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Background and Aims Budd-Chiari syndrome (BCS) is a rare vascular liver disease in which outcomes have improved with structured, stepwise management. Long-term survival data from contemporary real-world cohorts, particularly beyond 10-15 years, is limited despite the young age at presentation. We evaluated long-term survival and interventional outcomes in a large BCS cohort managed using a stepwise therapeutic strategy.Methods We analysed patients with BCS managed at a tertiary liver centre. Baseline clinical, laboratory, and aetiological data were collected. Liver disease severity was assessed using MELD score. Management included anticoagulation, hepatic venous outflow restoration (angioplasty and/or stenting), transjugular intrahepatic portosystemic shunt (TIPSS), and liver transplantation (LT) where required. Overall survival (OS) and transplant-free survival (TFS) were analysed using Kaplan-Meier Methods with multivariable Cox regression used to identify predictors of TFS.Results 122 patients were included (M:F ratio 1:1.7); median age at diagnosis was 34 years (IQR 27.6–43.2), and median MELD score was 13.2 (IQR 10.2–16.8). Liver histology was available in 49 patients, of whom 12 had cirrhosis.Underlying aetiology was identified in 73%, most acquired thrombophilia (52.5%), particularly myeloproliferative neoplasms (41%). Inherited thrombophilia was present in 20.5%.Presenting features: ascites (79.5%); abdominal pain (48.4%); GI bleeding (predominantly variceal)12.3%. 51.6% were diagnosed 1 - 6 months after symptom onset.Anticoagulation was initiated in 92%. Hepatic venous outflow restoration (by angioplasty/stenting) was performed in 73 patients (60%). Stent dysfunction occurred in 63% and 9 of these required subsequent TIPSS (5) and shunt surgery (4). 16% patients had experienced bleeding complication during FU.63 patients (51.6%) underwent TIPSS, most commonly for refractory ascites (47.6%) or progressive liver dysfunction (33%); secondary TIPSS patency was 78%. 11 patients (9.0%) underwent LT, mainly for liver failure (63%) or refractory ascites.Median follow-up was 149 months (range 0.2–455). OS at 5, 10, and 20 years was 93.4%, 88.5%, & 75.4%, respectively. TFS at 1, 5, 10, 15, and 20 years was 95.1%, 88.5%, 81.1%, 72.1%, & 66.4%. There were 38 deaths (13 liver-related). Hepatocellular carcinoma developed in 5 patients (4.1%). Increasing age at diagnosis was the only independent predictor of worse TFS.Conclusions In this large cohort with extended follow-up, BCS was associated with excellent long-term survival when managed using a structured stepwise strategy. Hepatic venous outflow restoration avoided TIPSS in many patients, TIPSS provided durable disease control when required. LT was infrequently necessary, and HCC incidence was low. Age at diagnosis was the principal determinant of long-term outcome.Abstract P59 Figure 1