Levoatrial cardinal vein is a rare congenital anomaly of the systemic veins. It is commonly associated with left-sided obstructive conditions such as aortic atresia, mitral atresia, and cor triatriatum. We report the case of a 14-year-old boy who was undergoing surgery for correction of a secundum atrial septal defect. Intraoperatively, we discovered that he had a levoatrial cardinal vein and no brachiocephalic vein. However, collateral vessels provided adequate flow to the right atrium, and the patient's left-sided venous pressure was not excessive, so we ligated the levoatrial cardinal vein and directly repaired the septal defect. Postoperatively, the left venous drainage was satisfactory and the patient was asymptomatic. In addition to our patient's case, we discuss the embryology, diagnosis, and treatment of levoatrial cardinal vein.
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