Left superior vena cave connection to unroofed coronary sinus associated with positional cyanosis: Successful transcatheter treatment using Gianturco-Grifka vascular occlusion device

A persistent left superior vena cava connection to an unroofed coronary sin us is a rare cardiac anomaly that is associated with a variable degree of c yanosis, We report an infant with this condition and the unusual feature of cyanosis dependent on head position. When the patient's head was rotated t o the left, he developed severe stenosis of the left internal jugular vein, enlarged cervical collateral veins that connected to the right superior ve na cava and had an oxygen saturation 95%, When the patient's head was rotat ed to the right, the left internal jugular vein was widely patent and syste mic oxygen saturation decreased to 87%, There was no right ventricular volu me overload. Temporary occlusion of the left superior vena cava documented tolerable proximal venous pressure. Cyanosis was relieved by transcatheter closure of the left superior vena cava with a Gianturco-Grifka vascular occ lusion device. Cathet Cardiovasc. Intervent 48:369-373, 1999, (C) 1999 Wile y-Liss, Inc.