LAUSR

Persistent left superior vena cava (PLSVC) is the most common venous anomaly of the thorax. Several variations have been described [1,2]. Usually PLSVC drains normally into the right atrium via the coronary sinus. We report a rare case of PLSVC connecting to the left atrium that induced extra-cardiac left-to-right shunt and symptomatic pulmonary arterial hypertension. A 66-year-old woman was referred for cardiac computed tomography (CT) to investigate an abnormal vessel connecting to the left atrium, identified during catheter ablation of an atrial fibrillation. At the age of 26, the patient underwent surgery for aortic coarctation. She was treated for arterial hypertension. She presented with grade III dyspnea according to the New York Heart Association and exerciseinduced tachycardia. CT showed PLSVC connecting to the left atrium between the left appendage and the left superior pulmonary vein. Innominate vein was present. Coronary arteries arose normally from the aorta. There was no septal defect and the coronary sinus was normal. Both atria were dilated with a surface of 35 cm2, as well as the right ventricle (end-diastolic diameter of 50 mm). The pulmonary artery was enlarged (43 mm) and bilateral mosaic perfusion was found in the lungs (Fig. 1). A jet of contrast medium flowing from the innominate vein to the right superior vena cava suggested a left-to-right shunt (Fig. 2). Contrast echocardiography revealed pulmonary arterial hypertension. This was confirmed by right heart catheterization that disclosed a mean and a maximum pulmonary arterial pressure of 42 and 70 mm Hg, respectively. Right heart blood flow was increased (cardiac index: 6.1 L/min/m2). No other cause of pulmonary arterial hypertension was found, so that surgery was decided. Peroperative findings confirmed the anatomic configuration and the extracardiac left-toright shunt (Fig. 3). Arterial blood was flowing through the PLSVC cephalad to the left cerebrobrachial vein. Tourniquet occlusion of the PLSVC at its upper extremity resulted in correction of the blood flow. When the lower end of the PLSVC was occluded, no bulging of the coronary sinus appeared i a l