Liver Transplantation For Budd-Chiari Syndrome Research Articles

Venous outflow reconstruction in living-donor liver transplantation for Budd-Chiari syndrome involving vena cava.

Ironically, the hepatic vena cava is mostly involved in Budd-Chiari syndrome in the Asia-Pacific region, whereas living-donor liver transplantation is predominant, which cannot replace the hepatic cava. Hata and colleagues introduced a new surgical technique for venous reconstruction in living-donor liver transplantation, providing a novel solution to this longstanding dilemma.

Recent outcomes of liver transplantation for Budd-Chiari syndrome: A study of the European Liver Transplant Registry (ELTR) and affiliated centers.

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.

Clinical outcomes after living donor liver transplantation for Budd-Chiari syndrome

Clinical outcomes after living donor liver transplantation for Budd-Chiari syndrome

Liver Transplantation for Budd-Chiari Syndrome in the MELD Era.

We extracted data from the United Network for Organ Sharing database on all adult (≥18 y old) waitlisted candidates and recipients of LT with BCS in the United States between 2002 and 2019. Multivariable Cox regression was used to determine predictors of mortality and hazard ratios (HRs). A total of 647 BCS patients were waitlisted between 2002 and 2019. BCS was an indication for LT in 378 (0.2%) of all adult LT recipients during the study period. Of BCS patients who received LT, approximately three-fourths (72.3%) were alive for up to 10 y. We found no significant difference in LT outcomes in BCS patients with or without TIPS. Longer length of hospital stay following LT (HR, 1.32; 95% confidence interval [CI], 1.19-1.47), Black/African American race (HR, 2.24; 95% CI, 1.38-3.64), diabetes (HR, 3.17; 95% CI, 1.62-6.21), donor risk index (HR, 1.44; 95% CI, 1.05-1.99), and lower albumin levels at the time of transplantation (HR, 0.66; 95% CI, 0.50-0.88) were negatively associated with survival after LT. Interestingly, neither the Model for End-stage Liver Disease nor prior TIPS showed a significant association with survival after LT. These findings demonstrate good comparable survival among TIPS versus no TIPS in LT recipients with BCS. The decision for TIPS versus LT should be individualized on a case-by-case basis.

Liver transplantation for Budd-Chiari syndrome: A challenging but handable procedure

BackroundEstablishing venous outflow in liver transplantation for patients with Budd-Chiari syndrome is crucial and requires various surgical techniques. The outcomes of these patients exibits distinct problems including vasculary thrombosis and biliary complications. MethodsIn this single center study, the outcomes and surgical features of 33 patients with Budd-Chiari syndrome who were carried out liver translantation (27 patients from living donor). Another group was formed among patients underwent liver transplantation due to other etiologies and the outcomes were compared. ResultsThe most-seen type was the classical type of Budd-Chiari syndrome (25, 75.8%). For twenty-six patients inherited or acquired prothrombotic disorders were identified (78.2%) in Budd-Chiari group. Average follow-up was 29.7 ± 15.5 months. We have observed no recurrence of disease in our BCS patients. When the two groups was compared in terms of thrombotic complications, there was a significantly increased risk in BCS group (p = 0.014). Our 1 and 3-year survival rates for the BCS group were 81.8% and 78.8%, respectively. In the control group, 1 and 3-year survival rates were 93.3% and 88.9%, respectively. Log-rank test analyses showed no statistically significant results. ConclusionLiver transplantation with individual surgical and postoperative treatment strategy for patients with Budd-Chiari syndrome provides comparable outcomes.

Orthotopic Liver Transplantation for Budd-Chiari Syndrome: Observations from a 30-Year Liver Transplant Program.

Background and objectives Budd-Chiari syndrome (BCS) refers to a complete thrombotic obstruction of the venous hepatic outflow tract due to various etiologies and constitutes a rare indication for ortothopic liver transplantation (LT). Few studies investigated long-term outcomes after LT for BCS. The aim of this study was to examine potential risk factors for late mortality and to evaluate long-term outcomes after LT for BCS. Materials and methods: 46 patients received an LT for BCS between 1989 and 2019 at the transplant center of the Charité-Universitätsmedizin Berlin. We analyzed potential effects of disease etiology, vascular events, rejection, and immunosuppression on long-term survival after transplantation using Kaplan-Meier curves and Cox logistic regression. Results: Of the 46 patients, 70% were female and 30% were male. Median age at the time of transplantation was 36 years. A total of 41 vascular events, including 26 thrombotic and 17 hemorrhagic incidents, occurred. The 1 year, the 5 year, the 10 year, and the 20 year survival rates were 87%, 83%, 76%, and 60%, respectively. By comparison, survival rates of the liver transplant cohort across all other indications at our center were slightly inferior with 85%, 75%, 65%, and 46%, respectively. In the study population, patients with myeloproliferative disorders showed worse outcomes compared to patients with other causes of BCS. Conclusion: Liver transplantation for BCS showed excellent results, even superior to those for other indications. Vascular events (i.e., thrombotic or hemorrhagic complications) did not have any prognostic value for overall mortality. Patients with myeloproliferative disorders seem to have a disadvantage in survival.

