Urgent Hepatic to Azygous Vein Connection for Thrombosis of Hepatic Vein to Pulmonary Artery Conduit

Abstract

We report on an emergent use of hepatic-to-azygous vein connection using a 10-mm polytetrafluoroethylene graft in a 6-year-old girl who developed acute thrombosis of an extracardiac graft conduit from the hepatic veins to the pulmonary arteries. She had left atrial isomerism, interrupted inferior vena cava with azygous continuation, and had undergone surgical creation of bilateral superior cavopulmonary connections 5 years earlier. The emergency procedure to create hepatic-to-azygous connection resulted in successful salvage of the patient who was subsequently able to maintain stable oxygen saturations and experienced rapid regression of hepatic congestion and pleural effusions. Six months following the procedure, oxygen saturation was above 90%. Repeated echocardiography and computed tomography angiography showed patency of the hepatic-to-azygous connection.