We report a congenital circumflex coronary artery to coronary sinus arteriovenous fistula diagnosed by transesophageal echocardiography in a 38‐year‐old asymptomatic male. The coronary arteriovenous fistula was suggested by two‐dimensional transthoracic color Doppler. A first nondiagnostic angiography was performed, and it was during the second one, that the fistula could finally be visualized after a special dye technique was used. The high fistula flow rate prevented us from seeing it with regular dye concentration. Transesophageal echocardiography was precise in the anatomical and hemodynamic evaluation and it was essential in guiding the angiographic examination and surgical repair.
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