Nine patients with Budd-Chiari syndrome (BCS) were treated by a portal systemic shunt. One had thrombosis of the superior mesenteric vein (SMV) and another had complete obstruction of the retrohepatic inferior vena cava (IVC). All other patients had a marked stenosis of the retrohepatic IVC with caval pressure ranging from 12 to 24 mmHg (mean: 17 mmHg). Seven patients had an interprosition mesocaval shunt using an autologous jugular vein. The patient with a thrombosed SMV had a portoartial shunt. The patient with an obstructed IVC had a cavoatrial shunt after an erroneous portacaval shunt had failed to relieve ascites. There were no operative deaths and no major postoperative complications. One patient died 19 months after operation of acute leukemia complicating polycythemia rubra vera. All other patients were alive and well 8 months to 6 years after operation. None of them had encephalopathy. These results suggest several comments: (1) Portal systemic shunts are a good treatment for BCS and have a low operative risk. (2) the mesocaval shung is an efficient procedure, even when there is stenosis of the IVC with high caval pressure: shunts to the right atrium should be performed only in the case of complete obstruction or inaccessibility of the IVC. (3) The long-term prognosis is excellent, except in patients with potential malignancies. Therfore, portal systemic shunts should be indicated early in patients with symptomatic BCS.
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