Liver and Bile
Clin Gastroenterol Hepatol. 2023;21(4):978–87.e2
Outcome of Budd-Chiari syndrome patients treated with direct oral anticoagulants: An Austrian multicenter study
Background and aims: Direct oral anticoagulants (DOACs) may simplify management of Budd-Chiari syndrome (BCS). Here, the authors report their experience with off-label use of DOACs for anticoagulation in BCS.
Methods: The safety of DOAC versus vitamin K antagonist treatment as well as associated clinical outcomes were retrospectively assessed in 47 BCS patients treated at 6 Austrian centers.
Results: Mean age at study inclusion was 37.9 ± 14.0 years and mean Model for End-stage Liver Disease (MELD) was 13.1 ± 5.1. Overall, 63.8% (n = 30) of patients had decompensated liver disease, and 87.2% (n = 41) showed clinical signs of portal hypertension. During a median follow-up of 82.5 (interquartile range [IQR], 43.1–121.8) months, 43 (91.5%) patients received anticoagulation alone or following interventional treatment, including 22 (46.8%) patients treated with DOACs (edoxaban: 10; apixaban: 4; rivaroxaban: 3; dabigatran: 3; > 1 DOAC sequentially: 2) for a median of 24.4 (IQR, 5.7–35.1) months. While 72.7% (n = 16/22) of patients were switched from low-molecular-weight heparin (n = 12) or vitamin K antagonist (n = 4) to DOAC after disease stabilization or improvement, 27.3% (n = 6/22) of BCS patients were initially treated with DOAC. Complete response (European Association for the Study of the Liver [EASL] criteria) was achieved or maintained in 14 (63.6%) of 22 patients, with ongoing response in 2 patients, while disease progressed in 6 patients (including 2 patients with hepatocellular carcinoma [HCC]). Four major spontaneous bleedings (18.2%; incidence rate = 8.8 per 100 patient-years; n = 2 upper gastrointestinal bleeding, n = 1 lower gastrointestinal bleeding, n = 1 HCC rupture), 7 minor bleedings, and 1 major procedure-related bleeding (4.5%; 2.2 per 100 patient-years) occurred during DOAC therapy. Overall transplant-free survival was 91.6% at 5 years.
Conclusions: Direct oral anticoagulants (DOACs) seem to be effective and safe for long-term anticoagulation in patients with Budd-Chiari syndrome, but confirmation by larger prospective studies is needed.