Abstract
A combination of completely unroofed coronary sinus and persistent left superior vena cava (PLSVC) is termed LSVC draining into left atrium (LA). LSVC draining into LA in patients with situs solitus is rare. We recently performed total correction in a 5-year-old girl with situs solitus who was diagnosed with a partial atrioventricular septal defect, PLSVC, and an abnormal septum in LA, like cor triatriatum, shortly after birth. Because she presented with mild cyanosis without heart failure, she was followed up without medication on an outpatient basis. However, left atrioventricular valve regurgitation (LAVVR) increased as detected by echocardiography, and she was admitted for thorough examination. Multidetector computed tomography (MDCT) and cardiac catheterization revealed grade Ⅱ LAVVR, LSVC draining into LA, and abnormal septum in LA. Intracardiac repair, followed by extracardiac rerouting from LSVC to the right atrium using autologous tissue was performed in addition to resection of the abnormal septum.
