Hepatic Vein Occlusion Research Articles

Combining occlusion of the right hepatic vein with the Pringle maneuver in laparoscopic anatomic right posterior liver resection.

Laparoscopic right posterior anatomic resection (LRPAR) presents challenges due to uncontrollable hemorrhage from the inferior vena cava and the risk of carbon dioxide (CO2) gas embolism. However, there is a lack of research specifically addressing the safe exposure of right hepatic vein (RHV). Herein, we introduced a novel technique of combining occlusion of the RHV with the Pringle maneuver and presented the outcomes of our initial series. All consecutive patients who underwent LRPAR using this novel technique were enrolled in this study from March 2021 to January 2024. The demographic characteristics, perioperative outcomes and follow-up data were collected and analyzed. A total of 12 patients underwent LRPAR using the technique of double occlusion during study period. All the procedures were performed laparoscopically, with no conversions to open surgery. The median operative time was 203min (range of 172-279min) and the median blood loss was 200ml (range of 50-280ml). No patient received a blood transfusion during the perioperative period. Of note, the main trunk of the RHV was fully exposed on the cutting surface in all cases, and no evidence of CO2 gas embolism was observed following double occlusion. None of the patients suffered from Clavien-Dindo grade II or higher postoperative complications, and the perioperative mortality was nil. The median postoperative stay was 5days (range of 5-7days). The median hospitalization cost was 43,048.5 RMB (40,240.35-57,921.53 RMB). At a median follow-up period of 24months (range of 4-35months), all patients were alive with normal daily living and no disease recurrence was observed. Combining occlusion of the right hepatic vein with the Pringle maneuver appears to be a feasible and expected technique for securing the exposure of RHV in LRPAR. Further follow-up and well-designed prospective comparative studies are needed to validate the feasibility and efficacy of this technique.

Liver venous deprivation in the prevention of post-resection hepatic failure

The paper presents a clinical observation of successful X-ray endovascular intervention performed in a patient with colorectal cancer metastases in the liver in order to prevent post-resection hepatic failure. Traditional embolization of the right branch of the portal vein was supplemented by occlusion of the right hepatic vein. Venous deprivation enabled a rapid growth of the future liver remnant to be achieved as indicated by CT volumetry results and SPECT-CT data that showed a significant increase in hepatic clearance. The first results demonstrate that liver venous deprivation is to be investigated further in clinical practice.

Caudo-dorsal approach combined with the occlusion of right hepatic vein and Pringle maneuver in laparoscopic anatomical resection of segment 7.

Laparoscopic anatomical resection of segment 7 (LARS7) remains a technically challenging procedure due to the deep anatomical location and the potential risk of injury to the right hepatic vein (RHV). Herein, we initiated an innovative technique of caudo-dorsal approach combined with the occlusion of the RHV and Pringle maneuver for LARS7 and presented the outcomes of our initial series. Since January 2021, the patients who underwent LARS7 by using this novel technique were enrolled in this study. The critical aspect of this technique was the interruption of communication between the RHV and the inferior vena cava. Meanwhile, the Pringle maneuver was adopted to control the hepatic inflow. A total of 11 patients underwent LARS7 by using this novel technique, which included 8 hepatocellular carcinoma, 2 bile duct adenocarcinoma and one focal nodular hyperplasia. The median operative time was 199min (range of 151-318min) and the median blood loss was 150ml (range of 50-200ml). The main trunk of the RHV was fully exposed on the cutting surface in all cases and no patient received perioperative blood transfusion. No procedure was converted to open surgery. Of note, no indications of CO2 gas embolism were observed in these cases after the introduction of double occlusion. Only one patient suffered from postoperative complications and healed after treatment. The median postoperative stay was 5days (range of 4-7days). The 90-day mortality was nil. At a median follow-up period of 19months, all of the patients were alive without any evidence of tumor recurrence. The caudo-dorsal approach combined with the occlusion of RHV and the Pringle maneuver may be a feasible and expected technique for safe exposure of RHV in LARS7. Further validation of the feasibility and efficacy of this technique is needed.

Efficacy and safety analysis of selective hepatic vein occlusion combined with arterial chemoembolization versus conventional transarterial chemoembolization in the treatment of hepatocellular carcinoma.

