Retrospective Cohort Study
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Jun 27, 2021; 13(6): 686-698
Published online Jun 27, 2021. doi: 10.4254/wjh.v13.i6.686
Mortality and health care burden of Budd Chiari syndrome in the United States: A nationwide analysis (1998-2017)
Joseph J Alukal, Talan Zhang, Paul Joseph Thuluvath
Joseph J Alukal, Talan Zhang, Paul Joseph Thuluvath, Institute of Digestive Health and Liver Diseases, Mercy Medical Center, Baltimore, MD 21202, United States
Paul Joseph Thuluvath, Department of Medicine, University of Maryland School of Medicine, Baltimore, MD 21202, United States
Author contributions: Alukal JJ and Thuluvath PJ contributed to the conception and design, the acquisition, analysis, interpretation of the data, the drafting of the article or critical revision for important intellectual content; Zhang T did the statistical analysis, and all authors approved the final version, and agree to be accountable for all aspects of the work.
Institutional review board statement: Since the data used for this this study were de-identified, IRB approval was not required as per local hospital IRB requirements.
Informed consent statement: Being a de-identified database study, consent form is not applicable.
Conflict-of-interest statement: No conflict of interest.
Data sharing statement: NIS datasets are available to everyone at a nominal fee.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Paul Joseph Thuluvath, FAASLD, AGAF, FACG, FRCP, MBBS, MD, Director, Professor, Institute of Digestive Health and Liver Diseases, Mercy Medical Center, 301 Saint Paul Place, Baltimore, MD 21202, United States.
Received: February 18, 2021
Peer-review started: February 18, 2021
First decision: March 16, 2021
Revised: March 27, 2021
Accepted: May 20, 2021
Article in press: May 20, 2021
Published online: June 27, 2021

The Budd Chiari syndrome (BCS) is a rare and potentially fatal disease, but there is a paucity of data on the in- hospital mortality as well its economic burden on the health care system.


To evaluate trends in mortality, length of hospital stays and resource utilization among inpatients with BCS.


Data on all adult patients with a diagnosis of BCS were extracted from the National Inpatient Sample (NIS) from 1998 to 2017. To make inferences regarding the national estimates for the total number of BCS discharges across the study period, sample weights were applied to each admission per recommendations from the NIS.


During the study period, there were 3591 (8.73%) in-patient deaths. The overall in-hospital mortality rates among BCS patients decreased from 18% in 1998 to 8% in 2017; the mortality decreased by 4.41% (P < 0.0001) every year. On multivariate analysis, older age, higher comorbidity score, acute liver failure, acute kidney injury, acute respiratory failure, hepatic encephalopathy, hepatorenal syndrome, inferior vena cava thrombosis, intestinal infarct, sepsis/septic shock and cancer were associated increased risk of mortality. The average of length of stay was 8.8 d and it consistently decreased by 2.04% (95%CI: -2.67%, -1.41%, P < 0.001) from 12.7 d in 1998 to 7.6 d in 2017.The average total charges after adjusted for Medical Care Consumers Price Index to 2017 dollars during the time period was $94440 and the annual percentage change increased by 1.15% (95%CI: 0.35%, 1.96%, P = 0.005) from $95515 in 1998 to $103850 in 2017.


The in-hospital mortality rate for patients admitted with BCS in the United States has reduced between 1998 and 2017 and this may a reflection of better management of these patients.

Keywords: National Inpatient Sample, Budd Chiari syndrome, Mortality, Length of stay, Total charges

Core Tip: Using a large administrative database, we were able to analyze the mortality and socioeconomic impact of Budd Chiari syndrome hospitalizations in the United States over a 19-year period with a high degree of granularity. We were able to show that while the mortality rate and length of stay has declined significantly, total charges continue to show an upward trend.