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Published on: August 11, 2015
Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome
Aurélie Plessier1, Annie Sibert, Yann Consigny
1Service d'Hépatologie, Hôpital Beaujon, Université Paris VII, France. aurelie.plessier@bjn.ap-hop-paris.fr
Insights
Budd-Chiari syndrome (BCS) patients achieve excellent survival with a stepwise therapeutic approach. This strategy prioritizes less invasive treatments before escalating, significantly improving outcomes.
Area of Science:
- Hepatology
- Vascular Surgery
- Interventional Radiology
Background:
- Budd-Chiari syndrome (BCS) historically has a high spontaneous mortality rate, approaching 70% at 1 year.
- There is a lack of prospective data evaluating the indications and survival impact of current therapeutic interventions for BCS.
- A standardized, stepwise therapeutic strategy has been uniformly applied in a single referral center over the past 8 years.
Purpose of the Study:
- To evaluate the efficacy and impact on survival of a systematically applied therapeutic strategy for Budd-Chiari syndrome.
- To assess the indications and outcomes of sequential interventions, including anticoagulation, hepatic vein recanalization, transjugular intrahepatic portosystemic shunt (TIPS), and liver transplantation.
Main Methods:
- A cohort of 51 consecutive BCS patients received initial anticoagulation and treatment for associated conditions.
- Symptomatic patients underwent a sequence of interventions: hepatic vein recanalization, followed by TIPS, and then liver transplantation, based on response.
- Feasibility and technical success rates of recanalization and TIPS were assessed, alongside patient survival and response to therapy.
Main Results:
- A complete response was achieved with medical therapy alone in 9 patients, after recanalization in 6, TIPS in 20, and liver transplantation in 9.
- Hepatic vein recanalization was not feasible in 27 of 41 patients and technically unsuccessful in 3; TIPS was not feasible in 9 of 34 and unsuccessful in 4.
- One- and 5-year survival rates from the initiation of anticoagulation were 96% and 89%, respectively, with 84% achieving a complete response to TIPS at 1 year.
Conclusions:
- An excellent survival rate can be achieved in Budd-Chiari syndrome patients using a strategy of increasing invasiveness.
- Therapeutic decisions should be guided by patient response to preceding treatments rather than solely by disease severity.
- This stepwise approach, starting with anticoagulation and escalating as needed, demonstrates significant efficacy in managing BCS.
Abstract:
The 1-year spontaneous mortality rate in patients with Budd-Chiari syndrome (BCS) approaches 70%. No prospective assessment of indications and impact on survival of current therapeutic procedures has been performed. We evaluated a therapeutic strategy uniformly applied during the last 8 years in a single referral center. Fifty-one consecutive patients first received anticoagulation and were treated for associated diseases. Symptomatic patients were considered for hepatic vein recanalization; then for transjugular intrahepatic portosystemic shunt (TIPS), and finally for liver transplantation. The absence of a complete response led to the next procedure. Assessment was according to the strategy, whether procedures were technically applicable and successful. At entry, median (range) Child-Pugh score and Clichy prognostic index were 8 (5-12), and 5.4 (3.1-7.7), respectively. A complete response was achieved on medical therapy alone in 9 patients; after recanalization in 6, TIPS in 20, liver transplantation in 9, and retransplantation in 1. Of the 41 patients considered for recanalization, the procedure was not feasible in 27 and technically unsuccessful in 3. Of the 34 patients considered for TIPS, the procedure was considered not feasible in 9 and technically unsuccessful in 4. At 1 year of follow-up, a complete response to TIPS was achieved in 84%. One- and 5-year survival from starting anticoagulation were 96% (95% CI, 90-100) and 89% (95% CI, 79-100), respectively. In conclusion, excellent survival can be achieved in BCS patients when therapeutic procedures are introduced by order of increasing invasiveness, based on the response to previous therapy rather than on the severity of the patient's condition.