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Image in Cardiology
Heart failure and pericardial mass leading to diagnosis of a rare hematologic disease
Insuficiência cardíaca e massa pericárdica no diagnóstico de doença hematológica rara
Ignacio Barriusoa,
Autor para correspondência
barriusobarrado@gmail.com

Corresponding author.
, Eva Pueoa, María Isabel Gilb, Diego Fernández-Rodrígueza, Dolors Vilesa, Fernando Wornera
a Department of Cardiology, Hospital Universitario Arnau de Vilanova, IRBLleida, Lleida, Spain
b Department of Radiology, Hospital Universitario Arnau de Vilanova, IRBLleida, Lleida, Spain
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A&#58; Ecocardiography showing mass at the auriculoventricular sulcus&#59; B&#58; Perirenal and retroperitoneal fibrosis seen in computed tomography&#59; C&#58; Magnetic resonance imaging &#40;MRI&#41; showing cardiac mass and hypointense tissue surrounding right atrium&#59; D and E&#58; MRI&#44; inversion-recovery sequences showing intense enhancement in heart mass and aortic wall&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 64-year-old woman was referred for evaluation of constitutional symptoms&#44; dyspnea and right heart failure&#46; There was a high level of inflammatory markers in the blood test&#44; so blood culture and image tests were performed to rule out endocarditis&#46; Echocardiography did not show valve disease and revealed normal systolic function and a mass at the auriculoventricular sulcus &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>A&#41;&#46; Thoracic-abdominal computed tomography ruled out inflammatory and neoplastic diseases&#44; showing the heart mass&#44; perirenal and retroperitoneal fibrosis spreading to pararenal and paraaortic space &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>B&#41; and areas of bone sclerosis in pelvis and vertebrae&#46; Magnetic resonance imaging was performed afterwards&#46; Cardiac cine-balanced fast field echo shows the cardiac mass as an extensive hypointense tissue surrounding the right atrium &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>C&#41; with intense enhancement after intravenous administration of contrast at the inversion-recovery sequences &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>D and E&#41;&#46; Intense enhancement was also seen in all aortic wall&#44; supra-aortic trunks&#44; visceral and pulmonary arteries suggesting extensive vascular adventitial fibrosis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>E&#41;&#46; Finally&#44; a retroperitoneal biopsy showed chronic IgG4-lymphoplasmacytic inflammation with a xanthogranulomatous component&#46; In the molecular test&#44; a specific mutation in the BRAF gene was found pointing to Erdheim-Chester disease as the diagnosis&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Erdheim-Chester disease is a rare non-Langerhans histiocytic multisystem disorder &#40;fewer than 1000 cases in the literature&#41; that principally affects long bones &#40;95&#37;&#41;&#44; the retroperitoneum &#40;59&#37;&#41; and the cardiovascular system &#40;57&#37;&#41;&#44; which can include pseudotumoral cardiac infiltration&#44; valve abnormalities&#44; rhythm defects and periaortic fibrosis&#46; Historically&#44; the prognosis has been poor but new treatments targeted at specific mutations &#40;BRAF inhibitors&#41; may improve survival&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0015" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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