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75-Year-Old Woman With Multiple Systemic Emboli

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 75-year-old woman recently diagnosed as having stage IV lung adenocarcinoma was admitted for dyspnea and weakness. There was no history of illicit drug use, autoimmune disease, or cardiopulmonary disease. Physical examination was remarkable only for asthenia. Cardiac examination was normal. Chest computed tomography (CT) demonstrated multiple pulmonary nodules, a spiculated mass in the right medial upper lobe, vertebral metastases, and small bilateral subsegmental pulmonary emboli. Cranial CT showed scattered subacute embolic cortical infarcts and dural metastases. Serum troponin was 2.6 ng/mL (type 2 myocardial infarction) with nonspecific electrocardiographic changes. Transthoracic echocardiogram showed normal biventricular function with normal wall motion and shaggy, bulky masses on the tips of both mitral valve leaflets (Figure) (Video 1). A saline contrast bubble study was negative for an intracardiac shunt, and commercial intravenous contrast showed no clot. Transesophageal echocardiogram showed large, 1.4 × 0.5-cm, frondlike, mobile masses on the atrial surface of the mitral leaflets with mild mitral regurgitation. In addition, a large 1.1  × 1.6-cm multilobulated, mobile mass was seen on the subvalvular chordae of the tricuspid valve, with trace tricuspid regurgitation. Gated cardiac CT angiogram confirmed mobile hypoattenuating masses on the mitral valve leaflets that prolapsed to the left atrium during systole (Video 2). No myocardial, pulmonary vein, or pericardial involvement was seen.

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Nonbacterial thrombotic endocarditis

C. Treat with full-dose anticoagulation

Results of blood cultures and serologies were normal, and the patient stayed afebrile. Antibiotics were not initiated because infective endocarditis (IE) was not suspected. The observed masses were felt to be vegetations from nonbacterial thrombotic endocarditis (NBTE), and low-molecular-weight heparin was initiated.

Also known as marantic or Libman-Sacks endocarditis, NBTE is a rare condition characterized by the deposition of thrombi on previously undamaged heart valves in the absence of an infection and an increased frequency of arterial embolic events. It normally occurs in the setting of advanced malignancy, such as adenocarcinomas, in hypercoagulable states, or in rheumatological conditions, such as lupus.1 The most commonly affected valves are the aortic and mitral, though involvement of right-sided heart valves has infrequently been reported.1 Vegetations typically present on the atrial surface of the atrioventricular valves and the ventricular surface of the semilunar valves. The sterile vegetations consist of degenerating platelets interwoven with strands of fibrin and vary in size from microscopic to large, with a tendency to detach and cause infarction more readily than vegetations seen in IE.2 Tumor necrosis factor and interleukins are released secondary to an interaction between macrophages and malignant cells; this causes endothelial damage and sloughing, creating a thrombogenic valvular surface.3 Platelets and clotting factors may also be activated by this interaction, further facilitating thrombosis.3

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Article Information

Corresponding Author: Dinesh K. Kalra, MD, Division of Cardiology, Department of Medicine, Rush University Medical Center, 1717 W Congress Pkwy, Kellogg Ste 320, Chicago, IL 60612 (dinesh_kalra@rush.edu).

Published Online: November 15, 2017. doi:10.1001/jamacardio.2017.3262

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
el-Shami  K, Griffiths  E, Streiff  M.  Nonbacterial thrombotic endocarditis in cancer patients: pathogenesis, diagnosis, and treatment.  Oncologist. 2007;12(5):518-523.PubMedGoogle ScholarCrossref
2.
Aryana  A, Esterbrooks  DJ, Morris  PC.  Nonbacterial thrombotic endocarditis with recurrent embolic events as manifestation of ovarian neoplasm.  J Gen Intern Med. 2006;21(12):C12-C15.PubMedGoogle ScholarCrossref
3.
Bick  RL.  Cancer-associated thrombosis.  N Engl J Med. 2003;349(2):109-111.PubMedGoogle ScholarCrossref
4.
Salem  DN, Stein  PD, Al-Ahmad  A,  et al.  Antithrombotic therapy in valvular heart disease—native and prosthetic: the Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy.  Chest. 2004;126(3, suppl):457S-482S.PubMedGoogle ScholarCrossref
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