両側肺動静脈瘻および左肺静脈狭窄を合併した多脾症候群に対する完全右心バイパス手術の１例―半奇静脈前方転位および半奇静脈‐肝静脈直接吻合―

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  • A Case of Total Right Heart Bypass Operation for Polysplenia with Diffuse Bilateral Pulmonary Arteriovenous Fistula and Left Pulmonary Vein Obstruction by Anterior Translocation of the Hemiazygos Vein and Direct Anastomosis of the Hemiazygos Vein to the Hepatic Vein
  • 半奇静脈前方転位および半奇静脈-肝静脈直接吻合

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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.

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