve the aortic valve as well. Involvement of a porcine bioprosthesis has been reported [6]. The clinical manifestations of valvular involvement range from an asymptomatic murmur to congestive heart failure. Most autopsy series report thickening and fibrosis of the valve leaflet, with chordal shortening similar to rheumatic heart disease. In our patient both the mitral and aortic leaflets were thickened and invested with multiple nodules.

Successful treatment of valvular Whipple’s disease requires valve replacement with a mechanical rather than a bioprosthetic valve to prevent recurrence. Systemic therapy subsequently involves treatment with trimethoprim and sulfamethoxazole. No recurrence has been reported with the use of this regimen [3].

	References

Top Abstract Introduction Comment References

 

1. Relman D.A., Schmidt T.M., MacDermott R.P., Falkow S. Identification of the uncultured bacillus of Whipple’s disease. N Engl J Med 1992;327:293-301.[Abstract]
2. Whipple G.H. A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues. Bull Johns Hopkins Hosp 1907;18:382-391.
3. Durand D.V., Lecomte C., Cathebras P., Rousset H., Godeau P. Whipple disease: clinical review of 52 cases. Medicine (Baltimore) 1997;76:170-184.[Medline]
4. McAllister H.A., Fenoglio J.J. Cardiac involvement in Whipple’s disease. Circulation 1975;52:152-156.[Abstract/Free Full Text]
5. Bostwick D.G., Bensch K.G., Burke J.S., et al. Whipple’s disease presenting as aortic insufficiency. N Engl J Med 1981;305:995-998.[Medline]
6. Ratliff N.B., McMahon J.T., Naab T.J., Cosgrove D.M. Whipple’s disease in the porcine leaflets of a Carpentier-Edwards prosthetic mitral valve. N Engl J Med 1984;311:902-903.[Medline]

This article has been cited by other articles:

				M. Marin, P. Munoz, M. Sanchez, M. del Rosal, M. Rodriguez-Creixems, E. Bouza, and on behalf of Grupo de Apoyo al Manejo de la Endoca Tropheryma whipplei Infective Endocarditis as the Only Manifestation of Whipple's Disease J. Clin. Microbiol., June 1, 2007; 45(6): 2078 - 2081. [Abstract] [Full Text] [PDF]

				D West, S Hutcheon, R Kain, T Reid, S Walton, and K Buchan Whipple's endocarditis J R Soc Med, August 1, 2005; 98(8): 362 - 364. [Full Text] [PDF]

				F. Dutly and M. Altwegg Whipple's Disease and ""Tropheryma whippelii"" Clin. Microbiol. Rev., July 1, 2001; 14(3): 561 - 583. [Abstract] [Full Text] [PDF]

				M. A. Smith Whipple Endocarditis without Gastrointestinal Disease Ann Intern Med, April 4, 2000; 132(7): 595 - 595. [Full Text] [PDF]

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Todd A. Shuman John S. Pirolo Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Elkins, C. Articles by Pirolo, J. S. Search for Related Content PubMed PubMed Citation Articles by Elkins, C. Articles by Pirolo, J. S.