Ann Thorac Surg 2003;75:1321-1323
© 2003 The Society of Thoracic Surgeons
Case report
Cardiac Wegener’s granulomatosis masquerading as left atrial myxoma
Anne Herbst, MDa,
Mary T. Padilla, MDb,
Anil R. Prasad, MDb,
Monty C. Morales, MDc,
Jack G. Copeland, MDd*
a University of Ulm, Ulm, Germany
b Department of Pathology, University of Arizona, , Tucson, Arizona, USA
c Tucson Heart Center, Tuscon, Arizona, USA
d University of Arizona Sarver Heart Center, Tucson, Arizona, USA
Accepted for publication September 27, 2002.
* Address reprint requests to Dr Copeland, University of Arizona Sarver Heart Center, 1501 N Campbell Ave, Tucson, AZ 85724-5071, USA
e-mail: jgcbriez{at}aol.com
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Abstract
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A 56-year-old woman was referred with mitral regurgitation, left ventricular dysfunction, and a sessile mass on the anterior leaflet of her mitral valve. The initial impression from echocardiography was that she had a left atrial myxoma. At operation, we found an intense inflammatory process diagnosed as Wegener’s granulomatosis. It also involved the aortic valve and contiguous myocardium.
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Introduction
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In 1936, Wegener [1] described a granulomatous process involving the upper and lower respiratory tract accompanied by glomerulonephritis. The necrotizing granulomatous vasculitis of small- to medium-sized vessels seen in Wegener’s granulomatosis is now thought to be an autoimmune process that can involve many organs including the heart. Antiinflammatory therapy with methotrexate and steroids usually leads to remission. As with most rare cardiac diseases, making a diagnosis at once may be the most difficult and most important step toward a cure.
A 56-year-old woman being evaluated for increasing fatigue, chest pain, shortness of breath, and dyspnea on exertion increasing over a 6-year period was found on transesophageal echocardiography to have 3+ central jet of mitral regurgitation and a mass 1 cm thick involving most of the anterior leaflet of the mitral valve as well as the fibrous aortic continuity (Fig 1).
The anterior leaflet barely moved. Her left ventricular ejection fraction was 45% to 55%. Coronary angiography revealed a 50% to 60% narrowing in the mid-left anterior descending artery. Her history was positive for chronic sinusitis, polyarthritis, hypertension, uterine fibroids, and an ovarian cyst. The arthritic deformity and discomfort of her hands was severe enough that she anticipated a hand operation. She had no history compatible with bacterial endocarditis or emboli.
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Fig 1. Transesophageal echocardiogram showing anterior leaflet of mitral valve thickened to approximately 1 cm (dotted oval).
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This sessile mass, although not resembling the usual polypoid anatomy of a left atrial myxoma, was considered to be most likely a variant of myxoma. However, at operation, nearly the entire anterior leaflet was 10-mm thick. It was homogeneously fibrotic and invaded the septum (Fig 2).
We removed the entire mass, resecting it well up into the left side of the noncoronary aortic cusp and well into the interventricular septum. On frozen section, the mass was found to be benign and fibrotic. Permanent sections (Fig 3)
of the mitral and aortic valve specimens demonstrated an intense inflammatory infiltrate with aggregates of neutrophils and eosinophils infiltrating collagen and forming small microabscesses surrounded by histiocytes, lymphocytes, and plasma cells. In addition, there was extensive fibroplasia with occasional calcification and vascular proliferation. A few small vessels were infiltrated by inflammatory cells. Occasional poorly-formed giant cells were seen. The inflammatory process infiltrated adjacent myocardium and produced a reactive myocyte atypia. Stains for fungi, bacteria, or other organisms were negative. The histologic findings were consistent with Wegener’s granulomatosis.
The valves were replaced with porcine prostheses (21-mm aortic and 33-mm mitral). The very large mitral prosthesis in this 65 kg female was necessitated by the extent of our resection. Stitches were passed through the adjacent portion of the sewing ring of the mitral prosthesis during the aortic implant. Postoperative laboratory evaluation revealed slight elevations of C-reactive protein and antinuclear antibodies, but c-ANCA and p-ANCA were negative (< 1:16). Transthoracic echocardiography showed left ventricular hypertrophy, an ejection fraction of 45% to 55%, an estimated pulmonary artery pressure of 30 to 35 mm Hg, and normal prosthetic valve function. The patient was discharged on mild doses of diuretics and a low dose beta blocker.
