Recanalization and Reconstruction of a Chronically Occluded Inferior Vena Cava Through an Existing Transjugular Intrahepatic Portosystemic Shunt in the Setting of Budd-Chiari Syndrome.

Budd-Chiari syndrome (BCS) results from the occlusion or flow reduction in the hepatic veins or inferior vena cava and can be treated with transjugular intrahepatic portosystemic shunt when hepatic vein recanalization fails.1-3 Hypercoagulable patients with primary BCS are predisposed to development of new areas of thrombosis within the TIPS shunt or IVC. This case details a patient with BCS, pre-existing TIPS extending to the right atrium, and chronic retrohepatic IVC thrombosis who underwent sharp recanalization of the IVC with stenting into the TIPS stent bridging the patient until his subsequent hepatic transplantation.

An Update on the Management of Budd-Chiari Syndrome.

Budd-Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review.

Sequential interventional therapy for Budd-Chiari syndrome associated with fresh inferior vena cava thrombosis.

OBJECTIVE: Our study aimed to evaluate the safety and efficacy of sequential interventional therapy for Budd-Chiari syndrome (BCS) caused by obstruction of the inferior vena cava (IVC) with fresh thrombus in the IVC. METHODS: Full medical records were obtained for 20 patients with BCS associated with fresh IVC thrombus who received sequential interventional therapy from 2014 to 2019 at our hospital. All patients underwent small-diameter percutaneous transluminal angioplasty (PTA) balloon catheter predilation combined with sequential catheter-directed thrombolysis and large-diameter PTA balloon dilation. Ultrasound examinations were performed at 1 week, 1 month, 3 months, and every 6 months thereafter. Therapeutic effects and perioperative and postoperative adverse effects were recorded to assess the safety of the treatment. RESULTS: All 20 patients were treated with small PTA balloon catheters (diameter, 10-14 mm) to predilate the occlusive segment of the IVC. Urokinase 400,000 to 600,000 (465,000 ± 93,000) units was administered to patients through the catheter for 6 to 20 (9.7 ± 4.2) consecutive days postoperatively. Ultrasound re-examination showed that the IVC thrombus disappeared completely in 14 patients (70.0%), and a small amount of the old thrombus remained in 6 patients (30.0%). After thrombolysis, all 20 patients received PTA balloon dilation (diameter, 26-30 mm) in the stenosed IVC segment, and blood flow recovered subsequently. No pulmonary embolism or death occurred in the perioperative course. The perioperative survival rate was 100.0%. CONCLUSIONS: Sequential interventional therapy for BCS associated with fresh IVC thrombus is safe and effective.

[Primitive Budd-Chiari syndrome: a diagnostic and therapeutic challenge]. / Budd-Chiari primitif: défi diagnostique et thérapeutique.

Primary Budd-Chiari syndrome is an hepatopathy characterized by obstruction of the hepatic venous flow in the vascular space between the hepatic venules and the junction between the inferior vena cava and the right atrium, thus excluding the causes of upstream (sinusoidal obstruction syndrome) and downstream (cardiac causes). This endoluminal venous obstruction is mainly due to thrombosis or its fibrotic consequences. This is a rare disease, mainly affecting young adults. Clinical manifestations are extremely variable; it can be asymptomatic, acute, subacute or chronic. The diagnosis is mainly based on Doppler ultrasound and/or magnetic resonance imaging. Several causes have been identified, in particular, myeloproliferative syndrome, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, inherited thrombotic disorders. Symptomatic and etiologic treatments are the commonly used approaches, treatment recommendations are organized in algorithm. Recent therapeutic advances can significantly improve the prognosis.

Effectiveness and Postoperative Prognosis of Using Preopening and Staged Percutaneous Transluminal Angioplasty of the Inferior Vena Cava in Treating Budd-Chiari Syndrome Accompanied with Inferior Vena Cava Thrombosis.

