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      Nonbacterial Thrombotic Endocarditis of the Tricuspid Valve in a Male Patient with Antiphospholipid Syndrome

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          Abstract

          Valve vegetations in nonbacterial thrombotic endocarditis consist of fibrin and platelet aggregates and can be related to circulating immune complexes, such as in the case of antiphospholipid syndrome. In patients with primary antiphospholipid syndrome, echocardiographic studies have disclosed heart valve abnormalities in about a third of patients. Unusual associations between antiphospholipid syndrome and nonbacterial thrombotic endocarditis include presentation as an intracardiac mass compatible with a myxoma on imaging studies, as well as isolated involvement of the tricuspid valve. Both of these scenarios have been previously reported in female patients. This article presents the case of a 53-year-old Hispanic male with antiphospholipid syndrome who presented to the hospital with symptoms of heart failure and persistent right calf pain. An intracardiac mass attached to the anterior leaflet of the tricuspid valve was found through transthoracic echocardiography. Further imaging studies suggested the mass to be a myxoma and the patient underwent mass excision with tricuspid valve replacement. Pathology report of the surgical specimen was consistent with a diagnosis of nonbacterial thrombotic endocarditis. This case highlights the importance of considering nonbacterial thrombotic endocarditis as a key differential diagnosis in patients with concomitant antiphospholipid syndrome and intracardiac masses, as well as challenges encountered in diagnosis and management.

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          Most cited references10

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          Nonbacterial thrombotic endocarditis: a review.

          The entity of NBTE is reviewed in this article. Historic aspects, epidemiology, and pathogenesis are discussed. The clinicopathologic findings are emphasized as well as the potential for antemortem diagnosis and therapy. NBTE is diagnosed infrequently before death. Clinical suspicion is aroused in patients with an underlying process such as malignancy, DIC, or a spectrum of other diseases and evidence of pulmonary and/or systemic embolization. Systemic infection must be excluded. Two-dimensional echocardiography can be utilized to confirm the diagnosis. Anticoagulation therapy with heparin may prevent embolization.
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            Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome.

            The antiphospholipid syndrome (APS) is defined by the presence of anti-phospholipid antibodies (aPLs) and venous or arterial thrombosis, recurrent pregnancy loss, or thrombocytopenia. The syndrome can be either primary or secondary to an underlying condition, most commonly systemic lupus erythematosus (SLE). Echocardiographic studies have disclosed heart valve abnormalities in about a third of patients with primary APS. SLE patients with aPLs have a higher prevalence of valvular involvement than those without these antibodies. Valvular lesions associated with aPLs occur as valve masses (nonbacterial vegetations) or thickening. These two morphological alterations can be combined and are thought to reflect the same pathological process. Both can be associated with valve dysfunction, although such association is much more common with the latter alteration. The predominant functional abnormality is regurgitation; stenosis is rare. The mitral valve is mainly affected, followed by the aortic valve. Valvular involvement usually does not cause clinical valvular disease. The presence of aPLs seems to further increase the risk for thromboembolic complications, mainly cerebrovascular, posed by valve lesions. Superadded bacterial endocarditis is rare but may be difficult to distinguish from pseudoinfective endocarditis. The current therapeutic guidelines are those for APS in general. Secondary antithrombotic prevention with long-term, high-intensity oral anticoagulation is advised. The efficacy of aspirin, either alone or in combination, is yet to be assessed. Corticosteroids are not beneficial and may even facilitate valve damage. Immunosuppressive agents should only be used for the treatment of an underlying condition. Current data suggest a role for aPLs in the pathogenesis of valvular lesions. aPLs may promote the formation of valve thrombi. These antibodies may also act by another mechanism, as indicated by the finding of subendothelial deposits of immunoglobulins, including anticardiolipin antibodies, and of colocalized complement components in deformed valves from patients with APS.
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              Cardiac masses and tumours.

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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                28 May 2018
                May 2018
                : 10
                : 5
                : e2695
                Affiliations
                [1 ] Medicine, University of Puerto Rico School of Medicine, San Juan, PRI
                [2 ] Cardiovascular Medicine Division, University of Puerto Rico School of Medicine, San Juan, PRI
                Author notes
                Nicole M. Yordan-Lopez nicole.yordan@ 123456upr.edu
                Article
                10.7759/cureus.2695
                6063382
                b475400a-143d-4022-b19e-362256ebad76
                Copyright © 2018, Yordan-Lopez et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 19 March 2018
                : 28 May 2018
                Categories
                Cardiac/Thoracic/Vascular Surgery
                Cardiology
                Internal Medicine

                nonbacterial thrombotic endocarditis,antiphospholipid syndrome,incracardiac mass,myxoma,tricuspid valve,nbte,aps

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