Fontan operation with bilateral conduits for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia after reconstruction of a non-confluent pulmonary artery.

We describe a case involving a successful Fontan procedure for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia. The patient was diagnosed with bilateral superior vena cavae, interrupted inferior vena cava with azygos connection, wide separate hepatic veins, and central pulmonary artery stenosis following repair of a non-confluent pulmonary artery. Bilateral extracardiac conduits were used to connect each hepatic vein to the pulmonary artery, opposite each superior vena cava. Clinical improvement in the pulmonary arteriovenous fistulas was observed within 4 months after surgery.