ABC | Volume 111, Nº6, December 2018

Brief Communication Costa et al Erdheim-Chester’s Disease Arq Bras Cardiol. 2018; 111(6):852-855 Skin biopsy was indicated, of which anatomopathological analysis showed accumulation of xanthomized histiocytes in the dermis, suggestive of xanthelasma, with negative S-100, positive CD68, negative CD1a and positive BRAF V600E staining. The patient underwent initial treatment with interferon, but due to bone disease progression, he is currently undergoing treatment with vemurafenib. In the follow-up 18-FDG PET-CT, RA roof uptake (SUV max = 5.7) was maintained. Case 2 This was a 64-year-old female patient, with no prior comorbidities, who was followed by the Hematology teamwith a diagnosis of ECD, with bone, lymph node and cardiovascular involvement, demonstrated by 18-FDG PET-CT examination. She showed radiotracer hyper-uptake with a heterogeneous pattern in the RA walls (SUV max : 5.8) and right ventricle (SUV max : 5.8) and discreet pericardial thickening/effusion. The TTE performed in the Cardiology department showed atrial pseudotumor in an echogenic image in the interatrial septum, measuring 2.2 cm x 1.2 cm, suggestive of lipomatous infiltration. The coronary artery angiotomography showed a calcium score (Agatston) of 4, at the 58 th percentile of the MESA (Multi-Ethnic Study of Atherosclerosis) study, with no significant coronary luminal reduction. As an additional finding, it showed a soft tissue density expansive lesion in the RA roof related to the interatrial septum and opening into the inferior vena cava. The sinus node artery, the right coronary artery branch, had a partial trajectory through the mass, in addition to atheromatosis in the descending thoracic aorta (Figure 1). Case 3 A 38-year-old male patient, with no prior comorbidities, diagnosed with ECD since 2005, identified through lung biopsy with CD68+ histiocyte, negative S-100, started treatment with interferon and prednisone. In 2017, he developed dyspnea at small efforts with NYHA III. The TTE identified left ventricle (LV) with moderate systolic dysfunction (LVEF of 40%) with diffuse hypokinesia, dilated left chambers, and preserved valvular system. The CMR showed discrete LV dilatation, with an end-diastolic diameter of 6.7 cm and an end-systolic diameter of 5.1 cm, mild diffuse hypokinesia, mild systolic dysfunction (LVEF of 46%) and late enhancement of the junction between the ventricles. Additional investigations were performed to rule out other etiologies of ventricular dysfunction: serology for Chagas' disease was negative, angiotomography of the coronary arteries with zero calcium score and absence of luminal reduction. Treatment for ventricular dysfunction was started, and the patient showed low tolerance for hypotension and cardiopulmonary rehabilitation was indicated, with an important improvement in dyspnea. Case 4 A 63-years-old female patient, a former smoker, with hypothyroidism, arterial hypertension and dyslipidemia, had generalized xanthomatous skin lesions in 2001. In 2004, due to abdominal pain, she underwent a computed tomography (CT) scan of the upper abdomen with contrast, which demonstrated hypoattenuating tissue involving the abdominal aorta and its branches. This promoted a discrete segmental narrowing of some of the vessels characterized by narrowing of the aorta in the emergence region of the renal arteries and the left subclavian artery (Figure 2). Tissue biopsy showed the presence of a pseudotumor, confirming the diagnosis of ECD. The 18-FDG PET-CT showed signs of retroperitoneal fibrosis involving the abdominal aorta immediately above and at the emergence region of the renal arteries. Concomitantly, there was infiltrative tissue surrounding the aortic arch, descending aorta and left common iliac artery. Initially, cardiac involvement had been ruled out by CMR, which had shown normal-sized chambers and preserved systolic function. Figure 1 – Images A to D refer to case 1 and the images from E to H refer to case 2. Images A and B represent images of 18-FDG PET-CT showing lesion in the right atrium roof. The C image represent CMR image, SSFP cine 4 chambers with hypointense lesion in the right atrium roof. The D image represents a transthoracic echocardiogram image with the same topography. The images E and F represent 18-FDG PET-CT with capturing lesion in the right atrium and G and H images represent contrast computed tomography showing evidence of expansive right atrial. 853