Outcomes of status 1 liver transplantation for Budd-Chiari Syndrome with fulminant hepatic failure.

There is a paucity of data on the outcome of liver transplantation (LT) in Budd-Chiari Syndrome (BCS) patients who are listed as status 1. The objective of our study was to determine patient or graft survival following LT in status 1 BCS patients. We utilized United Network for Organ Sharing (UNOS) database to identify all adult patients (> 18years of age) listed as status 1 with a primary diagnosis of BCS in the United States from 1998 to 2018, and analyzed their outcomes and compared it to non-status 1 BCS patients. Four hundred and forty-six patients with BCS underwent LT between 1998 and 2018, and of these 55 (12.3%) were listed as status 1. There was no difference in long-term post-liver transplant or "intention-to-treat" survival from the time of listing to death or the last day of follow-up between status 1 and non-status 1 groups. Graft and patient survival at 5years for status 1 patients were 75% and 82%, respectively. Cox regression analysis showed that patients listed as status 1 (aHR: 0.45, p<.02) were associated with a better survival. BCS patients listed as status 1 have excellent survival following emergency LT.

Post-Cardiac Injury Following Liver Transplantation for Budd-Chiari Syndrome.

Post-Cardiac Injury Following Liver Transplantation for Budd-Chiari Syndrome.

Post-transplant Epstein–Barr Virus-Related Lymphoproliferative Disorder: A Case Report and Review of Literature

AbstractPost transplant lympho-proliferative disorders (PTLD) are a serious complications of solid organ transplantation. Solid organ recipients have an increased risk of cancers related to immunosuppression and the Epstein-Barr virus (EBV)-in particular lymphomas and majority of PTLD are of B-cell origin. The occurrence of PTLD in solid organ recipients can have varied clinical presentation and histopathological features. Although lymphoproliferative disorders were initially reported to be rare complication of transplantation, observations in past decade shown that they are common and are associated with poor outcomes. We report the case of a patient with deceased donor liver transplantation for Budd-chiari syndrome, who presented, four years after liver transplantation, with an EBV-associated Burkitt lymphoma with gastrointestinal and extensive skeletal metastasis recovered completely after adjustment of immunosuppressive treatment and chemo-immunotherapy. Our suggestion is that patients with the risk factors like T-cell depleting agents or increasing immunosuppressive therapy must be closely monitored with quantitative EBV PCR. Improvements in immunosuppressive strategies for transplantation and advances in treatment resulted in improved outcomes and long term survival for patients with PTLD.

Hanging Hepato-Atrial Anastomosis in Deceased-Donor Liver Transplantation for Budd-Chiari Syndrome With Extensive Vena Cava Obliteration: A Case Report

IntroductionAlthough outcomes of liver transplantation (LT) have improved as the result of progress in surgical procedures, a failure to restore sufficient graft outflow may yield fatal consequences including graft dysfunction and even graft loss to date. In particular, patients with pre-existing obliterated venous drainage, such as those with Budd-Chiari syndrome (BCS), are at high risk of having venous complications followed by conventional LT. In selected cases, the transplant surgeons are compelled to modify the surgical procedures of LT from the conventional procedure. MethodsWe describe an LT performed in a BCS patient with complete inferior vena cava (IVC) obstruction. A procedure that we named “hanging hepato-atrial anastomosis” was performed, in which 2 major modifications were made. One modification was the dissection of the lower inlet of the right atrium by use of a trans-abdominal approach and hepato-atrial anastomosis. This was performed by exposing the thoracic IVC through a trans-abdominal approach. The other modification was the manufacture of a blind pouch from the graft's infra-hepatic IVC without anastomosis. ResultsModifications were made possible as the result of meticulous examination of the patient's vascular anatomy before the operation. Fortunately, the patient had a heavy network of pre-vertebral veins that drained blood from the lower extremity and both kidneys to the azygos–hemi-azygos veins. ConclusionsWe learned that a meticulous assessment of vascular anatomy and complete understanding of hemodynamics are the keys to the successful LT for BCS in patients with extensive IVC abnormality. Thoracotomy may not be necessary to explore thoracic IVC when performing hepato-atrial anastomosis in LT for BCS.

