A patient with Marfan syndrome undergoing Bentall operation was found to have an absent right superior vena cava and persistent left superior vena cava. The dilation of coronary sinus raised the suspicion of persistent left superior vena cava. The diagnosis was confirmed by agitated saline contrast echocardiography and computed tomography of the chest.
Keywords: Absent right superior vena cava; computed tomography; contrast echocardiography; persistent left superior vena cava.