Transarterial chemoembolization (TACE) is a non-radical treatment. How to improve the local response rate of tumor is the direction we have been exploring. The purpose of this study is to compare the efficacy and safety of occlusion-TACE (O-TACE) versus conventional TACE (C-TACE) in the treatment of patients with early to mid-stage hepatocellular carcinoma (HCC) of lesions being confined to the same hepatic venous drainage area. In this study, 40 patients with HCC were prospectively enrolled and randomly assigned to the O-TACE and C-TACE groups, and the efficacy of all patients was assessed at 1, 2, and 3 months after the first treatment, and adverse events (AEs) occurring during the treatment period were also recorded. At 1, 2 and 3 months after the first treatment, patients in the O-TACE group had significantly better complete response (CR) rates (35% vs. 5%, P=0.04; 50% vs. 15%, P=0.04; 70% vs. 30%, P=0.02) than those in the C-TACE group. The most common AE was abdominal pain, and the liver function indexes of patients in both groups returned to the baseline level at 1 month after the first treatment, and there was no statistically significant difference in the rate of AEs between the two groups. O-TACE has a better CR rate and a favorable safety profile in patients with early to mid-stage HCC of lesions being confined to the same hepatic venous drainage area.

Gut microbiota regulates hepatic ischemia-reperfusion injury-induced cognitive dysfunction via the HDAC2-ACSS2 axis in mice.

Hepatic ischemia-reperfusion injury (HIRI) resulting from hepatic inflow occlusion, which is a common procedure in liver surgery is inevitable. Previous research has confirmed that the cognitive dysfunction induced by HIRI is closely related to dysbiosis of the gut microbiota. This research aims to investigate the mechanisms underlying this complication. C57BL/6 mice underwent hepatic ischemia experimentally through the occlusion of the left hepatic artery and portal vein. To assess the HDAC2-ACSS2 axis, gut microbiota transplantation. Enzyme-linked immunosorbent assay and LC/MS short-chain fatty acid detection were utilized. The findings indicated a notable decline in ACSS2 expression in the hippocampus of mice experiencing hepatic ischemia-reperfusion injury, emphasizing the compromised acetate metabolism in this particular area. Furthermore, the cognitive impairment phenotype and the dysregulation of the HDAC2-ACSS2 axis could also be transmitted to germ-free mice via fecal microbial transplantation. Enzyme-linked immunosorbent assay revealed reduced Acetyl-coenzyme A (acetyl-CoA) and Acetylated lysine levels in the hippocampus. These findings suggest that acetate metabolism is impaired in the hippocampus of HIRI-induced cognitive impairment mice and related to dysbiosis, leading to compromised histone acetylation.

Liver transplantation in Budd–Chiari patients with history of portacaval shunt surgery

The paper presents the experience of successful treating a patient with diuretic-resistant ascites associated with Budd– Chiari syndrome. The authors provide a clinical observation of deceased donor liver transplantation to a patient with Budd–Chiari syndrome type 1, total hepatic vein occlusion, and diuretic-resistant ascites after portacaval shunt surgery. The post-transplantation period is observed as stable, with no signs of liver failure; ascites is reduced. The first experience of surgical treatment of a patient with Budd–Chiari syndrome, as well as the first experience of liver transplantation after portacaval shunting is appeared to be successful.

Helical Tomotherapy and Melphalan As the Conditioning Regimen in Autologous Stem Cell Transplantation for Multiple Myeloma with Extramedullary Plasmacytoma: A Single Center Clinical Research

Background Multiple myeloma(MM) is the second hematologic malignancy. Extramedullary myeloma indicates a poorer prognosis and survival. High dose Melphalan is the most widely used conditioning regimen. Helical tomotherapy(TOMO) is one of the most advanced intensity-modulated conformal radiotherapy technologies. We applied the combination of TOMO and Melphalan as the conditioning regimen in MM patients, and analyzed its efficacy and safety. Methods Clinical data were collected and compared between TOMO combined with melphalan group and non-TOMO group. SPSS V.25.0 was used for statistical analysis. Results Two methods were not significant difference by statistics analysis. The complete response rate of 100d in non-TOMO group significantly decreased compared to 1 month after transplantation (P=0.001). The 1-year PFS rate in the TOMO group was 86%, while non-TOMO group was 91%. There was no myeloma related death in the two groups within one year. TOMO group showed earlier enter platelet count below 20x109/L (P=0.003) and granulocyte deficiency (P=0.000). In terms of platelet implantation time,TOMO group was superior in platelet implantation time (P=0.041). No atrial fibrillation, heart failure, hepatic small vein occlusion, or transplant related death occurred during the transplantation period. Conclusion: The combination of TOMO and reduced dose Melphalan conditioning regimen is clinically feasible in terms of safety and efficacy evaluation. This scheme could improve the long-term survival of MM patients with extramedullary plasmacytoma.

