353 Left Superior Vena Cava Draining into the Left Atrium, Associated with Partial Anomalous Pulmonary Venous Connection: Surgical Correction Andrea Quarti, M.D., Marco Di Eusanio, M.D., Michele Danilo Pierri, M.D., and Giuseppe Di Eusanio, M.D. Department of Cardiac Surgery, G.M. Lancisi Hospital, Ancona, Italy ABSTRACT A persistent left superior vena cava draining into the left atrium may produce a symptomatic right-to-left shunt. Although intra-atrial rerouting techniques, in patients with no connecting vein, have proved to be reliable and successful, in many cases the extracardiac repair is preferable. We report a case of a 5-month-old patient with a not connected left superior vena cava draining into the left atrium, associated with atrial septal defect and partial anomalous pulmonary venous connection. The correction has been achieved by rerouting the pulmonary venous return into the left atrium and by transposition of the left vena cava on the right appendage. doi: 10.1111/j.1540-8191.2005.200471.x (J Card Surg 2005;20:353-355) Persistent left superior vena cava (LSVC) connect- ing to the coronary sinus associated with atrial sep- tal defect (ASD) is a common anomaly, but rarely, ap- proximately in the 8% of the cases, 1 it connects to the left atrium through the left atrial appendage caus- ing a right-to-left shunt responsible for cyanosis and heart failure.The patient’s anatomic characteristics de- termine the surgical technique of repair. If the LSVC is connected to the right superior vena cava by a left innominate vein of adequate size, the simple ligation is feasible. However, if there is no connection, intra- atrial rerouting or extracardiac techniques are com- monly used. 1-4 In this report we describe an extra car- diac technique to correct a LSVC draining into the left atrium associated to partial anomalous pulmonary ve- nous connection. CASE REPORT A 5-month-old boy with cyanosis, tachypnea, and signs of chronic heart failure was referred to our institu- tion. At a physical examination a systolic murmur with splitting of the second heart sound was noted at the left sternal border. The chest X-ray showed an enlarge- ment of the heart with images referring to pulmonary overloading. During an echocardiographic examination an ASD with persistent LSVC draining into the left atrium was noted. The cardiac catheterization showed an ASD with a persistent LSVC draining into the left atrium through the left appendage, a right bicarotideal aortic arch, and right pulmonary veins draining into the right atrium. An unroofed coronary sinus syndrome was excluded. Address for correspondence: Andrea Quarti, M.D., Department of Cardiac Surgery, Azienda Ospedaliera G.M. Lancisi Via Conca, 60128 Ancona, Italy. Fax: 071 5965301; e-mail: aquarti@libero.it Operation was performed through a median ster- notomy. The innominate vein was not found. The LSVC was isolated and mobilized (Fig.1). After cardiopul- monary bypass (CPB) was established and during deep hypothermic circulatory arrest (DHCA) the right atrium was opened and the ASD was closed with a rerouting of the right pulmonary veins into the left atrium, using an autologous pericardial patch. During the rewarming the LSVC was transected at the entrance in the left atrium, carried up over the ascending aorta, and anastomized end-to-end to the right appendage (Fig. 2). The postoperative course was uneventful and a sub- sequent echocardiographic examination showed the complete restoration of the circulation. COMMENT Persistent LSVC is a common anomaly and usually it drains in the coronary sinus. In a rare condition it drains in the left atrium causing a right-to-left shunt and heart failure. Usually the surgical approach to the LSVC drain- ing into the left atrium in patients with no connection between the LSVC and the right superior vena cava has been the rerouting of blood to the right atrium by mean of a patch or using the inverted left atrial appendage as a baffle. 1 Acceptable results have been reported with this technique but early detachment or deterioration of the baffle with venous obstruction have been de- scribed 2,5 ; furthermore, the flow inside the baffle may cause an enlargement of the LSVC to create a partial obstruction of the left ventricular inflow. 6 Simple ligation may be used when the innominate vein is present despite some authors 2 reported cases of ligation in absence of the innominate vein, but sometimes it leads to left jugular venous pressure elevation which may cause a concomitant cerebral damage. 2