A 14 year old boy presented with a two year history of central cyanosis. His oxygen saturation in air was 77%, but precordial echocardiography failed to detect any abnormality. Pulmonary angiography revealed a large direct communication (arrows) between the proximal right pulmonary artery (RPA) and the superior aspect of the left atrium (LA). The left pulmonary artery and the pulmonary venous return were normal, and there was no evidence of any other right to left shunting. Surgical repair of the defect is awaited.

Congenital right pulmonary to left atrial fistula usually involves the proximal right pulmonary artery or its lower lobe division. Although rare, it is an important cause of central cyanosis in the absence of other clinical findings.

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