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Ann Thorac Surg 1999;68:1407-1409
© 1999 The Society of Thoracic Surgeons


Case Reports

Constrictive pericarditis due to Coccidiomycosis

John L. Faul, MDa, Khanh Hoang, MDb, Joseph Schmoker, MDc, Randall H. Vagelos, MDb, Gerald J. Berry, MDd

a Divisions of Pulmonary and Critical Care Medicine, Stanford, California, USA
b Division of Cardiology, Stanford, California, USA
c Division of Cardiothoracic Surgery, Stanford, California, USA
d Department of Pathology, Stanford University Medical Center, Stanford, California, USA

Address reprint requests to Dr Berry, Department of Pathology, Stanford University Medical Center, Stanford, CA 94305-5407


    Abstract
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 Abstract
 Introduction
 Comment
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Coccidiomycosis is a fungal infection that rarely causes cardiac disease. Constrictive pericarditis in the setting of disseminated coccidiomycosis can be fatal, despite antifungal therapy and pericardiectomy. We report on a patient with constrictive pericarditis due to localized infection by Coccidioides immitis. The patient underwent successful surgical pericardiectomy and antifungal chemotherapy, and remains well 1 year later.


    Introduction
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 Abstract
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Constrictive pericarditis can be a serious sequela of chest trauma, radiation therapy, or granulomatous disease and is a rare and potentially lethal complication of fungal infection [1]. The fungus Coccidioides immitis is endemic in the southwestern United States and can lead to pulmonary infection and multiorgan dissemination [2, 3]. This article reports a case of constrictive pericarditis attributable to coccidiomycosis that was successfully managed with surgical pericardiectomy and antifungal chemotherapy.

A 20-year-old man was referred with a 6-month history of dyspnea and chest tightness. He reported a 15-pound weight gain over 1 month, without a change in appetite. His past medical history was unremarkable and he did not abuse alcohol, illicit drugs, or tobacco. He was a college student who was active in sports until the onset of his illness. He denied orthopnea and pedal edema. His family history was negative for cardiac disease. On admission, the pulse was regular, 88 beats/min, blood pressure 100/80 mm Hg, without a pulsus paradoxus, and he was afebrile. The jugular venous pressure was raised at 14 cm with a positive Kussmaul’s sign. The heart sounds were normal without a third heart sound, or evidence of pericardial rub or knock. The lung bases were dull. There was no ascites and no pedal edema. A chest radiograph revealed a normal heart size and bilateral pleural effusions (left larger than right). Electrocardiography showed widespread symmetric T-wave inversion in the precordial leads. Echocardiography demonstrated normal right and left ventricular size, and thickness, but reduced left ventricular systolic function. The pericardium appeared thickened (Fig 1). Cardiac catheterization revealed elevated right atrial pressure (14 mm Hg), with prominent x and y descent patterns. The right ventricular pressure was 30/14 mm Hg, pulmonary artery pressure was 30/14 mm Hg, and the mean pulmonary capillary wedge pressure was 14 mm Hg. The estimated cardiac output was 2.9 L/min (cardiac index, 1.8 L/min per m2) and no oxygen saturation step-up was observed from the superior vena cava to the pulmonary artery. The aortic pressure was 110/70 mm Hg and the coronary arteries were normal. The left ventricular ejection fraction was 49%. Simultaneous measurements of left and right ventricular diastolic pressures were perfectly equivalent, without separation during the Valsalva maneuver or premature ventricular complex (PVC) induction. Right ventricular endocardial biopsies showed no histologic evidence of myocarditis, hemochromatosis, amyloidosis, or granulomatous inflammation. A diagnosis of constrictive pericarditis was made and the patient was referred for operation.



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Fig 1. (Upper left) Transthoracic echocardiogram (two-dimensional, four-chamber view) showing thickened pericardium (arrowheads). (Upper right) Surgical pericardiectomy specimen showing thick fibrotic parietal pericardium. (Lower left) Low-power magnification showing numerous caseating and solid granulomas surrounded by inflammatory cells (hematoxylin and eosin stain, x125). (Lower right) Granuloma composed of epithelioid histiocytes and Langerhans giant cells (hematoxylin and eosin stain, x300). (Inset) Mature spherule of Coccidioides immitis containing uninucleate endospores (Grocott methenamine silver stain, x600).

