A 23-year-old man presented with cyanosis since birth. Precordial echocardiography failed to detect any structural heart abnormality except for enlarged left atrium. Pulmonary angiography revealed a type I right pulmonary artery-to-left atrial fistula. Simple ligation of the fistula without cardiopulmonary bypass was performed successfully, and the cyanosis disappeared postoperatively.
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