Plessier A et al. (2006) Aiming at minimal invasiveness as a therapeutic strategy for Budd–Chiari syndrome. Hepatology 44: 1308–1316

Occlusion of the inferior vena cava or hepatic vein causes Budd–Chiari syndrome (BCS), which can lead to liver failure. BCS has a 1-year spontaneous mortality rate reported to approach 70%, and many treatments have been proposed for the condition. Plessier et al. report on their strategy of commencing therapy with the least-invasive treatment, followed by successively more-invasive procedures in patients who do not respond. Use of this strategy in 51 consecutive patients who presented with BCS produced overall 1-year, 3-year and 5-year survival rates of 96%, 89%, and 89%, respectively.

Anticoagulation therapy was the first-line treatment; a complete response was obtained in nine patients, and one patient died of hematologic disease. Recanalization was carried out in the 14 patients who had an adequately short stenosis; this procedure resulted in a complete response in 7 patients. Of the remaining 34 patients, 25 were suitable for transjugular intrahepatic portal shunt placement; this intervention was successful in 21 patients, although 1 required transplantation 4 months later. Two patients died from procedure-related complications. The remaining 11 patients underwent liver transplantation (median time from anticoagulation initiation, 12 months), 1 of whom died from sepsis. In total, 5 of the 51 patients who commenced treatment died.

The authors conclude that a strategy of increasing the invasiveness of treatment according to patient responses produces excellent medium-term survival rates. The liver-transplant success rate suggests that performing transplantation only when other interventions have failed does not compromise patient survival.