A 75-year-old woman presented with a 2-week history of dyspnoea, non-pulsatile neck vein distension and oedema of the neck, upper thorax and arms. A colour-Doppler echocardiogram revealed thin, laminar, high-velocity blood flow surrounding an echo-free ovular hypo-echogenic mass (4.1 cm · 6.1 cm) within an enlarged right atrium. A thoracic computed tomography (CT) scan performed before intravenous contrast medium injection (top left) showed right pleural (arrow) and pericardial effusions (asterisk), and partial collapse of the right middle and inferior pulmonary lobes (arrowheads). A CT study with contrast medium (top right, bottom panels) showed enlargement of the right atrium (RA) with a solid, homogeneous mass (M) of about 7 cm that occupied almost the whole RA, and protruded toward the tricuspid valve (bottom left) and the ostium of the superior vena cava (SVC). Collateral vein circuits, tributary of the azygos vein, were also evident along the anterior and posterior thoracic walls (top right and bottom left, white and black arrows). The SVC showed a hypo-dense aspect during the angiographic CT phase (top right, arrowhead) suggestive of occlusion, but this was excluded by a delayed CT scan that showed patency of the vessel (bottom right, arrowhead). The patient underwent urgent cardiac surgery with removal of the tumour and pericardial reconstruction. Histological examination showed a diffuse high grade B-cell lymphoma invading the adjacent myocardium. The patient’s clinical condition improved significantly after surgery, and she is currently undergoing chemotherapy. SVC syndrome most commonly results from a tumour infiltration or extrinsic compression of the vein. We observed a rare case of SVC syndrome caused by a large intracardiac non-Hodgkin lymphoma. (Ao, aorta; LPa, left pulmonary artery; LV, left ventricle; RPa, right pulmonary artery; RV, right ventricle.)
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