Budd-Chiari syndrome revisited: 38 years' experience with surgical portal decompression

Ten years ago, we reported our results with what remains as the largest clinical experience with surgical portal decompression for Budd-Chiari syndrome (BCS) in the West. Since then, our series has expanded to 77 patients, and there has been an explosion of interest in and publications about BCS. The objectives of this study are to assess the validity of our observations and conclusions regarding BCS reported 10 years ago by expansion of our series of patients and observations of outcomes over an additional decade of close follow-up.

Seventy-seven patients with BCS were allocated to three groups: group I, 39 had hepatic vein occlusion alone, treated by side-to-side portacaval shunt (SSPCS); group II, 26 had inferior vena cava occlusion treated by mesoatrial shunt in eight and combined SSPCS and cavoatrial shunt (CAS) in 18; and group III, 12 had decompensated cirrhosis too late for portal decompression who were listed for liver transplantation (LT). An extensive diagnostic workup included angiography with pressure measurements and needle liver biopsy. Follow-up was 100%, lasting 5-38 years.

In group I, long-term survival is 95% with 36 free of ascites, leading lives of good quality 5-38 years post-SSPCS. In group II, mesoatrial shunt was discontinued after 1990 because of a high failure rate, but combined SSPCS-CAS has resulted in 100% survival for 5-25 years. In group III, six patients (50%) are alive and well for more than 5 years post-LT. Serial liver biopsies following portal decompression have shown long-term reversal of the lesions of BCS.

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.