[A Total Right Heart Bypass Operation for Polysplenia with Unilateral Pulmonary Arteriovenous Fistula;the Usefulness of the Anastomosis of the Hepatic Vein to the Azygous Vein Combined with Dilatable Pulmonary Artery Banding].

: Right pulmonary arteriovenous fistula(PAVF) developed in a 18-month-old girl after total cavopulmonary shunt(TCPS) operation. The patient was diagnosed as polysplenia, single atrium, single ventricle, dextrocardia, pulmonary atresia, congenitally complete atrioventricular block, and absent infrahepatic segment of the inferior vena cava with azygous continuation to the right superior vena cava. The hepatic veins were connected to the left-side atrium. At age of 7 month, TCPS operation was performed. Arterial oxygen saturation decreased to 70% at 8 months after TCPS operation. We performed total right heart bypass operation with connected hepatic vein to the azygous vein using a 8-mm ePTFE tube graft, combined with dilatable right pulmonary artery banding( PAB). With PAB on the right pulmonary artery adjacent to the Glenn anastomosis, arterial saturation was 80% and the patient was successfully weaned off cardiopulmonary bypass. At 3 months after TRHB operation, systemic saturation increased to 90%.The right pulmonary artery banding site was successfully dilated by balloon angioplasty.