Presence of Ascites at Presentation is Associated With Absence of Long-Term Response Amongst Patients With Budd-Chiari Syndrome When Treated With Medical Therapy Alone: A Single Centre Real-Life Experience

J Clin Exp Hepatol. 2022 May-Jun;12(3):861-870. doi: 10.1016/j.jceh.2021.10.148. Epub 2021 Nov 3.

Abstract

Background: There is lack of data on long-term outcomes of patients with Budd-Chairi Syndrome (BCS) treated with medical therapy including anticoagulation alone.

Methods: Consecutive patients (N = 138, mean [standard deviation, SD] age 29.3 [12.9] years; 66 men) with BCS, treated with medical therapy alone including anticoagulation, with minimum follow-up of 12 months were included. Initial response was classified as complete (CR), partial (PR) or nonresponse (NR) and on follow-up as loss of response (LoR) or maintenance of response (MoR). The association of baseline, clinical and biochemical parameters with different responses was evaluated.

Results: Seventy-six patients (55.1%) had CR, 26 (18.8%) had PR and 36 (26.1%) had NR. None with PR or NR had CR later. At a median follow-up of 40 (range 12-174) months, LoR was more common in PR group than in CR group (12 [46.2%] vs 18 [23.7%], P = 0.03). LoR was associated with presence of ascites (odds ratio [OR] 1.5; 95% confidence interval [CI] 0.06-0.71), gastrointestinal bleed (OR 1.33; 95% CI 0.09-0.82) or jaundice (OR 1.01; 95% CI 0.11-0.97) at baseline and duration of follow-up (OR 0.018; 95% CI 1.006-1.030). Mortality was higher in NR (28 [77.8%]) compared with CR (15 [19.7%], P = 0.001) and PR (8 [30.8%], P = 0.001). On binary logistic regression analysis, presence of ascites at baseline was associated with LoR (OR 0.303 [0.098-0.931]).

Conclusion: Patients with initial CR have better survival than nonresponders. One-third had LoR on follow-up. The presence of ascites at baseline is associated with LoR.

Keywords: AC-BCS, Acute on chronic BCS; BCS, Budd-Chairi Syndrome; CR, Complete response; CTP, Child-Pugh score; EVL, Endoscopic variceal band ligation; GAVE, Gastric antral vascular ectasia; GI, gastrointestinal; GOV1/2, Gastroesophageal varices 1 and 2; HVOTO, Hepatic venous outflow tract obstruction; INR, International Normalised Ratio; IVC, inferior vena cava thrombosis; LMWH, low-molecular-weight heparin; LoR, Loss of response; MELD, Model for end stage liver disease; MoR, Maintenance of response; NR, Nonresponse; PHG, portal hypertensive gastropathy; PR, Partial response; SBP, Spontaneous bacterial peritonitis; Warfarin; budd-chiari syndrome; cirrhosis; portal hypertension.