Texas Heart Institute Journal Perioperative Management of Levoatrial Cardinal Vein 201 © 2013 by the Texas Heart ® Institute, Houston Perioperative Management of a Levoatrial Cardinal Vein in the Absence of the Brachiocephalic Vein 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 provid- ed 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 asymptom- atic. In addition to our patient’s case, we discuss the embryology, diagnosis, and treat- ment of levoatrial cardinal vein. (Tex Heart Inst J 2013;40(2):201-3) L evoatrial cardinal vein, a rare congenital anomaly of the systemic veins, is often associated with left-sided obstructive conditions such as aortic atresia, mitral atresia, and cor triatriatum. 1 In this report of a patient who had a secundum atrial septal defect (ASD), we describe our intraoperative discovery and treatment of a levoatrial cardinal vein in the absence of a brachiocephalic vein. In addition, we dis- cuss the embryology and diagnosis of this rare anomaly. Case Report In October 2011, a 14-year-old boy with a large secundum ASD was referred to our hospital. Chest radiographs showed cardiomegaly from right atrial and ventricular en- largement. Transthoracic echocardiograms showed a 32-mm secundum ASD without inferior and aortic rims. The location of the pulmonary venous opening and the size of the coronary sinus were normal. We decided to treat the ASD surgically. At operation, the patient was placed on cardiopulmonary bypass. Intracardiac ex- ploration through an oblique right atriotomy revealed that the venous blood from the upper left side was draining into the left atrium through a sizable vein connected to the left atrial appendage. This vein was confirmed to be a levoatrial cardinal vein. There was no brachiocephalic vein, and its absence had not been noticed at the time of cannulation, which had been completed in routine bicaval fashion. The pulmonary veins and coronary sinus were in normal locations, and the superi- or vena cava (SVC) was of normal size. There was no left-sided obstructive condition or anomaly of the pulmonary veins. The vein connecting to the left atrial appendage was apparently draining the left subclavian vein. We snared the levoatrial cardinal vein with a tape and temporarily obliterated its blood f low without consequent change in the patient’s systemic oxygen saturation or blood pressure. His mean venous pressure increased from 8 to 14 mmHg. A periop- erative cardiac angiogram showed an anomalous vein draining into the azygos vein, as well as 2 collateral connections between the levoatrial cardinal vein and the SVC. Accordingly, we double-ligated the levoatrial cardinal vein. Our original surgical strategy was not affected by these anomalous connections: we closed the ASD directly without using a patch. At no time was cannulation affected by the anomalous systemic venous drainage. The patient was weaned from cardiopulmonary bypass without inotropic support. His recovery was uneventful, with no cyanosis or edema of the left arm, and he Case Reports Olcay Murat Disli, MD Bektas Battaloglu, MD Nevzat Erdil, MD Cemsit Karakurt, MD Ozlem Elkiran, MD Key words: Heart atria/ abnormalities/surgery; heart defects, congenital/surgery; treatment outcome From: Departments of Cardiovascular Surgery (Drs. Battaloglu, Disli, and Erdil) and Pediatric Cardiol- ogy (Drs. Elkiran and Kara- kurt), School of Medicine, Inonu University, 44280 Malatya, Turkey Address for reprints: Olcay Murat Disli, MD, Department of Cardiovas- cular Surgery, Faculty of Medicine, Inonu University, Elazig Rd. (15 km), 44280 Malatya, Turkey E-mail: olcaydisli@hotmail.com