The Use of SARS-CoV-2-Positive Donors in Hematopoietic Stem Cell Transplantation

Since 2019, the SARS-CoV-2 pandemic has become a global issue due to its high fatality rate. Over time, the characteristics of the virus have evolved and led to the creation of an omicron strain with higher infectivity but a significantly decreased fatality rate. For patients in urgent need of hematopoietic stem cell transplantation (HSCT), whether the SARS-CoV-2 infection status of donors has a significant impact on HSCT recipients should be clarified. To estimate the transplantation risk of SARS-CoV-2-positive donors, 24 patients who underwent HSCT from December 1, 2022 to January 30, 2023 were retrospectively included. The ratio of the observation group (SARS-CoV-2-positive donors, n=12) to the control group (SARS-CoV-2-negative donors, n=12) was 1:1. We observed the time of hematopoietic reconstruction, donor chimerism, severe infection, acute graft vs host disease, and hepatic vein occlusion disease during hematopoietic reconstruction. In the observation group, the average time of myeloid hematopoietic reconstruction was 11.58 days, and in the control group, it was 12.17 days (P=.3563 [>.05]). On average, all patients achieved a 90% donor chimerism rate of +13.58 (±4.5) days (P=.5121 [>.05]). The average percentage of patients that achieved successful hematopoietic reconstruction was 96.75% in the observation group and 96.31% in the control group (P=.7819 [>.05]). A total of 6 adverse events occurred during this study: 3 in the observation group and 3 in the control group. Our preliminary results showed favorable short-term outcomes in recipients of SARS-CoV-2-positive HCST donors.

Interventional Treatment of Budd–Chiari Syndrome

Medical treatment is regarded as the primary course of action in patients with Budd-Chiari syndrome (BCS). Its efficacy, however, is limited, and most patients require interventional treatment during follow-up. Short-segment stenosis or the occlusion (the so-called web) of hepatic veins or the inferior vena cava are frequent in Asian countries. An angioplasty with or without stent implantation is the treatment of choice to restore hepatic and splanchnic blood flow. The long-segment thrombotic occlusion of hepatic veins, common in Western countries, is more severe and may require a portocaval shunting procedure to relieve hepatic and splanchnic congestion. Since it was first proposed in a publication in 1993, the transjugular intrahepatic portosystemic shunt (TIPS) has gained more and more attention, and in fact it has been so successful that previously utilized surgical shunts are only used for few patients for whom it does not work. Both interventional treatment options can be performed successfully in about 95% of patients even after the complete obliteration of the hepatic veins. The long-term patency of the TIPS, a considerable problem in its early years, has been improved with PTFE-covered stents. The complication rates of these interventions are low and the survival rate is excellent with five- and ten-year survival rates of 90% and 80%, respectively. Present treatment guidelines recommend a step-up approach indicating interventional treatment after the failure of medical treatment. However, this widely accepted algorithm has several points of contention, and early interventional treatment is proposed instead.

Reply: Discussing the place of TIPS in non-cirrhotic patients with chronic extra hepatic portal vein occlusion refractory to standard of care therapy.

Department of Radiology, Section of Vascular and Interventional Radiology, Northwestern University, Chicago, Illinois, USA CorrespondenceRiad Salem ([email protected])

Protective Efficiency Comparison of Direct and Remote Ischemic Preconditioning on Ischemia Reperfusion Injury of the Liver in Patients Undergoing Partial Hepatectomy.