BACKGROUND: Budd-Chiari syndrome (BCS) is a rare disorder that has relatively high prevalence in the Huang-Huai area of China. Effective treatment of BCS accompanied with inferior vena cava thrombosis is challenging. OBJECTIVE: This study retrospectively analyzed the clinical effectiveness and safety of traditional open operations versus preopening and staged percutaneous transluminal angioplasty (PTA) of the inferior vena cava in treating BCS accompanied with inferior vena cava thrombosis. METHODS: Data from patients hospitalized and treated for BCS accompanied with inferior vena cava thrombosis between January 1997 and December 2017 were retrospectively analyzed. Thirty-two patients received traditional open operation (open group). Fifty-six patients received preopening and staged PTA of the inferior vena cava (PTA group). Baseline and clinical data were compared between groups. The patients were followed for up to 60 months. Postoperative recurrence rates and restenosis degree were recorded. RESULTS: Eighty-eight patients were included (47 males and 41 females), aged 41.82 ± 10.12 years (range 29-65). In the open group, no pulmonary arterial embolism was found during and after the operation, and the technique success rate was 100%. One patient died of intrathoracic bleeding. In the PTA group, 2 patients had shifting of thrombus in the inferior vena cava that blocked the blood flow restored by the preopening, one resulted in treatment failure, while the other had blood flow restored by dilation with a 12-mm balloon; the success rate was 55/56 (98.21%). The median follow-up time was 32 months (range 3-60). Two patients in the open group developed restenosis 2 years after operation (recurrence rate: 6.25%), and were successfully treated by balloon PTA. Seven patients in the PTA group had severe restenosis 18-42 months after operation (recurrence rate: 12.96%). No thrombosis was found in these 7 patients, and normal blood flow was restored in the inferior vena cava after balloon PTA. CONCLUSIONS: Preopening and staged PTA of the inferior vena cava is a safe and simple method for the treatment of BCS accompanied with inferior vena cava thrombosis, with satisfactory treatment effectiveness that could be applied in clinical practice.

Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome.

BACKGROUND: Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by obstruction in the hepatic venous outflow tract. AIM: To provide an update of the pathophysiology, aetiology, diagnosis, management and follow-up of BCS. METHODS: Analysis of recent literature by using Medline, PubMed and EMBASE databases. RESULTS: Primary BCS is usually caused by thrombosis and is further classified into "classical BCS" type where obstruction occurs within the hepatic vein and "hepatic vena cava BCS" which involves thrombosis of the intra/suprahepatic portion of the inferior vena cava (IVC). BCS patients often have a combination of prothrombotic risk factors. Aetiology and presentation differ between Western and certain Asian countries. Myeloproliferative neoplasms are present in 35%-50% of European patients and are usually associated with the JAK2-V617F mutation. Clinical presentation is diverse and BCS should be excluded in any patient with acute or chronic liver disease. Non-invasive imaging (Doppler ultrasound, computed tomography, or magnetic resonance imaging) usually provides the diagnosis. Liver biopsy should be obtained if small vessel BCS is suspected. Stepwise management strategy includes anticoagulation, treatment of identified prothrombotic risk factors, percutaneous revascularisation and transjugular intrahepatic portosystemic stent shunt to re-establish hepatic venous drainage, and liver transplantation in unresponsive patients. This strategy provides a 5-year survival rate of nearly 90%. Long-term outcome is influenced by any underlying haematological condition and development of hepatocellular carcinoma. CONCLUSIONS: With the advent of newer treatment strategies and improved understanding of BCS, outcomes in this rare disease have improved over the last three decades. An underlying haematological disorder can be the major determinant of outcome.

Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis.

Budd-Chiari syndrome and non-cirrhotic non-tumoral portal vein thrombosis are 2 rare disorders, with several similarities that are categorized under the term splanchnic vein thrombosis. Both disorders are frequently associated with an underlying prothrombotic disorder. They can cause severe portal hypertension and usually affect young patients, negatively influencing life expectancy when the diagnosis and treatment are not performed at an early stage. Yet, they have specific features that require individual consideration. The current review will focus on the available knowledge on pathophysiology, diagnosis and management of both entities.

Liver and Spleen Stiffness Measurements for Assessment of Portal Hypertension Severity in Patients with Budd Chiari Syndrome.

