Cardiac Wegener’s granulomatosis masquerading as left atrial myxoma

doi:10.1016/S0003-4975(02)04662-3

The Annals of Thoracic Surgery

Volume 75, Issue 4, April 2003, Pages 1321-1323

https://doi.org/10.1016/S0003-4975(02)04662-3 Get rights and content

Abstract

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.

Section snippets

Comment

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,

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Unusual cardiac complications of Wegener’s granulomatosis
Chest
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Citation Excerpt :
Mechanisms of valve disruption include valvular masses, some mimicking endocarditis, and valve thickening. Intracardiac atrial and ventricular masses are also uncommon and are limited to case reports.91 Subclinical cardiac involvement however may be much more frequent.
Granulomatous endocarditis in a patient with Wegener's granulomatosis
2011, Journal of Cardiology Cases
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Granulomatous valvular lesions have been frequently reported in histopathological studies of WG. Herbst et al. performed surgery to replace the mitral valve of a patient suffering from a large mass (10 mm) on the anterior mitral leaflet, masquerading as left atrial myxoma with moderate mitral regurgitation [2]. The patient's condition deteriorated post-operatively, and histopathological examination revealed an intense inflammatory infiltrate with aggregates of neutrophils and eosinophils, leading to the diagnosis of WG.
Echocardiographic examination of patients with granulomatous endocarditis in patients with Wegener's granulomatosis (WG) reveals vegetation-like lesions that may be misdiagnosed as infective endocarditis resulting in inappropriate therapy. Three-dimensional transesophageal echocardiography aids differential diagnosis. Here, we report the case of a WG patient with associated mitral and aortic granulomatous endocarditis. Although the patient was treated with prednisolone and cyclophosphamide, serial echocardiography did not reveal any significant changes in disease course.
Cardiac manifestations of Wegener's granulomatosis: Case report and review of the literature
2010, Journal of Cardiology Cases
Wegener's granulomatosis, first characterized as a clinical syndrome in 1936, is a rare form of vasculitis of the small- and medium-sized blood vessels affecting mainly the upper and lower respiratory tracts as well as the kidneys. This review article describes the case of a 45-year-old man who developed aortic regurgitation and third degree heart block secondary to Wegener's granulomatosis. He subsequently presented to our institution 3 years later with pulmonary hemorrhage. The ensuing literature review reveals that historically clinical cardiac involvement in Wegener's has been rare, particularly with valvular lesions. However, recent literature has shown an increase in reported cases of cardiac valvular lesions in Wegener's. The presented case report along with recently reported case reports highlight the morbidity and mortality associated with such cardiac lesions. We believe routine cardiovascular supervision including echocardiography and electrocardiograms are important for the screening and monitoring of patients with Wegener's granulomatosis.
Mitral valve involvement in Wegener's granulomatosis
2010, Annals of Thoracic Surgery
Citation Excerpt :
The mitral mass is a rare complication of Wegener's granulomatosis and has only been reported once in the literature [5].
Wegener's granulomatosis is an autoimmune condition resulting in the granulomatous vasculitis of small-to-medium-sized vessels, and is characterized by granulomatous lesions in the renal and respiratory systems. Cardiac involvement in Wegener's granulomatosis has been previously reported. However, involvement of cardiac valves is extremely rare. We present a patient with Wegener's granulomatosis with an extensive mitral mass extending to the aortic valve.
Mitral regurgitation associated with Wegener's granulomatosis
2009, Annales de Cardiologie et d'Angeiologie
La maladie de Wegener (MW) est une granulomatose systémique. Les organes les plus fréquemment atteints sont le tractus respiratoire dans sa globalité et le rein. Les manifestations cardiaques dans le cadre de cette vascularite sont rares.
Nous publions le cas d’un patient présentant une insuffisance mitrale de grade 4 associée à une MW sévère.
Le bilan lésionnel peropératoire a trouvé une perforation du feuillet antérieur de la valve mitrale (A2) sans preuve d’endocardite infectieuse. L’examen histopathologique d’A2 a mis en évidence des nodules dégénératifs myxoïdes, alors que les prélèvements bactériologiques étaient négatifs. La proximité de la lésion avec le bord libre d’A2, ainsi que l’état général du patient nous ont conduit à la mise en place d’une bioprothèse mitrale. Six mois après l’intervention, le patient est asymptomatique et le contrôle échographique est satisfaisant.
La MW est une vascularite nécrosante auto-immune rare. Les formes cardiaques sont rares. Le cœur peut être atteint dans sa globalité par la MW. Les valvulopathies semblent être les atteintes les moins fréquentes avec des lésions préférentiellement aortiques. La place des investigations cardiologiques semblent être indispensables lors du diagnostic initial de la MW, mais également pendant la surveillance.
Wegener's granulomatosis (WG) is a granulomatous disease that can affect many organ systems. The most frequently involved organs include the upper and lower respiratory tract as well as the kidney. Cardiac involvement is rare.
We report the case of a patient with grade 4 mitral insufficiency associated with severe WG.
Surgical analysis of the mitral valve revealed perforation of the anterior leaflet without evidence of endocarditis. Pathological examination of the anterior mitral leaflet revealed myxoid degeneration nodules and bacteriological examination was negative. As the perforated lesion was very close to the free margin of the anterior leaflet, valve replacement appeared a reasonable surgical option in a poor surgical candidate. Six months after the operation, the patient is doing well.
Wegener's granulomatosis is an autoimmune necrotizing vasculitis that can affect many organ systems. Cardiac involvement is rare. Heart in his globality can be affected by WG in many different ways. Cardiac valvular involvement in WG is rare. The aortic valve seems to be more frequently affected. Cardiologic investigations should have an important place at diagnosis and supervision.

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Case reportCardiac Wegener’s granulomatosis masquerading as left atrial myxoma

Abstract

Section snippets

Comment

Am J Kidney Dis

Semin Arthritis Rheum

Mayo Clin Proc

Chest

Case report
Cardiac Wegener’s granulomatosis masquerading as left atrial myxoma