Incidence rates and case fatality rates of portal vein thrombosis and Budd-Chiari Syndrome

Summary

Little information is available on the incidence of splanchnic vein thrombosis and on mortality rates during the acute phase of the disease. We performed a large epidemiologic study on hospital admissions for portal vein thrombosis (PVT) and the Budd-Chiari syndrome (BCS) between 2002 and 2012 in Northwestern Italy. Primary and secondary discharge diagnoses of PVT and BCS were identified using the 9th edition International Classification of Diseases codes 453.0, 572.1 and 452. Hospitalisations for recurrent events were not included. Information was collected on age and gender, vital status at discharge, duration of hospitalisation, and up to five secondary discharge diagnoses. Comorbidity was evaluated using the Charlson comorbidity index (CCI). A total of 3535 patients with PVT and 287 with BCS were hospitalized. The overall gender-specific incidence rates for PVT were 3.78 per 100,000 inhabitants in males and 1.73 per 100,000 inhabitants in females; for BCS 2.0 and 2.2 per million inhabitants, respectively. In-hospital case fatality was 7.3 % in patients with PVT and 4.9 % in patients with BCS. Age, non-abdominal solid cancer, and CCI were independently associated with in-hospital mortality in both PVT and BCS after stepwise regression analysis, male gender and haematologic cancer were associated with mortality in BCS patients only. In this large study we confirmed the low incidence of BCS and we found an incidence of PVT higher than previously reported. This incidence was stable during the period of observation. In-hospital mortality is not negligible, in particular in PVT patients.

Keywords

Portal vein thrombosis - Budd-Chiari syndrome - epidemiology - mortality

• References

• 1 Almdal TP, Sorensen TI. Incidence of parenchymal liver diseases in Denmark, 1981 to 1985: analysis of hospitalisation registry data. The Danish Association for the Study of the Liver Hepatology 1991; 13: 650-655
• 2 Rajani R, Bjornsson E, Bergquist A. et al. The epidemiology and clinical features of portal vein thrombosis: a multicentre study. Aliment Pharmacol Ther 2010; 32: 1154-1162
• 3 Rajani R, Melin T, Bjornsson E. et al. Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival-an 18-year experience. Liver Int 2009; 29: 253-259
• 4 Ögren M, Bergqvist D, Bjoörck M. et al. Portal vein thrombosis: Prevalence, patient characteristics and lifetime risk: A population study based on 23 796 consecutive autopsies. World J Gastroenterol 2006; 12: 2115-2119
• 5 Ageno W, Dentali F, Squizzato A. How I treat splanchnic vein thrombosis. Blood 2014; 124: 3685-3691
• 6 Ageno W, Squizzato A, Togna A. et al. Incidental diagnosis of deep vein thrombosis in consecutive patients undergoing a CT scan of the abdomen: a retrospective cohort study. J Thromb Haemost 2012; 10: 158-160
• 7 Thatipelli MR, McBane RD, Hodge DO, Wysokinski WE. Survival and recurrence in patients with splanchnic vein thromboses. Clin Gastroenterol Hepatol 2010; 8: 200-205
• 8 Ageno W, Riva N, Schulman S. et al. Long-term Clinical Outcomes of Splanchnic Vein Thrombosis: Results of an International Registry. JAMA Intern Med 2015; 175: 1474-1480
• 9 Darwish Murad S, Plessier A, Hernandez-Guerra M. et al. (European Network for Vascular Disorders of the Liver). Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009; 151: 167-175
• 10 Sogaard KK, Darvalics B, Horvath-Puho E, Sorensen HT. Survival after splanchnic vein thrombosis: a 20-year nationwide cohort study. Thromb Res 2016; Epub ahead of print.
• 11 Sundarayayan V, Henderson T, Perry C. et al. New ICD-10 version of the Charlson comorbidity index predicted in-hospital mortality. J Clin Epidemiol 2004; 57: 1288-1294
• 12 Condat B, Pessione F, Hillaire S. et al. Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy. Gastroenterology 2001; 120: 490-497
• 13 Dentali F, Ageno W, Pomero F. et al. Time trends and case fatality rate of pulmonary embolism during 11 years of observation in Northwestern Italy. Thromb Haemost 2016; 115: 399-405
• 14 Ageno W, Riva N, Schulman S. et al. Antithrombotic treatment of splanchnic vein thrombosis: results of an International registry. Semin Thromb Hemost 2014; 40: 99-105
• 15 Riva N, Ageno W, Schulman S. et al. Clinical history and antithrombotic treatment of incindentally detected splanchnic vein thrombosis: a multicentre international prospective registry. Lancet Haematol. 2016: e267-275