Ischemia reperfusion injury greatly damages liver function and deteriorates the prognosis of patients undergoing partial hepatectomy. This study is to compare the protective efficiency of direct and remote ischemic preconditioning (DIPC and RIPC) on ischemia reperfusion injury of the liver in patients undergoing partial hepatectomy. 90 patients scheduled for partial hepatectomy were enrolled and randomly divided into control (n = 30), DIPC (n = 30), and RIPC (n = 30) groups. Baseline and surgery characteristics were collected, and ischemic preconditioning methods were carried out. Intraoperative hemodynamics, liver function and liver reserve capacity, oxidative stress, and inflammatory responses were measured, and the incidence of postoperative adverse reactions was calculated finally. 10 patients were excluded from the study, and finally, the eligible patients in three groups were 27, 28, and 25, separately. No significant differences were observed in baseline and surgery characteristics among the three groups. SBP and DBP were significantly higher after hepatic portal vein occlusion while they were significantly lower after surgery in the DIPC and RIPC groups compared with that in the control group, SBP and DBP were of great fluctuation at different time points in the control group while they showed much more stabilization in the DIPC and RIPC groups. ALT, AST, and TBIL were significantly decreased on days 1, 3, and 5 after surgery, and ICG R15 was significantly decreased while ICG K value and EHBF were significantly increased on day 1 after surgery in the DIPC and RIPC groups compared with that in the control group. Moreover, antioxidant enzyme SOD was increased, and inflammatory factors TNF-α and IL-1β were decreased 24 hours after surgery in the DIPC and RIPC groups compared with that in the control group. DIPC and RIPC also decreased hospital stays and the incidence of nausea, vomiting, and hypertension. DIPC and RIPC both alleviated ischemia reperfusion injury of the liver and reduced perioperative complications with similar protective efficiency in patients undergoing partial hepatectomy.

Hepatic venous territory mapping in living donor liver transplantation using right liver graft: an objective parameter for venous reconstruction.

This study evaluated the clinical implication of hepatic venous territory mapping in living donor liver transplantation. Living donor liver transplantations performed using right graft since 2017 were included. Hepatic venous volume mapping was started in 2019. Risk factors for graft failure and overall survival were analyzed. Analysis for factors related to occlusion of reconstructed vein was performed. Among 445 patients included, 213 underwent hepatic venous mapping. Hepatic venous mapping itself was not a significant factor for graft (hazard ratio [HR], 0.958; 95% confidence interval [CI], 0.441-2.082; P = 0.913) and overall survival (HR, 0.627; 95% CI, 0.315-1.247; P = 0.183). Inferior hepatic vein occlusion was a significant risk factor for both graft survival (HR, 8.795; 95% CI, 1.628-47.523; P = 0.012) and overall survival (HR, 11.13; 95% CI, 2.460-50.300; P = 0.002). In a subgroup with middle hepatic vein reconstruction, occlusion was a significant risk factor for overall survival (HR, 3.289; 95% CI, 1.304-8.296; P = 0.012). In patients with middle hepatic vein reconstruction whose venous territory volumes were measured, right anterior volume of ≥300 cm3 was protective for vein occlusion (OR, 0.317; 95% CI, 0.152-0.662; P = 0.002). In patients with V5 reconstruction, V5 volume of ≥150 cm3 was protective for vein occlusion (OR, 0.253; 95% CI, 0.087-0.734; P = 0.011). Inferior and middle hepatic vein reconstruction has significant impact on clinical outcome. Hepatic venous territory mapping can provide an objective measure for successful reconstruction of venous branches.

Current diagnostics and treatment of portal hypertension

In industrial nations portal hypertension is mostly aconsequence of liver cirrhosis and is aprerequisite for complications, such as esophageal varices and ascites. The pathophysiology of portal hypertension is complex. It is defined as an increase in the hepatovenous pressure gradient to > 5 mm Hg, with complications to be expected at ≥ 10 mm Hg. Measurement of the pressure of the hepatic vein occlusion is the gold standard for estimating portal pressure but this is not very practical. Liver elastography, in particular, has proven to be an effective noninvasive tool to identify patients with clinically significant portal hypertension (CSPH). Current treatment concepts address the CSPH even before the onset of complications to reduce the likelihood of decompensation. In addition to beta blockers, a transjugular intrahepatic portosystemic shunt is the most important procedure to lower portal vein pressure and enables an improvement in the prognosis of selected patients.

The change in the liver volume after direct intrahepatic portocaval shunt in patients with Budd-Chiari syndrome with diffuse hepatic vein occlusion.

