Department of Gastroenterology and Hepatology, Erasmus MC Transplant Institute, Erasmus MC University Medical Center, Rotterdam, the Netherlands.
Department of Biostatistics, Erasmus University Medical Center, Rotterdam, The Netherlands.
Hepatology. 2024 Jul 1;80(1):136-151. doi: 10.1097/HEP.0000000000000778. Epub 2024 Feb 15.
Management of Budd-Chiari syndrome (BCS) has improved over the last decades. The main aim was to evaluate the contemporary post-liver transplant (post-LT) outcomes in Europe.
Data from all patients who underwent transplantation from 1976 to 2020 was obtained from the European Liver Transplant Registry (ELTR). Patients < 16 years, with secondary BCS or HCC were excluded. Patient survival (PS) and graft survival (GS) before and after 2000 were compared. Multivariate Cox regression analysis identified predictors of PS and GS after 2000. Supplemental data was requested from all ELTR-affiliated centers and received from 44. In all, 808 patients underwent transplantation between 2000 and 2020. One-, 5- and 10-year PS was 84%, 77%, and 68%, and GS was 79%, 70%, and 62%, respectively. Both significantly improved compared to outcomes before 2000 ( p < 0.001). Median follow-up was 50 months and retransplantation rate was 12%. Recipient age (aHR:1.04,95%CI:1.02-1.06) and MELD score (aHR:1.04,95%CI:1.01-1.06), especially above 30, were associated with worse PS, while male sex had better outcomes (aHR:0.63,95%CI:0.41-0.96). Donor age was associated with worse PS (aHR:1.01,95%CI:1.00-1.03) and GS (aHR:1.02,95%CI:1.01-1.03). In 353 patients (44%) with supplemental data, 33% had myeloproliferative neoplasm, 20% underwent TIPS pre-LT, and 85% used anticoagulation post-LT. Post-LT anticoagulation was associated with improved PS (aHR:0.29,95%CI:0.16-0.54) and GS (aHR:0.48,95%CI:0.29-0.81). Hepatic artery thrombosis and portal vein thrombosis (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%).
LT for BCS results in excellent patient- and graft-survival. Older recipient or donor age and higher MELD are associated with poorer outcomes, while long-term anticoagulation improves both patient and graft outcomes.
在过去几十年中，布加氏综合征（BCS）的治疗得到了改善。主要目的是评估欧洲当前肝移植（post-LT）后的结果。
从欧洲肝移植登记处（ELTR）获得了 1976 年至 2020 年期间所有接受移植的患者的数据。排除了年龄<16 岁、继发性 BCS 或 HCC 的患者。比较了 2000 年前后患者生存率（PS）和移植物生存率（GS）。多变量 Cox 回归分析确定了 2000 年后 PS 和 GS 的预测因素。向所有 ELTR 附属中心请求补充数据，并从 44 个中心收到。总共，808 名患者在 2000 年至 2020 年间接受了移植。PS 为 1 年、5 年和 10 年分别为 84%、77%和 68%，GS 为 79%、70%和 62%。与 2000 年之前的结果相比，这两个结果均显著改善（p<0.001）。中位随访时间为 50 个月，再移植率为 12%。受体年龄（aHR：1.04，95%CI：1.02-1.06）和 MELD 评分（aHR：1.04，95%CI：1.01-1.06），特别是>30，与 PS 较差相关，而男性则有更好的结果（aHR：0.63，95%CI：0.41-0.96）。供体年龄与 PS（aHR：1.01，95%CI：1.00-1.03）和 GS（aHR：1.02，95%CI：1.01-1.03）较差相关。在 353 名（44%）有补充数据的患者中，33%患有骨髓增生性肿瘤，20%在 LT 前接受 TIPS，85%在 LT 后使用抗凝剂。LT 后抗凝与 PS（aHR：0.29，95%CI：0.16-0.54）和 GS（aHR：0.48，95%CI：0.29-0.81）的改善相关。肝动脉血栓形成和门静脉血栓形成（PVT）的发生率分别为 9%和 7%，而复发性 BCS 较为罕见（3%）。
BCS 的 LT 治疗可获得优异的患者和移植物生存率。受体或供体年龄较大和 MELD 评分较高与预后较差相关，而长期抗凝可改善患者和移植物的预后。