One month postoperatively the patient presented with worsening shortness of breath, dyspnea on exertion, and paroxysmal nocturnal dyspnea. Echocardiography confirmed adequate prosthetic valve function, but a left ventricular ejection fraction of 20% to 30%. Cardiac catheterization reconfirmed the mild left anterior descending artery disease, and documented a left ventricular ejection fraction of 35% to 40%, a pulmonary artery wedge mean pressure of 16 mm Hg, a pulmonary artery systolic pressure of 40 mm Hg, and a right atrial mean pressure of 8 mm Hg. Chest and abdominal computed tomographic scans were negative except for small bilateral pleural effusions.
On the basis of these findings and in view of the aggressive inflammatory changes in the surgical specimen, a diagnosis of Wegener’s granulomatosis with cardiac involvement including valvulitis and cardiomyopathy was made. Diuretic therapy was increased, ACE inhibitor therapy was started, and the patient was begun on therapy with prednisone 60 mg every day, as well as intravenous immune globulin and Cytoxan (Bristol-Myers Squibb, Princeton, NJ). The anticipated remission rate of Wegener’s granulomatosis with this therapy is more than 90%.
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Comment
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Wegener’s granulomatosis is a necrotizing granulomatous vasculitis of small- to medium-sized vessels. Classic manifestations include upper and lower respiratory tract involvement along with glomerulonephritis [1, 2]. The four major diagnostic criteria in Wegener’s granulomatosis are (1) nasal or oral inflammation, (2) abnormal chest roentgenograms, (3) urinary sediment, and (4) typical histology [3]. Elevation of C-reactive protein level, sedimentation rate and serologic markers such as c-ANCA, antinuclear antibodies, and anti-DNA antibodies are typical [4].
Cardiac involvement has been reported in 6% to 44% of Wegener’s cases [2]. About 50% of these cases with cardiac involvement present with pericarditis or coronary arteritis, 25% with myocarditis, 21% endocarditis or valvulitis, 17% with an involvement of the conduction system, and 11% with myocardial infarction [5]. More severe manifestations include congestive cardiomyopathy [6], complete heart block [5], and pericardial tamponade [7]. Valvular involvement is most commonly aortic; Morelli and colleagues [8] reported 9 patients with Wegener’s granulomatosis, 8 of whom presented with aortic valve abnormalities. Three patients underwent surgical replacement of the aortic valve.We believe that involvement of the mitral valve with regurgitation has not been reported.
This patient presented with history of chronic sinusitis and polyarthritis, had mild elevations of C-reactive protein and antinuclear antibodies, and had a consistent histology. We believe that her valvulitis and cardiomyopathy were caused by Wegener’s granulomatosis. It was the initial diagnosis of a possible left atrial myxoma that led to the diagnosis and treatment.
This uncommon presentation has provided us with a fascinating diagnostic and therapeutic challenge. Awareness of this experience with some unique cardiac manifestations of Wegener’s granulomatosis may help others evaluate and manage unusual cardiac masses and cardiomyopathies.
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References
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- Wegener F. Ueber generalisierte, septische Gefaesserkrankungen. Verh Dtsch Ges Pathol 1936;36:202-210.
- Grant S.C., Levy R.D., Venning M.C., Ward C., Brooks N.H. Wegener’s granulomatosis and the heart. Br Heart J 1994;71:82-86.[Abstract/Free Full Text]
- Leavitt R.Y., Fauci A.S., Bloch D.A., et al. The American college of rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 1990;33:1101-1107.[Medline]
- Lesavre P. Antineutrophil cytoplasmic autoantibodies antigen specificity. Am J Kidney Dis 1991;18:159-163.[Medline]
- Forstot J.Z., Overlie P.A., Neufeld G.K., Harmon C.E., Forstot S.L. Cardiac complications of Wegener’s granulomatosis: a case report of complete heart block and review of the literature. Semin Arthritis Rheum 1980;10:148-154.[Medline]
- Delevaux I., Hoen B., Selton-Suty C., Canton P. Relapsing congestive cardiomyopathy in Wegener’s granulomatosis. Mayo Clin Proc 1997;72:848-850.[Abstract]
- Schiavone W.A., Ahmad M., Ockner S.A. Unusual cardiac complications of Wegener’s granulomatosis. Chest 1985;88:745-748.[Abstract/Free Full Text]
- Morelli S., Gurgo Di Castelmenardo A.M., Conti F., et al. Cardiac involvement in patients with Wegener’s granulomatosis. Rheumatol Int 2000;19:209-212.[Medline]
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Abstract
Introduction
