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.

Highlights

  • Received 03/19/2018 Review began 03/20/2018 Review ended 05/23/2018 Published 05/28/2018Nonbacterial thrombotic endocarditis (NBTE) was first described by Zeigler in 1888 when he introduced the term thromboendocarditis to refer to the deposition of fibrin on cardiac valves

  • We found no published studies addressing gender differences in the prevalence of antiphospholipid syndrome (APS) with NBTE

  • In patients with APS with recurrent emboli, the early use of echocardiography can lead to the detection of a potential cardiogenic source before symptomatic valvular dysfunction

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Summary

Introduction

Received 03/19/2018 Review began 03/20/2018 Review ended 05/23/2018 Published 05/28/2018. We discuss the challenges in diagnosis and management of a male patient with APS, who presented with signs of heart failure and a right cardiac mass on imaging. The patient had an inferior vena cava (IVC) filter placed two years before due to the recurrence of these thrombotic events despite being on anticoagulation therapy His only medication was warfarin of unknown dose. Chest computed tomography (CT) demonstrated a lobulated hypo-attenuating intracavitary right heart mass which appeared to be centered in the tricuspid valve (Figure 2A). Together, these findings suggested a right cardiac myxoma causing TV regurgitation and congestion (hepatomegaly, ascites, dilated inferior vena cava). Pathology report of the surgical specimen was consistent with chronic endocarditis with thrombi, rejecting the hypothesis of a cardiac myxoma and suggesting a diagnosis of NBTE instead

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