Abstract

A 63-year-old-man presented to our institution with dyspnea. Transthoracic echocardiography (TTE) showed severe degenerative aortic stenosis with regurgitation and decreased left ventricular contractility. Subsequent transesophageal echocardiography (TEE) and cardiac computed tomography (CT) revealed an outpouching structure at aortomitral intervalvular fibrosa (AMIVF) (Fig A to D), which was compatible with pseudoaneurysm and suggested sequelae of previous infective endocarditis. On surgery, thickened AV leaflets were noted, especially on NCC and LCC (Fig E). When extracting the AV leaflets, subannular pouching was seen without evidence of active inflammation or infection (Fig F). AV replacement with 25-mm Sorin Mitroflow was performed.One-year TTE follow-up showed markedly increased transAV pressure gradient. TEE and cardiac CT revealed symmetric nodular thickening of bioprosthetic AV leaflets with opening limitation (Fig G to J). The patient was admitted on the diagnosis of subclinical bioprosthetic valve thrombosis, and anticoagulation with warfarin and aspirin was started. During the hospitalization, the patient had persistent fever with bicytopenia and hepatosplenomegaly, but repetitive blood cultures were negative. There was no definite fever focus even in 18F-FDG PET CT (Fig K). Since the patient worked on a farm in Dangjin, Chungcheongnam-Do, South Korea, where Q fever is prevalent, an indirect immunofluorescence assay for Coxiella burnetii was conducted and the result turned out to be positive. Additional coagulopathy laboratory findings showed positive for lupus anticoagulant, anti-cardiolipin IgM, and anti-beta2 GPI IgM. Finally, the patient was diagnosed with Q fever endocarditis combined with antiphospholipid syndrome and treated with doxycycline with hydroxychloroquine and maintained anticoagulation. Recent follow-up of TTE and cardiac CT showed complete resolution of prosthetic AV thrombus.

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