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Giant coronary sinus and absent right superior vena cava

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This 66 year old woman underwent investigation for atypical chest pain. Chest radiography showed cardiomegaly, and echocardiography showed a giant coronary sinus 4 cm in diameter and larger than the aortic root and the left atrium, as shown on the parasternal long axis view (top). Intravenous injection of agitated saline from either the left or right brachial vein showed contrast enhancement of the giant coronary sinus. Venous angiography with contrast material injected simultaneously from both the right and left brachial veins showed that the right superior vena cava was absent and the contrast material entered the right atrium via a persistent left superior vena cava and the giant coronary sinus (bottom).  A persistent left superior vena cava is not an uncommon anomaly and usually drains into the right atrium through the coronary sinus which is dilated, measuring about 1 to 2 cm in diameter. In the rare setting of an absent right superior vena cava, this anomaly produces a truly giant coronary sinus because of the severely increased flow into the coronary sinus. Although this malformation can be associated with other cardiac anomalies, it may be an isolated finding as in this case and not associated with significant haemodynamic consequences. Recognition of this anomaly is important to avoid problems in venous catheterisation, pacemaker insertion, and cardiopulmonary bypass. (Ao, aorta; CS, coronary sinus; LA, left atrium; LV, left ventricle; RA, right atrium.)