Portal venous gas: a clinical finding in obstructed infracardiac total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection is a cyanotic congenital heart defect that presents with a multitude of clinical presentations. We report a case of obstructive total anomalous pulmonary venous connection (TAPVC) which presented in a way that has not previously been described. An infant with mixed type obstructive TAPVC was found to have pulmonary interstitial emphysema and portal venous gas on a scout radiograph obtained prior to computed tomography scan. The presence of portal venous gas indicated a connection between the pulmonary venous system and the portal venous system infradiaphragmatically.