Validation of Prognostic Indices in Budd Chiari Syndrome Egyptian Patients: A Single Centre Study

Abstract

The aim of this study was to identify and compare the ability of prognostic indices in primary BCS Egyptian patients to predict one year survival rate following different patterns of management in addition to their ability for prediction of patency of applied stents following radiological intervention (angioplasty with stenting and TIPS) after one year of follow up. Our secondary aim was to find out different clinical and laboratory prognostic indicators. All available Budd-Chiari patients fulfilling the inclusion criteria from the year 2005 up to the year 2015 were included in the study. The current study was conducted on 194 consecutive Egyptian patients with confirmed diagnosis of BCS who were admitted to Ain Shams University Hospitals, Tropical Medicine Department or attending the outpatient clinics. The patients were affiliated to the Budd Chiari Study Group (BCSG). Baseline and follow up clinical, laboratory and imaging studies of patients were recorded with calculation of all PIs including Child-Pugh, MELD, Rotterdam, Clichy, New clichy scores. BCS-TIPS score was calculated only in TIPS patients. None of these PIs were used for making decision on individual patient management. The mean age of the current series was 28.79± 8.94 years with females representing 57.2% of the patients. The most common aetiology was FVLM as inherited thrombophilic disorder found in 57 (29.4%) patients and APS as acquired thrombophilic disorder found in 59 (30.4%) of patients. Regarding the management of cases, 63 (32.5%) were treated medically (anticoagulation, treatment for the underlying etiology and diuretics) while the remainder underwent interventions. TIPS was done in 107 patients with revision of the stent in 45 (42.1%) of them while, HV stent was done in 20 patients and 19 (95%) didn’t need further stent revision within their follow up for 1 year. Little is known about factors that may be of relevant predictive value for the survival of BCS patients and several prognostic indices (PIs) have been proposed for Budd-Chiari syndrome (BCS). However, patient characteristics, causal factors, and treatment outcomes have changed since these indices have been elaborated. Regarding patients’ survival, we found a striking difference regarding survival at 12 months being higher in the group of patients who underwent intervention than those who were unfit and managed only medically (85.2% versus 14.8% respectively). The overall three and five year survival rates were 65.8% and 39.6% before 2010 being higher in the intervention group (80.6% and 48.4%) compared to non intervention one (46.9% and 28.6%) with statistical significant difference. However, after 2010 the overall three year survival rate was slightly lower than that before 2010 being 60.3% but the five year survival was better after 2010 being 50%. All PIs were significantly related to one year survival and can distinguish survival from death in BCS Egyptian patients. Only Child, Clichy and New Clichy scores can be regarded as valid for individual management. TIPS procedure remains an extremely effective therapy for those BCS patients who are eligible for it. Survival at one year post-TIPS in the current study among 107 cases who underwent TIPS was 86.9%. Although BCS-TIPS score has been related significantly to one year survival, its predictive ability remains lower than other PIs. Regarding prognostic indicators in the current study, neither age nor gender was found to be related to prognosis. Presence of oral and genital ulcers which is associated with Behet disease as well as use of hormonal therapy in female patients were related to poor prognosis. In addition, Patients presented with PVT and IVC thrombosis showed higher mortality rates in the current study. Multivariate analysis showed that ascites resistant to medical treatment (score 3), presence of encephalopathy, high total bilirubin level and low serum albumin level are independent risk factors for mortality at one year.