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A 27-year-old woman presented with progressive dyspnoea, orthopnoea, left-sided pleuritic chest pain (improved on upright posture), 20 mm of pulsus paradoxus, distant heart sounds, and impending pericardial tamponade (confirmed on two-dimensional echocardiogram). One litre of bloody fluid was removed by pericardiocentesis.
A two-dimensional Doppler echocardiogram and transoesophageal echocardiogram demonstrated a 5 cm right atrial mass laminating the lateral and posterior walls of the atrium. A coronary angiogram showed normal coronaries, ejection fraction of 60, and tumour blush with arterial supply from a right atrial branch and from the mid to distal right coronary artery in a retrograde fashion (see panel and supplementary video). The patient underwent mediastinotomy with biopsy of the lesion that disclosed an angiosarcoma.
Only one in four of all primary cardiac tumours is malignant, and angiosarcomas constitute about a third of these. Fewer than 200 published cases have been reported. Diagnosis in the past was made usually on necropsy. Use of transthoracic echocardiography, transoesophageal echocardiography, CT and magnetic resonance imaging has facilitated increasing diagnosis before death. Most patients present with right-sided heart failure or cardiac tamponade. Pericardiocentesis yields bloody fluid that often does not contain malignant cells even when the tumour has invaded the pericardium, and biopsy may be necessary for definitive diagnosis. Metastasis is usually present at the time of presentation. Extensive surgical resection with adjunctive multimodal treatment, including combination chemotherapy with dacarbazine, doxorubicin, ifosfamide, and cyclophosphamide and radiation, may lead to long-term survival.
This article has been adapted from Malani A K, Hindupur M, Gupta C. Vascular tumour blush of cardiac angiosarcoma on coronary angiography Heart 2007;93:1237