[A case of completely unroofed coronary sinus with persistent left superior vena cava]

A 34-year-old female presented with exertional dyspnea. Investigation by echocardiography and cardiac catheterization showed completely unroofed coronary sinus with persistent left superior vena cava (PLSVC) (coronary sinus atrial septal defect, absence of the coronary sinus, and PLSVC-left atrium connection) combined with tricuspid valve regurgitation. Angiocardiography made by injection into the PLSVC demonstrated that the PLSVC was connected to the hemiazygos vein before it drained into the left atrium and the left innominate vein was absent. Although jugular vein pressure rose up to 18 mmHg when the PLSVC was temporarily occluded, it remained unchanged. Therefore, simple ligation of the PLSVC was selected for therapy. Patch closure of the atrial septal defect, tricuspid valve repair, and ligation of the PLSVC was performed successfully.