Aims: Budd-Chiari Syndrome (BCS) is a rare vascular disease of the liver caused by the obstruction of the hepatic venous outflow located from the small hepatic venules up to the entrance of the inferior vena cava (IVC) into the right atrium. Current prognostic indexes are suboptimal for an individual prognostic assessment and subsequent management of patients with BCS. Liver (LSM) and spleen (SSM) stiffness measurements are widely validated prognostic tools in hepatology, but the evidence in patients with BCS is limited. This paper describes LSM and SSM in patients with BCS and their correlation with clinical, biochemical, and ultrasound findings from the same patients. Methods: We investigated a case series of seven patients with BCS diagnosis and available LSM and SSM evaluated by transient elastography (TE). Biochemical, imaging, and endoscopic findings nearest to the TE evaluation were recorded. Clinical outcomes and BCS evolution were described for each patient. When available, repeated TE assessments were also recorded. Results: Patients with acute nonfulminant manifestation of BCS presented near-the-upper-limit values (75 kPa) of LSM and SSM, which often persist until the placement of a transjugular intrahepatic portosystemic shunt (TIPS). On the other hand, TE values were markedly lower in patients with compensated BCS. In some patients with repeated TE measurement years after TIPS placement, LSM had decreased to values of <10 kPa years. SSM changes in these patients were, however, less evident. Conclusions: Extremely elevated values of LSM and SSM are suggestive of BCS. The evaluation of both LSM and SSM by TE could help clinicians in the initial evaluation, risk stratification, and therapy response monitoring of patients with BCS.

Effect of Transjugular Intrahepatic Portosystemic Shunt Creation on Pulmonary Gas Exchange in Patients with Hepatopulmonary Syndrome: A Prospective Study.

PURPOSE: To evaluate effect of transjugular intrahepatic portosystemic shunt (TIPS) creation on pulmonary gas exchange in patients with hepatopulmonary syndrome (HPS). MATERIALS AND METHODS: All patients with cirrhosis or Budd-Chiari syndrome undergoing elective TIPS creation at a single institution between June 2014 and June 2015 were eligible for inclusion. Twenty-three patients with HPS (age 55.0 y ± 14.4; 11 men; Model for End-Stage Liver Disease score 10.2 ± 2.7) who achieved technical success were included in the analysis. Diagnosis of HPS was established by contrast-enhanced echocardiography demonstrating intrapulmonary vascular dilatation and arterial blood gas analysis demonstrating arterial oxygenation defects. RESULTS: Mean portosystemic gradient was reduced from 21.7 mm Hg ± 8.3 before TIPS creation to 10.8 mm Hg ± 5.1 after TIPS creation. Among the 5 (21.7%) patients who experienced dyspnea, 4 (80.0%) reported improvement after TIPS creation. This improvement was not maintained at 3 months after TIPS creation in 2 (50.0%) patients. Compared with before TIPS creation, mean change in alveolar-arterial oxygen gradient for patients with HPS was statistically significant at 1 month (-9.2 mm Hg ± 8.0; P < .001) after TIPS creation, but not at 2-3 days (-0.9 mm Hg ± 10.5; P = .678) or 3 months (-3.4 mm Hg ± 11.8; P = .179) after TIPS creation. CONCLUSIONS: TIPS creation can transiently improve pulmonary gas exchange in patients with HPS.

Radical surgical treatment of Budd-Chiari syndrome through entire exposure of hepatic inferior vena cava.

OBJECTIVE: Therapies for Budd-Chiari syndrome (BCS) can be divided into three main categories: medical, endovascular, and surgical. Surgery is applicable to the disease when other therapeutic options have failed. We introduce a surgical method of recanalization through exposure of the entire hepatic inferior vena cava (IVC) and hepatic vein (HV) outflow tract for BCS and investigate the long-term outcomes. METHODS: From July 2002 to December 2015 in our center, 83 consecutive symptomatic BCS patients with failure of endovascular therapy were treated by radical surgical recanalization. IVC recanalization was the first goal for all patients, and recanalization of at least one HV was the second goal for selected patients at the same surgical operation. Patients were followed up, and data on technical and clinical success, survival, and patency of target vessels were analyzed. RESULTS: Technical success of surgical recanalization was achieved in 80 patients (96.4%), with relief of clinical symptoms and improvement of liver function. During a mean follow-up of 84 ± 25.9 months, the cumulative 1-, 3-, and 5-year primary patency rates of the HV were 96.7%, 90.0%, and 83.3%, respectively. The cumulative 1-, 3-, and 5-year primary patency of the IVC was 86.7%, 71.7%, and 68.3%, respectively. No factor demonstrated significant association with recurrence of obstruction. During follow-up, 10 patients died, 8 of end-stage hepatic disease and 2 of unknown causes. The cumulative 1-, 3-, and 5-year all-cause survival rates were 91%, 90%, and 87%, respectively. Female sex, encephalopathy, severe ascites, and hypersplenism had an impact on survival in univariate analysis. With Cox regression, encephalopathy was the only independent determining factor for surgical survival. CONCLUSIONS: Surgical recanalization through exposure of the entire hepatic IVC for BCS is suitable for most primary BCS patients after failure of endovascular therapies.