 
The patient underwent surgical removal of the pericardium through a sternotomy. A thickened fibrotic parietal pericardium, measuring up to 1.4 cm in thickness (Fig 1) was found to overlie a gelatinous visceral pericardium that was adherent to the myocardium. Both the parietal and visceral pericardial layers were removed anterior to the phrenic nerves, resulting in an immediate decrease in right atrial pressures. Histologic examination of the resected pericardium revealed numerous granulomas containing spherules of Coccidioides immitis (Fig 1). Many colonies of Coccidioides immitis mold grew in tissue and fluid cultures of his resected pericardial specimens. Postoperative serum complement fixation tests were positive for both immunoglobulins M and G to Coccidioides immitis (titer, 1:8). A lumbar puncture revealed no evidence of central nervous system dissemination. He was treated with amphotericin B (0.7 mg/kg per day) for 6 weeks followed by oral fluconazole for 3 months. Treatment with amphotericin was associated with self-limiting rigors, tremors, and renal failure. Ten months postoperatively, two-dimensional echocardiography demonstrated a normal left ventricular ejection fraction (57% to 60%) with no evidence of constriction. Eighteen months after pericardiectomy the patient is asymptomatic and has returned to normal daily activities.


    Comment
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 Abstract
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 Comment
 References
 
Cardiac involvement by coccidiomycosis can be life threatening [2, 3], but is rarely diagnosed premortem or prethoracotomy. In this patient, the diagnosis was not considered preoperatively because our patient had no complaints suggestive of infection. Coccidioides immitis commonly infects the lung, lymph nodes, spleen, skin, and liver, but there was no evidence of extracardiac involvement in our patient.

Coccidiomycosis frequently involves all layers of the heart [2, 4]. Fibrogranulomatous pericarditis, miliary granulomas, and cardiac muscle abscesses can occur [5]. Autopsy studies suggest that 5% to 15% of patients with disseminated coccidiomycosis have lesions in the myocardium [2, 5] and rare cases of endocarditis attributable to coccidiomycosis have also been reported [6]. Pericarditis can result from rupture of a superficial granuloma located just beneath the pericardium or by direct extension from the lung, pleura, or mediastinal lymph nodes [4]. Although no evidence of coccidiomycosis was seen on right ventricular myocardial biopsy in this patient, the grossly abnormal electrocardiograph and reduced left ventricular systolic function (evident on echocardiography) were suggestive of myocardial involvement [7].

Currently, there are no data on the possible long-term effects of coccidioidal myocarditis and pericarditis. It is unknown whether therapy with antifungal agents, in addition to surgical pericardiectomy, can lead to a cure of pericardial coccidioidal infections but treatment with oral fluconazole and amphotericin on an empiric basis would seem worthwhile [8]. Schwartz and colleagues [5] have described coccidioidal constrictive pericarditis that resulted from a chronic (> 1 year) pericardial effusion. In that report pericardiocentesis and pericardial window proved ineffective at preventing the development of constrictive pericarditis. Our patient demonstrated an occult progression to constriction in the absence of clinically overt pericarditis, pericardial effusion, or cardiac tamponade.

Most cases of coccidioidal pericarditis have been reported before the availability of amphotericin B therapy. In this patient, the surgical resection of constrictive pericarditis due to coccidiomycosis proved an effective management strategy, although the long-term clinical outlook is unclear. Current therapeutic options for fungal pericarditis include local installation of antifungal chemotherapy, intravenous amphotericin B, and oral agents. Antiinflammatory therapies, including corticosteroids, are sometimes used in the management of idiopathic constrictive pericarditis [1]. The current case care underscores the need to evaluate the possibility of alternative diagnoses, such as tuberculous and fungal infections, before initiating potentially deleterious immunosuppressive therapy.


    References
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 Abstract
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 Comment
 References
 

  1. Osterberg L., Vagelos R., Atwood J.E. Constrictive pericarditis. West J Med 1998;169(4):232-239.[Medline]
  2. Chapman M.G., Kaplan L. Cardiac involvement in coccidiomycosis. Am J Med 1957;23:87-98.[Medline]
  3. Bayer A.S., Yoshikawa T.T., Galpin J.E., Guze L.B. Unusual syndromes of coccidioidomycosis. Medicine 1976;55:131-152.[Medline]
  4. Forbus W.D., Bestebreurtje A.M. Coccidiomycosis. Milit Surg 1946;99:653-719.
  5. Schwartz E.L., Waldmann E.B., Payne R.M., Goldfarb D., Kinard S.A. Coccidioidal pericarditis. Chest 1976;70:670-672.[Medline]
  6. Evans N., Ball H.A. Coccidioidal granuloma. JAMA 1929;93:1881-1885.
  7. Oudiz R., Mahaisavariya P., Peng S.K., et al. Disseminated coccidioidomycosis with rapid progression to effusive-constrictive pericarditis. J Am Soc Echocardiogr 1995;8:947-952.[Medline]
  8. Chowdhury J.K., Habibzadeh A. Disseminated coccidioidomycosis with pericarditis. Successful treatment with amphotericin B. Chest 1977;71:533-535.[Medline]
Accepted for publication March 24, 1999.




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This Article
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