Surgical improvement of hepatic venous mixing to resolve systemic arterial hypoxemia associated with post-Fontan pulmonary arteriovenous fistulae.

Pulmonary arteriovenous fistulae are known to develop in patients who have functional single-ventricle heart disease and interruption of the inferior vena cava with direct hepatic drainage to the heart, in which a bidirectional Glenn shunt is the only source of pulmonary blood flow. The progressive systemic arterial hypoxemia that is associated with pulmonary arteriovenous fistulae can have important clinical consequences. Baffling the hepatic venous return to the pulmonary circulation can alleviate pulmonary arteriovenous fistulae.Herein, we present the case of a 13-year-old patient with modified Fontan anatomy and pulmonary arteriovenous fistulae, in whom redirection of a previously placed hepatic venous-to-right pulmonary artery conduit was required in order to increase systemic arterial oxygen saturation. Revision of the conduit improved mixing of hepatic venous effluent with blood flow from the bidirectional Glenn shunt. Three years after this revision, the patient's oxygen saturation remained stable at 90%, and his physical activity was markedly improved. We present our rationale for selected redirection of the conduit and discuss other surgical options that can improve hypoxemia that is associated with pulmonary arteriovenous fistulae.