To evaluate the changes in the liver volume and function after direct intrahepatic portocaval shunt (DIPS) in patients with Budd-Chiari syndrome (BCS) with diffuse hepatic vein (HV) occlusion. The clinical data of 29 patients with BCS who underwent DIPS for intractable ascites caused by diffuse hepatic vein occlusion in the Affiliated Hospital of Xuzhou Medical University were analysed retrospectively; the patients included 8 males and 21 females, with an average age of 33.3 ± 6.3years. The patients underwent abdominal CT scanning and liver function examinations before DIPS, and 1week, 3months, 6months and 12months after DIPS. The changes of the liver volume and liver function before and after DIPS were compared. All 29 patients underwent DIPS successfully. 28 patients survived during the follow-up of 12-33months, with a median follow-up of 16months. The patients' liver function were significantly improved at 3, 6 and 12months after the operation compared to before the operation (P < 0.05). The liver volumes measured before the operation and 1week, 3months, 6months and 12months after the operation were 2124.586 ± 420.889 cm3, 1926.263 ± 372.268 cm3, 1480.592 ± 183.061 cm3, 1461.904 ± 153.027 cm3 and 1469.286 ± 148.549 cm3, respectively. Compared with the preoperative liver volume, the liver volume had decreased significantly at 1week, 3months, 6months and 12months after the operation (P < 0.05). However, there was no significant difference in the liver volumes at 6 and 12months after the operation (P = 0.35). Direct intrahepatic portocaval shunt has achieved satisfactory clinical results in the treatment of BCS with diffuse hepatic vein occlusion. The congestive hepatomegaly was gradually reduced after the operation. The liver volume (which was defined as the clinical efficacy in this study) remained stable after 6months.

Preclinical evaluation of a veno-venous bypass device for liver transplantation based on the principle of magnetic levitation drive

Objective: To explore the performance of a self-made venous-venous bypass (VVB) device for liver transplantation based on the principle of magnetic levitation drive. Methods: Experimental study was conducted from August 2020 to January 2022. Eight Bama minipigs underwent VVB of hepatic portal vein-femoral vein-internal jugular vein after occlusion of hepatic portal vein and inferior vena cava. The animals were divided into two groups according to the VVB devices used during VVB. A self-made VVB device was used in group A(n=5),and an imported VVB device was used in group B(n=3). The hemodynamic changes of the two groups of animals were compared at 6 time points including before vascular occlusion, during vascular occlusion, 30 minutes, 60 minutes, 90 minutes after the start of VVB, and 30 minutes after vascular opening. In addition,the changes of blood compatibility indexes,intestinal injury indexes,kidney injury indexes and internal environment indexes of the two groups of animals at each time point were compared. The independent samples t test was used for the quantitative data between the two groups with non-repeated measures,and the repeated measures analysis of variance was used for the quantitative data between the two groups with repeated measures. Results: During the VVB of the two devices,the venous drainage was sufficient,and the main manifestations were that the color of the intestine of the Bama miniature pig was ruddy, the peristalsis was normal, and the urine output was normal. There were no significant differences in hemodynamics,blood injure indexes,intestinal injury indexes,kidney injury indexes,neutropil gelatinase-associated lipocalin,and internal environment indexes(all P>0.05).The indexes at 30 minutes after vascular opening in the group A and the group B were as follows:mean arterial pressure were (71.0±7.7)mmHg(1 mmHg=0.133 kPa) and (74.0±8.7)mmHg,central venous pressure were (7.0±1.4)cmH2O(1 cmH2O=0.098 kPa) and (7.7±0.6)cmH2O,heart rate were (131±10) beats/minutes and (132±8)beats/minutes; red blood cell count were (6.43±0.89)×1012/L and (6.32±0.58)×1012/L,hemoglobin were (108.4±5.9)g/L and (110.0±3.5)g/L,free hemoglobin were (78.28±3.96)mg/L and (78.08±4.54)mg/L; intestinal fatty acid binding protein were (2.27±0.49)μg/L and (2.40±0.78)μg/L;creatinine were (68.30±9.77)μmol/L and (79.90±26.91)μmol/L,blood urea nitrogen were (3.94±1.39)mmol/L and (3.45±0.65)mmol/L;neutropil gelatinase-associated lipocalin were (4.02±0.53) μg/L and (3.86±0.23)μg/L;pH value were 7.27±0.04 and 7.23±0.03,lactic acid were (6.18±2.62)mmol/L and (4.30±0.50)mmol/L,concentrations of Na+ were (136.3±3.0)mmol/L and (137.6±1.6) mmol/L,concentrations of K+ were (3.89±0.42) mmol/L and (3.98±0.17)mmol/L,concentrations of Ca2+ were (1.40±0.03)mmol/L and(1.40±0.04)mmol/L;all indexes in the two group had no differences(all P>0.05). Conclusion: The self-made venous bypass device can be safely and effectively applied to VVB of Bama minipigs,and achieves the same performance as the imported venous bypass device.

