Résumé
Diffuse bilateral pulmonary arteriovenous fistula (PAVF) developed in a 19-month-old girl with polysplenia, single atrium, single right ventricle, left superior vena cava and interrupted inferior vena cava (IVC) after total cavopulmonary shunt (TCPS) operation. In addition, left pulmonary vein obstruction (PVO) by compression between the dilated hemiazygos vein and the atrium was identified. We performed total right heart bypass operation with anterior translocation of the hemiazygos vein for release of PVO and direct anastomosis of the hemiazygos vein to the hepatic vein to divert hepatic venous flow to the bilateral pulmonary circulation. Follow-up cardiac catheterization 9 months later showed disappearance of PAVF and no stenotic region. The merits of this operation are: 1) to provide balanced distribution of hepatic venous flow through the hemiazygos vein to the pulmonary circulation, 2) to release the PVO caused by the dilated hemiazygos vein, and 3) to allow for potential growth. This operation can be performed to Fontan candidates with interrupted IVC.
Résumé
A 70-year-old man was admitted to our hospital because of cough and dyspnea. On the 7th hospital day, he suddenly suffered by severe pulmonary congestion and bilateral pleural effusion with a prominent heart murmur. After improvement of the symptoms, 3-D CT scan and cardiac catheterization confirmed patent ductal aneurysm of about 10.5cm in diameter. Because of pulmonary hemorrhage, an emergency operation was performed using a left thoracotomy approach. A large aneurysmal mass of about 12cm in diameter was transected and the pulmonary end and aortic end of the ductus arteriosus were closed using a patch under partial cardiopulmonary bypass. His postoperative course was uneventful and he was discharged on the 27th day after operation.