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A 47 year old woman was admitted to our hospital because of worsening shortness of breath on exertion which had initially occurred about 10 years previously. Transthoracic echocardiography showed the dilated coronary sinus protruding into the left atrium (below left, LA, left atrium; LV, left ventricle; Ao, aorta; arrow, dilated coronary sinus). The coronary sinus had a defect of 8 mm × 18 mm in size through which a shunt flow from the left atrium to the sinus was demonstrated by colour Doppler echocardiography. Contrast (hand agitated saline) injected into the left arm vein appeared in the coronary sinus before the right atrium. Estimated systolic pulmonary artery pressure from moderate tricuspid regurgitation was 42 mm Hg. By transoesophageal echocardiography with three dimensional reconstruction, we could clearly recognise the shape of the coronary sinus and the defect (below centre, viewed from the lateral side; right, viewed from the anterior side; arrow, coronary sinus with defect). From these findings, we diagnosed this patient as having an unroofed coronary sinus with persistent superior vena cava. Cardiac catheterisation confirmed the diagnosis and demonstrated a significant left-to-right shunt with a shunt ratio of 2.53. The patient was operated on and surgical findings were consistent with those revealed by echocardiography. The patient recovered uneventfully and was discharged 14 days later.