Role of interventional radiology in management of post-liver transplant anastomotic complications

BackgroundLiver transplantation is considered to be the treatment of choice in cases of end-stage liver disease; however, as a major procedure, the operation is fraught with complications. The etiology, symptoms, and diagnostic methods for arterial, portal, and biliary issues are thoroughly discussed. Interventional procedures such as balloon angioplasty and stent placement in the arterial and portal systems, as well as biliary interventional procedures, are described.ResultsIn our study, we reviewed 25 cases of post-living donor transplanted liver, with anastomotic complications including biliary stenosis 40%, hepatic vein stenosis 20%, portal vein stenosis 16%, biliary leakage 16%, and hepatic artery stenosis or pseudo-aneurysm 16%. We had 10 cases of biliary stenosis, 7 of which were successfully treated with the insertion of an internal/external drain, and one case failed. Two patients died. We had four cases of hepatic venous obstruction with successfully implanted stents and a perfect outcome, efficacy, and patency rate of 100%. We also had two cases of hepatic artery stenosis that were perfectly managed by stent placement, with a patency rate of 100%. We came across two cases of hepatic artery pseudo-aneurysm. One case failed due to large sac size, while the other was successful. Finally, in our study, we had a 100% success rate in 5 cases of portal vein stenosis in the early postoperative period.ConclusionsPercutaneous IR was effective treatment for hepatic vein occlusion, portal vein stenosis, hepatic artery stenosis, and anastomotic biliary stricture after living donor liver transplantation. The interventional radiology team is now an integral part of the multi-disciplinary care of transplant patients. As new interventional instruments are developed and experience is gained, the outcomes of interventional treatments will continue to improve.

Transarterial chemoembolization for hepatocellular carcinoma with vascular invasion.

Although the global guidelines only recommend systemic therapy for hepatocellular carcinoma with vascular invasion, various treatments are performed for it. Among them, transarterial chemoembolization (TACE) is the most frequent option; however, standard techniques have not been established. Conventional TACE (cTACE) has also been frequently performed for tumors invading the portal vein (PVTT), hepatic vein (HVTT), and bile duct (BDTT). In cTACE for PVTT, selective catheterization into the tumor-feeder is essential to avoid adverse effects. However, if it is unsuccessful, injection of embolic agents under balloon occlusion of the hepatic artery can improve the therapeutic effects and avoid hepatic infarction. When marked arterioportal shunts are demonstrated, embolization with gelatin sponge particles soaked with a chemotherapeutic solution is another option. Arteriovenous shunts accompanied by HVTT may cause systemic embolization due to migration of embolic agents, and occlusion of a shunt-draining hepatic vein using a balloon catheter can reduce the risk. BDTT is often accompanied by obstructive jaundice; therefore, endoscopic or percutaneous biliary drainage is required when the serum total bilirubin concentration is ≥3 mg dl-1. TACE should be performed as selectively as possible and attention should also be paid to the risk of obstructive jaundice and/or pancreatitis caused by sloughing of necrotized BDTT.

A Case of Budd-Chiari Syndrome Associated with Erythrocytosis and Homozygous MTHFR C677T Mutation

An erythrocytosis describes an increased erythrocyte, subclassified into relative due to hemoconcentration or absolute by an increase in erythrocyte mass, defined as an increase in hemoglobin concentration and/or hematocrit in the peripheral blood above the sex-specific normal range. Budd-Chiari Syndrome (BCS) is related to an obstruction of the hepatic venous flow leading to occlusion of hepatic veins and their tributaries. Genetic and environmental factors can interact for risk determination of venous thromboembolism. The risk associated with SNP 677C&gt;T and 1298A&gt;C of the methylenetetrahydrofolate reductase (MTHFR), 1691G&gt;A of the Factor V Leiden (FVL) and 20210G&gt;A of the prothrombin (FII) genes were investigated in many studies involving thrombosis. This case report describes the clinical, hematological and biochemistry data about a 48-year-old woman diagnosed with PV and a BCS associated, also carrying 677C&gt;T SNP in homozygosity. The patient started therapy with phlebotomy, hydroxyurea and oral anticoagulant. Currently, she presents a better clinical and laboratory condition with normalized values of hematological and platelet indices. This case report aims to contribute with evidence of related comorbidities and makes it possible to report that genetic factors are involved since the patient's mother had already been diagnosed with absolute erythrocytosis in 2016 at 78 years old. For this main result, we understand that it is clear that a family genetic study can reveal clinical modifying factors in these patients, as there are different clinical severities in the family. Furthermore, we believe in the need for a greater number of randomized clinical trials to add better evidence to complement an ideal therapeutic approach in these patients.

