Long-term results of treatment of Budd-Chiari syndrome with portal decompression.

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.