Living-Donor Liver Transplantation for Budd-Chiari Syndrome: Case Series

BackgroundVenous reconstruction in living-donor liver transplantation for Budd-Chiari syndrome (BCS) has challenges because the grafts from living donors lack vena cava, and hepatic venous anastomosis must be performed on an already-thrombosed and/or stenosed inferior vena cava. Several techniques are described to overcome this problem, and we represent our experience with 22 patients. MethodsMedical recordings of 22 patients were retrospectively collected, and disease-specific data as well as recordings about surgical technique were analyzed. ResultsCreation of a wide, triangular de novo orifice was the main method used for venous drainage, which was used in 19 patients. The remaining 3 patients had totally thrombosed vena cava; thus, direct anastomosis to the supra-hepatic portion of the vena cava was used in 2 patients and an anastomosis to the right atrium was used in 1 patient. ConclusionsVenous reconstruction in BCS can be achieved without the use of patch-plasty, and the inferior vena cava can be safely resected in selected patients. Living-donor liver transplantation is a feasible option for the treatment of BCS, considering the scarcity of cavaderic donors.

Hepatic Venous and Inferior Vena Cava Morphology No Longer a Barrier to Living Donor Liver Transplantation for Budd-Chiari Syndrome: Surgical Techniques and Outcomes.

Living donor liver transplantation (LDLT) for Budd-Chiari syndrome (BCS) has been reported with<10 inferior vena cava (IVC) replacements with vascular/synthetic graft. The goal of this study was to review outcomes of LDLT for BCS at our center, with an emphasis on surgical techniques and postoperative anticoagulation therapy. Between October 2011 and December 2015, a total of 1027 LDLTs were performed. Nine of these patients had BCS. We analyzed their etiologies, operative details, postoperative complications, and outcomes. The indication was chronic liver disease for all patients. Two patients required retrohepatic IVC replacement with a polytetrafluoroethylene graft due to severe adhesions and thrombosis, respectively. One patient required V-Y plasty for suprahepatic IVC narrowing. Five patients had portal venous thrombosis, 3 treated by thrombectomy, and 1 by renoportal anastomosis. The mean follow-up time was 18 ± 16 months. Only 1 early death occurred due to sepsis. The anticoagulation therapy involved heparin infusion from postoperative day 1, conversion to low-molecular-weight-heparin on postoperative days 3 to 6, followed by warfarin (postoperative days 9-16 to maintain an international normalized ratio of 2-3 long term), along with low-dose aspirin for 6 months. There was no recurrence of thrombosis. LDLT for BCS is well documented in literature. Prevention of recurrent thrombosis depends on meticulous surgical technique, perfect and wide outflow anastomoses, and a strict anticoagulation protocol. A synthetic (polytetrafluoroethylene) graft for IVC interposition is a safe and feasible option for reconstruction with good results. Low-dose aspirin with low-molecular-weight-heparin later converted to warfarin provides excellent results and prevents recurrence of thrombosis.

Living donor liver transplantation for Budd-Chiari syndrome: Overcoming a troublesome situation.

Background:The aim of the study was to report the detailed surgical techniques of living donor liver transplantation (LDLT) in patients with Budd–Chiari syndrome (BCS).Methods:Demographic and surgical techniques characteristics of 39 patients with BCS who underwent LDLT were retrospectively reviewed. Thirty-two of them had native vena cava inferior (VCI) preservation and 6 had retrohepatic VCI resection with venous continuity established by cryopreserved VCI (n = 4) or aortic graft (n = 2). In 1 patient, the anastomosis was established between the graft hepatic vein (HV) and the suprahepatic VCI. For preservation of the native VCI, immediately before the graft implantation, the thickened anterior, and right/left lateral walls of the recipient VCI were resected caudally and cranially until the intact vein wall was reached, and then an anastomosis was created between the (HV) of the graft reconstructed as a circumferential fence and the reconstructed recipient VCI. For resection of the retrohepatic VCI, the anastomosis was created with the same technique in all 6 patients in whom VCI was reformed by using a vascular graft.Results:Post-LT complications developed in 19 of the patients. Complications related to the biliary anastomosis accounted for 12 of these cases, with 11 treated by PTC and/or ERCP, and 1 by hepaticojejunostomy. Two of the 39 patients developed recurrent BCS and were treated by interventional radiological methods. Thirteen patients died and none were related to the BCS recurrence.Conclusion:Favorable outcomes are achievable with LDLT treatment of patients with BCS, which carries important implications for countries with inadequate cadaveric donor pools.

