Over 12 years, 22 patients with the Budd-Chiari syndrome were treated surgically. Eighteen underwent a mesenterico-caval shunt (MCS); two, a side-to-side portacaval shunt; one, a mesenterico-atrial shunt (MAS); and one, a liver transplantation (OLT). One patient died after operation from the precipitating condition, and two MCS grafts that thrombosed were restored. All 21 surviving patients remain well, free from ascites, and all shunts are patent after a mean follow-up of 5.6 +/- 1 years, five patients with more than 10 years' follow-up. This long-term survival achieved by portasystemic shunts suggests that they have a major role in the treatment of the Budd-Chiari syndrome. The authors prefer the mesenterico-caval shunt using a jugular graft. This ensures a total portasystemic shunt, avoids subhepatic surgery, and reduces the long-term risk of prosthetic graft thrombosis. The MAS was reserved for cases with complete caval thrombosis. Patients with significant degrees of caval compression were satisfactorily decompressed by MCS. In patients not promptly treated, the disease progresses to cirrhosis, and such patients must be evaluated for transplantation similarly to those with other hepatopathies.