Vol.:(0123456789) 1 3 Digestive Diseases and Sciences https://doi.org/10.1007/s10620-019-06005-7 ORIGINAL ARTICLE Acute‑on‑Chronic Liver Failure in Budd–Chiari Syndrome: Profle and Predictors of Outcome Shalimar 1  · Sanchit Sharma 1  · Shivanand R. Gamanagatti 2  · Ashish Chauhan 1  · Sudheer Kumar Vuyyuru 1  · Anshuman Elhence 1  · Gyanranjan Rout 1  · Anoop Saraya 1  · Deepak Gunjan 1  · Baibaswata Nayak 1  · Ramesh Kumar 3  · Subrat Kumar Acharya 1 Received: 14 October 2019 / Accepted: 10 December 2019 © Springer Science+Business Media, LLC, part of Springer Nature 2020 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 profle and outcomes of endo- vascular interventions in patients with BCS-ACLF. Methods All BCS-ACLF patients presenting between October 2007 and April 2019 satisfying the Asian Pacifc Association for the Study of the Liver (APASL) defnition 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 defnition. 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 identifed. 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. Keywords Ascites · Liver · Cirrhosis · Portal hypertension · Vascular Abbreviations ACLF Acute-on-chronic liver failure APASL Asian Pacifc 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 * Shalimar drshalimar@yahoo.com Extended author information available on the last page of the article