Liver Transplantation for Budd-Chiari Syndrome With Large Solitary Focal Nodular Hyperplasia of the Liver in a Patient With Essential Thrombocytemia: Case Report

Budd-Chiari syndrome is a rare condition caused by interrupted hepatic venous outflow in the hepatic veins, inferior vena cava, or right atrium. Reports from the literature have delineated on focal nodular hyperplasia (FNH)–like lesions in association with Budd-Chiari Syndrome. To our knowledge, there are no reports about true FNH lesions in patients with Budd-Chiari Syndrome. Focal nodular hyperplasia develops in disorders with aberrant circulation and vasculature. We report a case of Budd-Chiari syndrome in association with large solitary FNH in a 22-year-old man who was referred to our institution with sudden intermittent right upper quadrant abdominal pain, vomiting, diarrhea with pale stool, decreased appetite, dark urine, and abdominal distention for 15 days. Laboratory investigations revealed anemia, thrombocytosis, and abnormal liver function tests and coagulation profile. Imaging revealed hepatic vein thrombosis, confirming Budd-Chiari syndrome, and a 6.2 × 6.1 × 6.8 cm lesion in segment 8 of the liver. Primary cause of Budd-Chiari syndrome was essential thrombocythemia according to bone marrow biopsy and molecular testing results. The patient was treated medically and underwent transjugular intrahepatic portosystemic shunt insertion. The lesion in segment 8 continued to enlarge. Cadaveric liver transplantation was carried out. On gross and histologic examination of the explanted liver, the lesion was found to be a true FNH.

Living donor liver transplantation for Budd–Chiari syndrome with hepatic inferior vena cava obstruction after open pericardial procedures

Living donor liver transplantation (LDLT) for Budd-Chiari syndrome (BCS) presents a unique challenge as it does not involve replacement of the hepatic inferior vena cava (IVC). We report a case of successful LDLT in a patient with BCS associated with occlusion of the hepatic veins as well as the IVC. A 34-year-old woman with a history of two open pericardial procedures had decompensated liver failure and portal hypertension. Venography showed complete obstruction of the hepatic IVC and well-developed collateral vessels. We performed LDLT via sternotomy and laparotomy, with an end-to-end anastomosis between the left hepatic vein of the donor and the patient's suprahepatic vena cava in the pericardium. The patient recovered uneventfully and has been doing well for 5years. LDLT without caval replacement for BCS in a patient with hepatic IVC obstruction is feasible if the patient has good functional collaterals before liver transplantation.

Long-Term Prognosis of Myeloproliferative Disorders (MPD) after Liver Transplantation for Budd-Chiari Syndrome

Long-Term Prognosis of Myeloproliferative Disorders (MPD) after Liver Transplantation for Budd-Chiari Syndrome

Long-Term Follow-Up of Liver Transplantation for Budd-Chiari Syndrome With Antithrombotic Therapy Based on the Etiology

Because myeloproliferative disorders (MPDs) are a frequent cause of Budd-Chiari syndrome (BCS), treatment directed toward altering platelet production and function may be more rational and effective than anticoagulation after liver transplantation. We reviewed data on 25 patients who received liver transplantation for BCS at our institution from 1987 to 2007. Posttransplant antithrombotic treatment was based on the cause of BCS: 17 patients with MPDs received hydroxyurea/aspirin; 5 received warfarin; and 3 (2 whose hypercoagulable disorder was corrected and 1 with sarcoidosis) received no therapy. Both graft survival (88% at 5 years) and patient survival (92% at 5 years) were superior in the BCS group compared with the 2609 patients who received liver transplants for other indications. Vascular complications included three instances of hepatic artery stenosis (NS compared with non-BCS liver recipients), one of portal vein thrombosis (nonsignificant [NS]), and one of portal vein stenosis (NS). All 25 patients underwent multiple liver biopsies with no bleeding complications. Using hydroxyurea and aspirin to treat patients with BCS caused by an MPD seems to be safe and effective and avoids the risks of anticoagulation with warfarin.

OP26 Predicting thrombotic complications after liver transplantation in patients with Budd Chiari syndrome

IntroductionMyeloproliferative disorders (MPD) are the commonest cause of Budd Chiari syndrome (BCS). A somatic mutation of the tyrosine kinase JAK2 gene (JAK2V617F) is present in a large proportion of patients...

Live donor liver transplantation for Budd-Chiari syndrome: Anastomosis of the right hepatic vein to the right atrium

Live donor liver transplantation for Budd-Chiari syndrome: Anastomosis of the right hepatic vein to the right atrium

Liver transplantation for Budd-Chiari syndrome: When is it really necessary?

Liver transplantation for Budd-Chiari syndrome: When is it really necessary?