Trend and Outcomes of Budd Chiari Syndrome Hospitalizations in the United States: 841

Introduction: Budd Chiari Syndrome (BCS) is a rare disorder with an incidence of 0.2 to 2.5 per million/year in Europe and Asia respectively. Currently, there are no studies quantifying hospitalization of patients with Budd Chiari syndrome in the United State (US). The aim of this study is to characterize the trend of BCS hospitalizations and in-hospital mortality in the US and also determine etiology, treatment, length of stay and hospital costs. Methods: All adult patients (≥18 years) with a principal diagnosis of BCS (ICD-9- CM code 453.0) and those with a principal diagnosis consistent with a clinical manifestations of BCS and secondary diagnosis of BCS were identified in the National Readmission Database(NRD). Patients with liver cirrhosis and pregnancy were excluded. The primary outcomes, trends of admission and in-hospital mortality were analyzed using the Cochran-Armitage test. Patients' demographics, clinical presentation, etiology, treatment, length of stay (LOS) and hospital cost were also analyzed. Results: There were a total of 1,250 hospitalized cases of BCS from January 2010 to December 2014 with a mean age of 47.8 years and a predominance of female (59.7%). The commonest clinical presentation of BCS was ascites (47.6%) followed by liver failure (19.7%) then hepatic encephalopathy (HE) (16.5%). The leading etiology for BCS was lymphoproliferative and myeloproliferative disorders. Only 32.8 % of BCS patients had listed treatment. The overall inpatient mortality was 8.8%. The mean LOS was 9.5 days with mean cost of $30,848 per admission.(Table 1) There were no significant trends in BCS admissions (p=0.275) or in-hospital mortality rate (p=0.615) over the years studied(Fig 1). As of 2014, there were 10.1 admissions for BCS/1 million hospital admissions with an in-hospital mortality of 6.6% (Table 2)841_A Figure 1. Demographics, comorbidities, treatment, etiology, hospital characteristics, and outcomes for patients with Budd Chiari Syndrome in the United States.Conclusion: A steady trend of BCS is observed during the study period with a mortality of 6.6% as of 2014. Ascites followed by liver failure and HE were the most common presentation. Only one third of BCS patients had therapeutic interventions. As a result of its rarity, there is a paucity of research on this disease in the US. A national registry would enable better identification and treatment of this rare disease.841_B Figure 2. Trend of Budd Chiari Syndrome admissions and mortality from 2010 to 2014.841_C Figure 3. Trend of Budd Chiari Syndrome admissions/1 million admissions and mortality rate for each year. There was no trend in BCS admissions (p=0.275) or in-hospital mortality rate (p=0.615) using Cochrane-Armitage test.