FULMINANT DEVELOPMENT OF PULMONARY ARTERIOVENOUS-FISTULAS IN AN INFANT AFTER TOTAL CAVOPULMONARY SHUNT

An infant with cyanotic congenital heart disease and polysplenia syndr ome developed profound cyanosis within months of undergoing bilateral cavopulmonary anastomoses. Intrapulmonary shunting was diagnosed by co ntrast echocardiography with peripheral venous and selective pulmonary artery injection. Histopathology revealed abnormal, thin-walled vesse ls within the interstitium of the lung lobule. These vessels have not been reported previously and are likely to be the anatomic site of art eriovenous shunting. This case demonstrates that pulmonary arterioveno us fistulas (PAVFs) may develop rapidly after cavopulmonary anastomosi s in young infants. It also illustrates the use of contrast echocardio graphy for following PAVF progression in these patients.