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Acute-on-Chronic Liver Failure in Budd–Chiari Syndrome: Profile and Predictors of Outcome

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Abstract

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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Abbreviations

ACLF:

Acute-on-chronic liver failure

APASL:

Asian Pacific Association for the Study of the Liver

APS:

Antiphospholipid antibody syndrome

BCS:

Budd–Chiari syndrome

BCS-ACLF:

Budd–Chiari syndrome–acute-on-chronic liver failure

CLIF-OF:

Chronic liver failure–organ failure

CTP:

Child–Turcotte–Pugh

EASL-CLIF:

European Association for the Study of the Liver–Chronic Liver Failure

HCC:

Hepatocellular carcinoma

HE:

Hepatic encephalopathy

HV:

Hepatic veins

INR:

International normalized ratio

IVC:

Inferior vena cava

MELD:

Model for end-stage liver disease

OF:

Organ failure

OLT:

Orthotopic liver transplant

PNH:

Paroxysmal nocturnal hemoglobinuria

ROC:

Receiver operating characteristic

TIPS:

Transjugular intrahepatic portosystemic shunts

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Acknowledgments

Mr. Amar, Mr. Dilshad, and Ms. Manisha for their work in data-keeping and maintenance of records.

Funding

None.

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Authors and Affiliations

  1. Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, New Delhi, 110029, India

    Shalimar, Sanchit Sharma, Ashish Chauhan, Sudheer Kumar Vuyyuru, Anshuman Elhence, Gyanranjan Rout, Anoop Saraya, Deepak Gunjan, Baibaswata Nayak & Subrat Kumar Acharya

  2. Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India

    Shivanand R. Gamanagatti

  3. Department of Gastroenterology, All India Institute of Medical Sciences, Patna, Bihar, India

    Ramesh Kumar

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Contributions

S helped in study concept and design, analysis and interpretation of data, and critical revision of the manuscript for important intellectual content. SS contributed to analysis and interpretation of data and drafting of the manuscript. SRG conducted endovascular interventions, analysis, and interpretation of data. SKV, AC, AE, GR, AS, DG, and BN helped in acquisition of data and drafting of the manuscript. RK contributed to drafting of the manuscript and critical revision. SKA helped in study concept and design, analysis and interpretation of data, and critical revision of the manuscript for important intellectual content. All authors approved the final version of the manuscript. Shalimar and Subrat Kumar Acharya are guarantors of the article.

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Correspondence to Shalimar.

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Shalimar, Sharma, S., Gamanagatti, S.R. et al. Acute-on-Chronic Liver Failure in Budd–Chiari Syndrome: Profile and Predictors of Outcome. Dig Dis Sci 65, 2719–2729 (2020). https://doi.org/10.1007/s10620-019-06005-7

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