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ObjectiveTo determine the effects of surgical portal decompression in Budd-Chiari syndrome (BCS) on survival, quality of life, shunt patency, liver function, portal hemodynamics, and hepatic morphology during periods ranging from 3.5 to 27 years. Summary Background DataExperiments in the authors' laboratory showed that surgical portal decompression reversed the deleterious effects of BCS on the liver. This study was aimed at determining whether similar benefit could be obtained in patients with BCS. MethodsFrom 1972 to 1999, the authors conducted prospective studies of the treatment of 60 patients with BCS who were divided into three groups: the first had occlusion confined to the hepatic veins treated by direct side-to-side portacaval shunt (SSPCS); the second had occlusion involving the inferior vena cava (IVC) treated by a portal decompressive procedure that bypassed the obstructed IVC; and the third group, who had advanced cirrhosis and hepatic decompensation and were referred too late for treatment by portal decompression, required orthotopic liver transplantation.

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Thirty-three patients with Budd-Chiari syndrome were studied for 1 to 19 years following portal decompression. All had ascites, hepatomegaly, abnormal liver function, angiographic demonstration of inferior vena cava and/or hepatic vein occlusion, and biopsy specimens showing intense hepatic congestion and necrosis. When thrombosis was confined to hepatic veins (20 patients), side-to-side portacaval shunt resulted in 95% operative survival, 90% prolonged survival, permanent shunt patency, relief of ascites, reversal of liver dysfunction, and reversal or improvement of hepatic lesions. When thrombosis involved the inferior vena cava, mesoatrial shunt (eight patients) was unsatisfactory because of a 63% mortality rate from liver failure due to shunt thrombosis. In contrast, a new procedure consisting of combined portacaval and caval-atrial shunts (five patients) has been highly successful, with 100% survival, shunt patency, relief of ascites, and reversal of pathologic abnormalities.

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Long-term survival following portal decompression of BCS in the West has not been equaled by any other form of therapy, medical or surgical. It is imperative to perform surgical portal decompression early in the course of BCS in order to avoid irreversible liver damage.