Efficacy of anti-TNF alpha in severe and refractory major vessel involvement of Behcet's disease: A multicenter observational study of 18 patients.

OBJECTIVE: To describe the outcome and tolerance in patients treated with anti-TNFα in severe and refractory major vessel disease in Behçet's disease (BD). METHODS: A multicenter study evaluating 18 refractory BD patients with major vessel involvement [pulmonary artery (n = 4), aorta (n = 4) or peripheral artery aneurysm (n = 1) and/or pulmonary artery (n = 7), inferior vena cava (n = 5), or intra-cardiac (n = 3) thrombosis or Budd Chiari Syndrome (n = 2)] treated with anti-TNFα agents. RESULTS: Vascular remission was achieved in 16 (89%) patients. The 9 months risk of relapse was significantly higher with conventional immunosuppressants used prior anti-TNFα agents as compared to anti-TNFα therapy [OR = 8.7 (1.42-62.6), p = 0.03]. The median daily dose of corticosteroids significantly decreased at 12 months. Side effects included infection (n = 4) and pulmonary edema (n = 1). CONCLUSION: TNFα-antagonists are safe and might be associated with a decreased risk of relapse at 9 months compared to conventional immunosuppressants in BD patients with major vessels disease.

Angioplasty for Budd-Chiari Syndrome in a Child with 26-Year Follow-up.

Budd-Chiari syndrome (BCS) is a rare but life-threatening disease. If not treated promptly, it is almost always lethal with progressive liver failure, severe nutritional depletion, and renal failure at the late stage. We report the successful treatment of a 5-year-old boy with BCS due to a stenosis of the inferior vena cava (IVC) with a 26-year follow-up. We performed a percutaneous transluminal angioplasty, which resulted in a great improvement of BCS and the disappearance of ascites. Restenosis occurred at 25 years after the initial angioplasty, for which balloon angioplasty was repeatedly performed. The IVC was patent at 1-year follow-up after the second angioplasty.

An Individualised Strategy and Long-Term Outcomes of Endovascular Treatment of Budd-Chiari Syndrome Complicated by Inferior Vena Cava Thrombosis.

OBJECTIVES: The aim was to evaluate individualised treatment and long-term outcomes of endovascular treatment of Budd-Chiari syndrome (BCS) complicated by inferior vena cava (IVC) thrombosis. METHODS: Between April 2005 and December 2015, 108 consecutive patients with BCS complicated by IVC thrombosis underwent endovascular treatment. According to the type, size, extent, and degree of organisation of the thrombus, agitation thrombolysis (n = 7), agitation thrombolysis combined with retrieval stent filter (n = 5), pre-dilation (n = 32), retrieval stent filter (n = 56), or direct large balloon dilation (n = 8) was performed. Peri- and post-operative follow-up data were recorded. RESULTS: The endovascular treatment was technically successful in 107 of the 108 patients (99.1%). The incidence of thrombosis related complications was 7.4% (8/108). Major and minor complications occurred in four patients. The mean follow-up duration was 61.7 ± 39.3 months (range 3-140 months). The cumulative 1, 2, 5, and 10 year primary patency rates were 91%, 88%, 79%, and 79%, respectively, and the cumulative 1, 2, 5, and 10 year secondary patency rates were 100%, 100%, 97%, and 97%, respectively. The cumulative 1, 5, and 10 year survival rates were 95%, 86%, and 81%, respectively. Serum albumin and total bilirubin values were independent predictors of survival. CONCLUSIONS: For patients with BCS complicated by IVC thrombosis, an individualised endovascular treatment strategy based on the type, size, extent, and degree of organisation of the thrombus is associated with long-term patency of the IVC and favourable survival and complication rates.

Direct reopening of the occluded hepatic veins of Budd-Chiari syndrome: verification of our operative method by the perioperative course of esophageal varices.