Analysis of curative efficacy of transjugular intrahepatic portosystemic shunt using viabahn stent in the treatment of Budd-Chiari syndrome with extensive hepatic vein occlusion

Objective: To investigate the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) using viabahn stent in patients with Budd-Chiari syndrome (BCS) with extensive hepatic vein occlusion. Methods: Technical success rates, portal pressure gradient (PPG), complications and shunt patency rate of 25 cases with BCS with extensive hepatic vein occlusion treated by TIPS using viabahn stent were retrospectively analyzed. Color Doppler ultrasound was used for postoperative follow-up. Patency rates were evaluated by Kaplan-Meier curve. Results: The technical success rate of TIPS was 100%. Mean PPG decreased from (22.7 ± 6.2) mmHg preoperatively to (9.7 ± 2.6) mmHg postoperatively (t = 9.58, P < 0.05). Hepatic encephalopathy incidence was 8.0% (2/25), and there were no complications such as intra-abdominal hemorrhage. Twenty-five cases were followed up for 1 to 39 ( mean 19.0 ± 11.9) months, and one patient died of liver failure three months after interventional treatment. The primary patency rates at 1, 2, and 3 years after interventional treatment were 91.3%, 91.3%, and 78.2%, respectively, while the repatency rates were 100%, 92.9%, and 92.9%, respectively. Conclusion: TIPS using viabahn stent can bring better short-term and mid-term curative efficacy in treatment of BCS with extensive hepatic vein occlusion.

A SYSTEMATIC LITERATURE REVIEW OF BUDD-CHIARI SYNDROME IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Antiphospholipid syndrome is associated with multiple and recurrent episodes of arterial and venous thrombosis, thrombocytopenia, spontaneous fetal losses, and high levels of antiphospholipid antibodies. These antibodies can also be present in systemic lupus erythematosus (SLE), and predispose patients with Lupus to a hypercoagulable state. Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction that can lead to hepatic congestion and it is deeply associated with acquired or inherited hypercoagulable states. Therefore, the aim of this study was to conduct a systematic literature review to analyze the association between Budd-Chiari syndrome and SLE. A systematic literature review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Articles were selected from PubMed/Medline, SciELO and LILACS databases in August 2021 using the search terms [Lupus] OR [SLE] AND [Budd-Chiari syndrome]. The inclusion criterion was limited to observational studies, case reports or case series that reported an association between Budd-Chiari syndrome and SLE. Language was restricted to English, Spanish or Portuguese. Among 76 articles initially identified, 31 were eligible for this review after full texts were read. The vast majority of the studies have linked the antibodies lupus anticoagulant and anticardiolipin as etiological factors of BCS in patients with lupus. One study has reported six cases in which factor V Leiden was strongly related to SLE, suggesting that this mutation should also be investigated in patients with lupus and BCS. Few studies have mentioned isolated deficiency of protein C and/or protein S as common etiologies of BCS in patients with lupus. Studies suggest that Lupus anticoagulant can be sought in patients with idiopathic hepatic venous thrombosis. Possible mechanisms by which antiphospholipid antibodies induce thrombotic events in patients with SLE have been suggested: it can bind to platelet membrane phospholipids and accentuate their adhesion and aggregation ability, it can interfere in the interactions of proteins C and S and impair the formation of the coagulation control complex (activated protein C, protein S and factor V). Budd-Chiari syndrome can be a rare complication in patients with SLE. A methodical search for hepatic vein occlusion in patients with SLE who develop unexplained hepatomegaly should be performed. In addition, screening for lupus anticoagulant and anticardiolipin antibodies should take place to reduce the percentage of apparently idiopathic BCS cases.