Unusual Manifestation of Libman-Sacks Endocarditis in Systemic Lupus Erythematosus
A 23-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.
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.
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