‹ Scimitar syndrome

. The Scimitar syndrome with its anomalous pulmonary venous connection from the right lung to the inferior vena cava should be considered in differential diagnosis when dealing with left-to-right shunt of unknown origin. Picture a presents a sub-costal view of the liver with dilated inferior vena cava and liver veins.

However, we can also see a vessel (F) crossing the diaphragm and entering the inferior vena cava laterally. The entrance of that vessel into the inferior vena cava is documented with colour Doppler echocardiography (b).

This large red-coded vessel entering the inferior vena cava was proven to represent the common pulmonary vein of the right lung in a documented Scimitar syndrome. In the long axis view (c) this abnormal vein, which causes relevant left-to-right shunt, can be traced from its entry into the vena cava up to the right lung.

Note the physiological dilatation of the left renal vein (asterix) prior to its traverse across the narrow site between the mesenteric artery and the abdominal aorta in this patient with Scimitar syndrome(g).