Images in Cardiovascular Medicine

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1 Images in Cardiovascular Medicine Numerous Small Vegetations Revealing Libman-Sacks Endocarditis in Catastrophic Antiphospholipid Syndrome Hideo Yamamoto, MD; Tamaki Iwade, MD; Ryuji Nakano, MD; Masahiro Mohri, MD; Yoshifumi Amari, MD; Mitsuru Noma, MD A 48-year-old man was referred to our cardiology department for the treatment of acute myocardial infarction. He had no risk factors for arteriosclerosis. Two months before this admission, he had been admitted to another hospital because of stroke with right hemiparesis; 1 month before this admission, he developed acute renal failure and hemodialysis was started. Chest radiograph demonstrated cardiomegaly and pulmonary congestion (Figure 1A). The ECG demonstrated a QS pattern in the V 1 through V 3 leads with ST elevation and coronary T waves in the V 4 through V 6 leads and left atrial overload in the V 1 lead (Figure 1B). Transthoracic echocardiography (TTE) showed diffuse left ventricular hypokinesis with severe hypokinesis of the septum and anterior wall (Figure 2). Emergency coronary angiogram revealed normal epicardial coronary arteries (Figure 3). The increase in creatine kinase, diffuse left ventricular hypokinesis by TTE, and normal coronary angiogram indicated acute myocardial infarction at the level of small coronary arteries. TTE showed severe mitral regurgitation and numerous small vegetations on the atrial surface of the mitral valve (Figure 4). TTE also showed moderate aortic regurgitation and clusters of small vegetations on the ventricular surface of the aortic valve (Figure 5). Vegetations were apposing on both the anterior and posterior leaflets of the mitral valve and on all 3 leaflets of the aortic valve at the valve closure contact line away from the leaflet tips. The day after coronary angiography, severe thrombocytopenia occurred. The titer of anticardiolipin antibody was highly increased. Catastrophic antiphospholipid syndrome was diagnosed. The anticoagulation, pulse therapy of methylpredonisolone, and cyclophosphamide were done, and therapeutic plasma exchanges were repeatedly performed. The patient, however, died of multiorgan failure that included encephalopathy. Autopsy confirmed the TTE findings of the mitral and aortic valves (Figure 6). Numerous thrombi were present in the intramyocardial small vessels associated with multiple small foci of necrosis (Figure 7). Similar lesions were seen in the small vessels of the kidneys and brain (Figure 8). No evidence was found of vasculitis. These findings suggested that multiorgan failure was caused by systemic thrombosis in the small vessels but not by vasculitis. Only 6% of reported cases with catastrophic antiphospholipid syndrome had vegetations revealing Libman-Sacks endocarditis. However, these suggest possible microthrombotic occlusive disease in the setting of catastrophic antiphospholipid syndrome. 1,2 None. Disclosures References 1. Nihoyannopoulos P, Gomez PM, Joshi J, Loizou S, Walport MJ, Oakley CM. Cardiac abnormalities in systemic lupus erythematosus: association with raised anticardiolipin antibodies. Circulation. 1990;82: Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med. 2002;346: From the Departments of Cardiology (H.Y., T.I, M.M., Y.A., M.N.) and Pathology (R.N.), Kyushu Kosei-Nenkin Hospital, Kitakyushu, Japan. Correspondence to Hideo Yamamoto, MD, Department of Cardiology, Kyushu Kosei-Nenkin Hospital, Kishinoura, Yahata-Nishiku, Kitakyushu , Japan. hideo.yamamoto@qkn-hosp.jp (Circulation. 2007;116:e531-e535.) 2007 American Heart Association, Inc. Circulation is available at DOI: /CIRCULATIONAHA e531

2 e532 Circulation November 13, 2007 Figure 1. Chest radiograph (A) and ECG (B) on admission day. Figure 2. M-mode echocardiogram.

3 Yamamoto et al Catastrophic Antiphospholipid Syndrome e533 Figure 3. Coronary angiograms (A and B, left coronary artery; C, right coronary artery). Figure 4. Two-dimensional echocardiograms of the mitral valve (A, long-axis; B, short-axis; and C, apical 4-chamber views).

4 e534 Circulation November 13, 2007 Figure 5. Two-dimensional echocardiograms of the aortic valve (A, B, and D, long-axis; C, short-axis views). Figure 6. Autopsy image demonstrates the clusters of numerous small vegetations on the ventricular surface of the aortic valve (white arrows) and multiple small vegetations on the atrial surface of the mitral valve (green arrows).

5 Yamamoto et al Catastrophic Antiphospholipid Syndrome e535 Figure 7. Myocardial tissue demonstrating scattered small scar (A). Fresh (B) and organized (C) thrombus in the intramyocardial small vessels surrounded by myocardial necrosis. Figure 8. Kidney (A and B) and brain (C) tissue demonstrating organized (A and B) and fresh (C) thrombus in the small vessels.