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A 67 year old woman presented with syncope. Physical examination revealed pulsus paradoxus 20 mm Hg and jugular venous distention, leading to a diagnosis of pericardial effusion and possible tamponade. Echocardiogram showed massive circumferential pericardial effusion, diastolic right ventricular collapse, and dilated inferior vena cava without respiratory changes. Pericardiocentesis yielded 1100 ml of serosanguinous fluid. All cytological and bacteriological tests on the fluid were negative. Repeat echocardiogram showed significant pericardial effusion posteriorly. Chest computed tomography (CT) scan showed large bilateral pleural effusion and a 3 cm mass at the junction of the right atrium (RA) and the right ventricle (RV), probably blood or tumour (top right).
Transoesophageal echocardiography showed a mass in the pericardial sac between the right atrium and the right ventricle extending off the atrioventricular groove and into the right atrium (bottom right).
Surgical exploration revealed an extremely vascular pericardium with dense adhesion to the epicardium. A mass was palpated in the right atrium and the wall of the right ventricle. Wedge biopsy showed this was angiosarcoma. The patient received chemotherapy with doxorubicin and is currently in remission.
Most cardiac tumours are secondary; 75% of the primary tumours are benign, and 75% of the malignant tumours are sarcomas. Widespread metastases, especially pulmonary, are common by the time the diagnosis is made. Since the signs and symptoms are non-specific, the diagnosis is often delayed which leads to poor outcomes. Diagnostic imaging such as CT scan, transoesophageal echocardiography, and magnetic resonance imaging may allow early diagnosis and treatment. Several combinations of treatment strategies were tried, but the optimum therapy is still unknown.