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Acute-on-Chronic Liver Failure in Budd-Chiari Syndrome: Profile and Predictors of Outcome
Shalimar1, Sanchit Sharma2, Shivanand R Gamanagatti3
1Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, New Delhi, 110029, India. drshalimar@yahoo.com.
Insights
Budd-Chiari syndrome (BCS) can manifest as acute-on-chronic liver failure (ACLF). Endovascular intervention significantly improves survival outcomes for BCS-ACLF patients compared to those without intervention.
Area of Science:
- Hepatology
- Interventional Radiology
- Critical Care Medicine
Background:
- Budd-Chiari syndrome (BCS) rarely presents as acute-on-chronic liver failure (ACLF).
- Limited data exists on the clinical profile and outcomes of BCS-ACLF.
- Understanding these outcomes is crucial for patient management.
Purpose of the Study:
- To describe the clinical characteristics of BCS-ACLF patients.
- To evaluate the outcomes of endovascular interventions in BCS-ACLF.
- To compare survival rates between intervened and non-intervened BCS-ACLF patients.
Main Methods:
- Retrospective study of BCS-ACLF patients (October 2007-April 2019) meeting APASL criteria.
- Comparison of 30-, 90-, and 180-day survival.
- Comparison with a historical cohort of Child-C BCS patients without ACLF undergoing intervention.
Main Results:
- 28 (5%) of 553 BCS patients had ACLF; predominantly males (60.7%), mean age 29.6 years.
- Hepatic vein (HV) involvement was most common (68%); common precipitants included stent thrombosis and viral hepatitis.
- Endovascular intervention in BCS-ACLF showed significantly improved survival (93% at 30 days) versus no intervention (46% at 30 days).
Conclusions:
- Budd-Chiari syndrome can present as acute-on-chronic liver failure.
- Endovascular intervention is associated with improved outcomes in BCS-ACLF.
- Endovascular intervention and absence of hepatic encephalopathy predict better survival.
Background And Aim:
There is a paucity of data on the clinical presentations and outcome of Budd-Chiari syndrome (BCS) patients presenting as acute-on-chronic liver failure (BCS-ACLF). We aimed to describe the profile and outcomes of endovascular interventions in patients with BCS-ACLF.
Methods:
All BCS-ACLF patients presenting between October 2007 and April 2019 satisfying the Asian Pacific Association for the Study of the Liver (APASL) definition were studied. We compared 30- , 90- and, 180-day survival among BCS-ACLF patients who underwent endovascular intervention with those who did not, and with a historical cohort of Child-C BCS patients without ACLF who underwent endovascular intervention.
Results:
Twenty-eight (5%) of 553 BCS patients presented as ACLF as per APASL definition. The majority (60.7%) were males, and mean age was 29.6 ± 11.2 years. The most common site of the block was isolated involvement of hepatic veins-HV (68%), followed by combined inferior vena cava (IVC) and HV block (25%) and isolated IVC block (7%). The acute precipitants were stent thrombosis (17.9%), acute HV thrombosis (10.7%), acute viral hepatitis (7.1%), and antituberculosis drug with hepatitis B virus reactivation (3.6%). In 60.7% patients, no acute precipitant could be identified. The 30- , 90- , and 180-day survival in BCS-ACLF post-endovascular intervention (n = 15), BCS-ACLF without endovascular intervention (n = 13), and Child-C BCS without ACLF who underwent endovascular intervention (n = 25) were (93%, 87%, and 87%), (46%, 28%, and 0%) and (96%, 92%, and 88%), respectively (log-rank test, p value < 0.001). On multivariate Cox proportional analysis, endovascular intervention and the presence of hepatic encephalopathy were independent predictors of mortality.
Conclusion:
Budd-Chiari syndrome can present as acute-on-chronic liver failure. Endovascular intervention is associated with an improved outcome.
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