Ann Thorac Surg 2003;75:1328-1330
© 2003 The Society of Thoracic Surgeons
Case report
Coccidioidal pericarditis: implications of surgical treatment in the elderly
Antonio L. Visbal, MDa,
Patrick A. DeValeria, MDa*,
Janis E. Blair, MDb,
Matthew A. Zarka, MDc,
Louis A. Lanza, MDa
a Division of Cardiovascular and Thoracic Surgery, Scottsdale, AZ, USA
b Division of Infectious Diseases, Scottsdale, AZ, USA
c Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, Arizona, USA
Accepted for publication October 8, 2002.
* Address correspondence to Dr DeValeria, Mayo Clinic Hospital, 5777 East Mayo Boulevard, Phoenix, AZ 85054, USA
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Abstract
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Coccidioidal pericarditis, an uncommonly diagnosed entity, may evolve to a constrictive process. Constrictive coccidioidal pericarditis requires pericardiectomy and antifungal therapy. In the elderly, pericardiectomy may be complicated by coagulopathy and septic shock. Despite potential toxicity, use of antifungal therapy early postoperatively offers the best chance for survival.
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Introduction
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Coccidioides immitis is a fungus found in the desert soil of the southwestern United States. Inhalation of arthrospores in the dust causes 100,000 new infections annually. The infection is subclinical in 60% of cases. Most patients presenting for medical attention have an acute or subacute pneumonia that often spontaneously resolves. Extrapulmonary disease occurs in less than 5% of cases [1].
Pericardial involvement by C. immitis has been rarely reported, and most cases are found in autopsy series [2]. Most of the previously reported patients with constrictive coccidioidal pericarditis (CCP) treated surgically were young and healthy [3]. We describe our experience with CCP and its surgical treatment in the elderly.
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Case reports
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Patient 1
A 73-year-old man presented with congestive heart failure, ascites, peripheral and pulmonary edema, bilateral pleural effusions, and a 20-pound weight loss. He had a history of pulmonary coccidioidomycosis treated with ketoconazole. The coccidioidal complement fixation test was positive at 1:2. Computed tomography of the chest demonstrated severe calcification in the pericardium (Fig 1).
Echocardiography revealed biatrial enlargement and normal left ventricular size and ejection fraction. The pericardium was thickened, encasing the right and left ventricles with marked adhesions. Cardiac catheterization demonstrated a mean right atrial pressure of 23 mm Hg, pulmonary artery pressure of 44/23 mm Hg, and mean pulmonary wedge pressure of 23 mm Hg. Prominent x and y descending patterns were present.
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Fig 1. Computed tomography scan of chest, demonstrating severe calcification surrounding and constricting the pericardium.
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The patient underwent median sternotomy and complete pericardiectomy. Cardiopulmonary bypass was required because of the severe constrictive process. The pericardium was heavily calcified, nearly 2-cm thick, and firmly adherent to the myocardium with cystic cavities containing caseous material. Histologic examination revealed a suppurative pericarditis, caseating granulomas, spherules, and endospores (Fig 2).
The first 24 hours postoperatively were associated with septic shock. Two grams of amphotericin B was administered over 7 weeks; therapy was then continued with oral fluconazole. Septic shock resolved by postoperative day 7, and he went home after an 8-week hospitalization. Fourteen months later, he is improved and continues oral fluconazole therapy.
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Fig 2. Coccidioides immitis spherule (center) surrounded by acute inflammation, necrosis, macrophages, and granulation tissue (hematoxylin-eosin, x200).
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Patient 2
A 78-year-old man with a history of non-Hodgkin lymphoma and renal insufficiency presented with increasing dyspnea on exertion, progressive angina, congestive heart failure, and presyncope. Six years before, culture-confirmed coccidioidal pneumonia was treated with fluconazole. His current coccidioidal complement fixation test was positive at 1:16. Echocardiography demonstrated a bicuspid aortic valve with an area of 0.9 cm2 and moderate insufficiency. There was pericardial thickening without significant effusion or constriction. Cardiac catheterization demonstrated normal chamber pressures.
At operation, the pericardium was scarred, thickened, and adherent to the epicardium and myocardium. The patient underwent bioprosthetic aortic valve replacement, two-vessel coronary artery bypass grafting, and complete pericardiectomy. Histologic examination revealed severe calcification without evidence of coccidioidomycosis. The postoperative period was complicated by sepsis and multiorgan failure. He died on postoperative day 7. Autopsy revealed microabscesses and spherules of C. immitis in lung, lymph nodes, myocardium, and residual pericardium (Fig 3).
