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Cardiac Aspergillosis in Patients With Acquired Immunodeficiency Syndrome: A Case Report and Review of the Literature

Archives of Pathology & Laboratory Medicine, Apr 2005 by Xie, Linjun, Gebre, Wondwossen, Szabo, Katarina, Lin, Jen H

Cardiac aspergillosis is uncommon in patients with acquired immunodeficiency syndrome (AIDS) in the absence of open heart surgery. We report a unique case of a 62-year-old man with AIDS who developed Aspergillus pancarditis with Aspergillus vegetations on mitral valve without evidence of pulmonary aspergillosis. There was extensive embolization to the brain and multiple foci of Aspergillus infection in kidneys and adrenal glands. There are only 10 documented cases of cardiac aspergillosis in the literature (1966-2003) in severely immunocompromised AIDS patients with CD4 T-lymphocyte counts ranging from 10 to 121 cells/µL. The cardiac aspergillosis could result from invasive pulmonary aspergillosis, either by hematogenous dissemination or by direct invasion, and skin Aspergillus infection can be carried through the bloodstream to the right heart in intravenous drug abusers. Most of the reported cases of cardiac aspergillosis were diagnosed at autopsy. Mortality among AIDS patients with cardiac aspergillosis is 100%, despite appropriate therapy.

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(Arch Pathol Lab Med. 2005;129:511-515)

Aspergillosis was initially included in acquired immunodeficiency syndrome (AIDS)-defining opportunistic infections by the Centers for Disease Control in 1982.1 However, it was subsequently excluded from the revised case definition of AIDS, because patients with CD4 lymphopenia showed no predisposition for or increase in the incidence of developing invasive aspergillosis.2 However, the world literature that we reviewed, including our case, shows that AIDS patients with cardiac aspergillosis without exception had marked CD4 T lymphopenia (Table). There is close association between marked CD4 T lymphopenia and increased incidence of developing cardiac aspergillosis in patients with AIDS. These findings support the view that cardiac aspergillosis should be reconsidered as an AIDS-defining condition.

Although cardiac aspergillosis is very rare in patients with AIDS who have not undergone open heart surgery, it carries a very high mortality rate. The first case of cardiac aspergillosis as an AIDS-related opportunistic infection was reported in 1985.3 Ever since, there have been a few reports, mostly from North America and Europe. Patients with cardiac involvement have frequently previously experienced pulmonary aspergillosis with Aspcrgillus endocarditis and myocarditis (Table). Our patient with AIDS had Aspergilhis pancarditis (endocarditis, myocarditis, pericarditis with pericardial effusion) embolizing and infecting multiple organs in the absence of pulmonary aspergillosis. The unique case is presented in this report. We also reviewed the literature from 1966 to 2003 and did a thorough analysis of the 10 cases previously reported of AIDS patients with Aspergilhis infection in the heart.

REPORT OF A CASE

A 62-year-old male AIDS patient with recent Pneumocystis carinii pneumonia was admitted to our hospital for fever, shortness of breath, and productive cough. The tests showed CD4 lymphocyte count of 79 cells/µL and human immunodeficiency virus (HIV) viral load of

MATERIALS AND METHODS

A complete autopsy was performed. The gross and histologie findings of all organs were available for studies. Hematoxylineosin-stained sections and Gomori methenamine silver-stained sections were evaluated. Aspergillus was microscopically confirmed based on the characteristic features of small and regular fungal hyphae with dichotomous branching at acute angles and distinct cross-septa.

We did a MEDLINE search for reports of cardiac aspergillosis. All reported cases in the literature, including the present case, are summarized in the Table.3-10 Ten of the total 11 patients are male. In one case, the patient's gender was not mentioned. Patient ages ranged from 29 to 62 years, with mean of 41 years. All cases were confirmed as cardiac aspergillosis at autopsy. Aspcrgillus fumigatus was recovered from 6 patients, either from premortem blood culture and biopsy tissues or at postmortem tissue culture. The mean interval between the diagnosis of AIDS and aspergillosis was 3.5 months (range, O to 13 months). Details of cardiovascular features of documented AIDS patients with cardiac aspergillosis are recorded in the Table.

PATHOLOGIC FINDINGS

At autopsy, there was 100 mL of serosanguineous pericardial effusion. The heart weighed 660 g and showed biventricular hypertrophy and right ventricular dilatation. The mitral valve showed a large, polylobular, fungating, reddish vegetation (3.0 × 2.0 × 1.5 cm) on the atrial surface of the posterior leaflet, extending to the left atrial endocardium just above the annulus of the posterior leaflet (Figures 1 and 2). Multiple small vegetations (0.5 cm in greatest dimension each) were attached along the line of closure of the posterior leaflet (Figure 2). The vegetations did not extend onto the chordae. The mitral valve itself, except for the vegetations, and the rest of the cardiac valves were unremarkable. The myocardium revealed a pale-gray ovoid infarcted area (0.7 × 0.6 × 0.4 cm) in the ventricular septum. All coronary arteries were unremarkable. Microscopic findings of the heart include endocarditis with characteristic Aspergillus fungal elements in necrotic debris on both the anterior and posterior leaflets, Aspergillus myocarditis with micro-abscess formations in the left ventricle and left atrium (Figure 3), and foci of Aspergillus aggregates in necrotic debris present in the thickened pericardium in the left ventricle and atrium (Figure 4), as well as microscopic old myocardial fibrous foci in the ventricular septum and left ventricle. The specificity of the morphologic identification of Aspergillus in tissue was based on the regular hyphae with dichotomous branching at 45° angles and distinct cross-septa (Figure 3, inset in Figures 4 and 6). The extensive cerebral infarction in the left temporal parieto-occipital lobes (Figure 5) and focal infarct in the right basal ganglia were found grossly. There was subfalcine herniation (inset in Figure 5). Microscopically, infarcted areas demonstrated coagulative necrosis, containing sporadic Aspergillus fungal clusters with scattered areas of micro-abscesses. The left middle cerebral artery was almost completely occluded by fungal emboli. There was diffuse mycotic arteritis in the left middle and posterior cerebral arteries (Figure 6). The posterior cerebral arteries also showed severe atherosclerosis, with approximately 85% luminal narrowing. There was approximately 1000 mL of serosanguineous pleural effusion bilaterally. The lung revealed bilateral diffuse interstitial pulmonary fibrosis, pulmonary edema, hemorrhage, and congestion. No fungal elements were identified by histopathology or tissue cultures of both lungs after extensive sampling. Kidneys showed extensive chronic interstitial inflammation with micro-abscesses containing Aspergillus. The right adrenal gland showed focal aspergillosis.

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Cardiac Aspergillosis in Patients With Acquired Immunodeficiency Syndrome: A Case Report and Review of the Literature

Archives of Pathology & Laboratory Medicine, Apr 2005 by Xie, Linjun, Gebre, Wondwossen, Szabo, Katarina, Lin, Jen H

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