Reconstruction of cavopulmonary pathway for the patient with persistent arteriovenous malformations due to offset flow from hepatic vein

The hypoxemia caused by arteriovenous malformations after cavopulmonary shunt in patients with heterotaxy, an interrupted inferior vena cava and single ventricle physiology have been treated by incorporation of hepatic vein flow into the pulmonary circulation. However, some patients have persistent arteriovenous malformations because of offset hepatic venous flow to one pulmonary artery. Various approaches have been used to change offset flow to achieve balanced hepatic flow to the lungs in this patient population. This case report highlights the challenges that may be associated with anastomosis of the azygos vein to the inferior vena cava at the level of the diaphragm and illustrates an alternative technique to direct hepatic venous blood into an affected lung with arteriovenous malformations. The redirection of hepatic venous flow to the affected pulmonary artery resulted in resolution of symptoms within months of surgery.