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Fig 3. Coccidioides immitis spherules within microabscess adjacent to myocardium (hematoxylin-eosin, x200).
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Comment
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Coccidioidal pericarditis is uncommon and may present acutely with rapidly progressing effusive-constrictive pericarditis or as a chronic constrictive process [3, 4]. Autopsy series of disseminated coccidioidomycosis identified pericardial infection in 5% to 14% [2]. The active granulomatous inflammation of the pericardium becomes calcified, resulting in a nondistensible pericardium with constrictive physiology.
Diagnosis of coccidioidomycosis can be problematic. It can be definitively diagnosed by culturing C. immitis from a clinical specimen or by demonstrating spherules in a pathologic specimen. For patients with suspected coccidioidal pericarditis, these methods require an invasive procedure. Skin tests are insensitive and no longer available. Therefore, serologic tests are useful for diagnosis. Qualitative tests include immunodiffusion and enzyme-linked immunosorbent assays. The complement fixation test is a quantitative assay that detects IgG antibodies arising later in the course of the infection. The concentration of complement-fixing antibodies roughly correlates with the extent of infection and can be monitored serially to follow the course of infection. Titers of 1:32 should prompt an evaluation for disseminated infection [1].
With or without appropriate antifungal therapy, patients with coccidioidal pericarditis have significant mortality. The diagnosis may be established post mortem, as in our second patient. Previously, coccidioidal pericarditis has been described in young, otherwise healthy adults [3, 4]. Elderly patients with any form of coccidioidomycosis have a 26.8% mortality. Chronic illness, such as chronic obstructive pulmonary disease and renal, hepatic, and vascular diseases, increases the risk of death [5]. Both of our elderly patients had many of these risk factors.
Interventions for CCP may include pericardiocentesis, pericardial window, or complete pericardiectomy. The former two have been associated with little or transient improvement. Pericardiectomy for CCP has been successful in young patients [3]. The goal is complete resection, freeing the heart from the constrictive process.
Coagulopathy and septic shock complicated the postoperative period in both of our patients. Septic shock due to coccidioidomycosis has a remarkably high mortality. One series of 8 patients with coccidioidal sepsis had 100% mortality despite antifungal therapy. Coccidioidomycosis was recognized ante mortem in only 62% [6].
Therapy for coccidioidomycosis includes azoles or amphotericin B. Azoles are often selected for patients with subacute or chronic clinical presentations. Amphotericin B is preferred for severe disease. It is given at a dosage of 0.5 to 0.7 mg/kg per day, and this may be changed to an every-other-day regimen or to azole therapy once clinical improvement has occurred [7]. Amphotericin B is associated with renal toxicity, a worrisome issue after cardiopulmonary bypass. This should not inhibit its use, however, because patients who survived after pericardiectomy for CCP received amphotericin B early postoperatively.
In conclusion, coccidioidal pericarditis is an uncommon entity that may evolve to a constrictive process. Preoperative diagnosis may be difficult to establish. Physicians should suspect CCP in patients presenting with constrictive pericarditis who live in areas endemic for coccidioidomycosis. Septic shock and death may complicate surgical excision of the pericardium. Despite potential toxicity, early use of antifungal therapy in the postoperative period offers the best chance for survival.
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References
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- Galgiani J.N. Coccidioidomycosis. West J Med 1993;159:153-171.[Medline]
- Schwartz E.L., Waldmann E.B., Payne R.M., Goldfarb D., Kinard S.A. Coccidioidal pericarditis. Chest 1976;70:670-672.[Abstract/Free Full Text]
- Faul J.L., Hoang K., Schmoker J., Vagelos R.H., Berry G.J. Constrictive pericarditis due to coccidioidomycosis. Ann Thorac Surg 1999;68:1407-1409.[Abstract/Free Full Text]
- Chowdhury J.K., Habibzadeh A. Disseminated coccidioidomycosis with pericarditis. Successful treatment with amphotericin B. Chest 1977;71:533-535.[Abstract/Free Full Text]
- Arsura E.L. The association of age and mortality in coccidioidomycosis. J Am Geriatr Soc 1997;45:532-533.
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Abstract
Introduction
