We report a 7-year-old boy with unilateral choanal atresia who was referred for cardiac consultation before surgery. On echocardiography, the heart was normal except the presence of a double-circle appearance and a V-shaped structure on the suprasternal notch view. Cardiac computed tomography (CT) angiography confirmed the diagnosis of the double left innominate vein (DLIV). DLIV is a rare systemic venous anomaly, which often produces diagnostic confusion on echocardiography. Correct diagnosis is essential before performing any invasive procedure through this vein. Co-existence of the two echocardiographic signs of the double-circle appearance and the V sign confirms this tricky diagnosis.
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