Circulation. 2003;107:e202-e204
doi: 10.1161/01.CIR.0000063926.32375.D3
(Circulation. 2003;107:e202.)
© 2003 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Unusual Manifestation of Libman-Sacks Endocarditis in Systemic Lupus Erythematosus
Carsten Schneider, MD;
Edda Bahlmann, MD;
Matthias Antz, MD;
Rudolph Bauer, MD;
Jacobus Reimers, MD;
Werner Raut, MD;
Christian Busch, MD;
Frank Rathjen, MD;
Roland Moll, MD;
Karl-Heinz Kuck, MD
From the Department of Cardiology, St Georg Hospital, Hamburg (C.S., E.B., M.A., R.B., J.R., W.R., K.-H.K.); Department of Internal Medicine, Federal Armed Forces Hospital, Hamburg (C.B., F.R.); and Department of Pathology (R.M.), Philipps-University Marburg, Germany.
Correspondence to Karl-Heinz Kuck, MD, Department of Internal Medicine II–Cardiology, Allgemeines Krankenhaus St Georg, Lohmühlenstrasse 5, 20099 Hamburg, Germany. E-mail Dr_C_Schneider{at}hotmail.com
A23-year-old man presented with complaints of exertional dyspnea, symmetrical arthritis, and facial exanthema. Auscultation revealed a loud systolic murmur (IV/VI) at the apex of the heart. Transthoracic and transesophageal echocardiography showed severe mitral regurgitation caused by a mass involving the posterior leaflet of the mitral valve and extending to the posterolateral and inferior wall of the left ventricle. The anterior leaflet of the mitral valve was thickened. The unchanged regional signal intensity and delineation after use of an intravenous ultrasound contrast agent (Optison) (Figure 1, Movies A through D) suggested the presence of a left ventricular thrombus. Cardiac magnetic resonance imaging confirmed this suspicion (Figure 2). Coronary angiography showed no coronary anomalies and no signs of coronary arteritis. Endomyocardial left ventricular biopsies revealed thrombotic material with macrophage cell infiltration (Figure 3). Laboratory evaluation was notable for a positive antinuclear and anti–double-stranded DNA antibody. Antiphospholipid syndrome testing (lupus anticoagulants and anticardiolipin antibodies) was negative. On the basis of all findings, Libman-Sacks endocarditis in systemic lupus erythematosus could be confirmed. After immunosuppressive treatment with high-dose prednisolone and 5 cycles of cyclophosphamide, the left ventricular mass decreased. Because of the destruction of the posterior leaflet of the mitral valve, regurgitation volume remained severe (Figure 4, Movies A and B). Consequently, operative repair of the mitral valve was initiated.
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Figure 1. Transesophageal echocardiography. A, Transgastric midventricular view shows echodense mass extending from the anterior to the posterolateral and inferior wall of the left ventricle. B, Same view with use of perfluoropropane-filled microbubbles (Optison) and assessment of unchanged regional signal intensity and delineation of the thrombus formation. C, Four-chamber view revealing thrombotic-involved posterior leaflet and the thickened anterior leaflet of the mitral valve. D, Transesophageal 4-chamber view showing severe mitral regurgitation.
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Figure 2. Cardiac magnetic resonance imaging. A, Longitudinal cardiac view in diastole shows thrombotic masses of the posterior leaflet with low density. B, Angulated transversal view with assessment of endocardial thrombotic material of low density attaching to the lateral left ventricular wall; in addition, pericardial and pleural effusions are evident.
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Figure 3. Photomicrograph of the endomyocardium. A, Layer of organizing fibrin (hematoxylin-eosin stain); the subjacent myocardial tissue shows degeneration. B, Isolated thrombotic particle with singular immigrated macrophages shown as a sign of a recent thrombotic event (hematoxylin-eosin stain). C, Immunohistochemistry reveals a layer of fibrin (brown) in separated thrombotic material.
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Figure 4. Transthoracic echocardiography 8 weeks after immunosuppressive treatment and anticoagulation. A, Parasternal long-axis view showing severe mitral regurgitation. B, Parasternal short-axis view showing decreased mass in left ventricle.
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Footnotes
Movie versions of Figures 1 (A through D) and 4 (A and B) are available in the online-only Data Supplement at http://www.circulationaha.org.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