OBJECTIVE: A total of 69 patients with Budd-Chiari syndrome (BCS) were operated by direct approach under cardiopulmonary bypass (CPB). To assess the operative procedure, the perioperative course of esophageal varices (EVs) was evaluated. PATIENTS AND METHODS: Of the 69 patients, 59 (22 females) were enrolled in this study because they had complete follow-up data for endoscopic evaluation of EVs. Their mean age was 46.3 ± 13.0 years (range 21-73.3 years). EVs were found in 52 patients. Under partial cardiopulmonary bypass, the inferior vena cava (IVC) was incised. The obstruction of the IVC was excised, and the occluded hepatic veins were reopened. The incised IVC was reconstructed with an auto-pericardial patch. RESULTS: Postoperatively, the repaired IVC was patent in all patients. The average number of patent hepatic veins (HVs) increased from 1.23 ± 0.81 to 2.21 ± 0.97/patient. The pressure gradient between the IVC and right atrium (RA) decreased from 12.4 ± 5.52 to 4.46 ± 3.21 mmHg. The indocyanine green clearance test (ICG) at 15 min decreased from 31.57 ± 17.44 to 22.27 ± 15.23%. EVs had disappeared in 13 patients at discharge and in 6 patients at late postoperative follow-up. CONCLUSION: Our operative procedure for BCS is useful for decreasing portal pressure, which is reflected by disappearance of EVs. Therefore, the high risk of EV rupture could be avoided by reopening the occluded HVs.

Budd-Chiari syndrome with upper gastrointestinal hemorrhage: Characteristic and long-term outcomes of endovascular treatment.

Purpose To identify the characteristics and evaluate the long-term outcomes of endovascular treatment of Budd-Chiari syndrome with upper gastrointestinal hemorrhage. Methods Forty-seven consecutive Budd-Chiari syndrome patients with upper gastrointestinal hemorrhage were referred for the treatment with percutaneous transluminal balloon angioplasty, and subsequently underwent follow-up. Data were retrospectively collected and follow-up observations were performed at 1, 2, 2-5, and 5-8 years postoperatively. Results Cirrhosis was presented in 16 patients, and splenoportography reviewed obvious varices in 18 patients. Percutaneous transluminal balloon angioplasty was technically successful in all patients. Major procedure-related complications occurred in 3 of the 47 patients (6.38%). The cumulative 1, 2, 2-5, and 5-8 year primary patency rates were 100% (46/46), 93.2% (41/44), 90.9% (40/44), and 86.4% (19/22), respectively. The cumulative 1, 2, 2-5, and 5-8 year secondary patency rates were 100% (47/47), 100% (44/44), 100% (44/44), and 95.5% (21/22), respectively. Mean and median duration of primary patency was 65.17 ± 3.78 and 69.0 ± 5.69 months, respectively. No upper gastrointestinal hemorrhage recurred during follow-ups. The mean survival time was 66.97 ± 3.61 months and the median survival time was 69.0 ± 4.10 months. Conclusion PTBA was an effective treatment that can prevent recurrence of the life-threatening complications and ensured long-term satisfactory clinical outcomes for Budd-Chiari syndrome patients with upper gastrointestinal hemorrhage. Percutaneous transhepatic variceal embolization was not recommended for all Budd-Chiari syndrome patients with upper gastrointestinal hemorrhage.

Splanchnic vein thrombosis in myeloproliferative neoplasms: risk factors for recurrences in a cohort of 181 patients.

We retrospectively studied 181 patients with polycythaemia vera (n=67), essential thrombocythaemia (n=67) or primary myelofibrosis (n=47), who presented a first episode of splanchnic vein thrombosis (SVT). Budd-Chiari syndrome (BCS) and portal vein thrombosis were diagnosed in 31 (17.1%) and 109 (60.3%) patients, respectively; isolated thrombosis of the mesenteric or splenic veins was detected in 18 and 23 cases, respectively. After this index event, the patients were followed for 735 patient years (pt-years) and experienced 31 recurrences corresponding to an incidence rate of 4.2 per 100 pt-years. Factors associated with a significantly higher risk of recurrence were BCS (hazard ratio (HR): 3.03), history of previous thrombosis (HR: 3.62), splenomegaly (HR: 2.66) and leukocytosis (HR: 2.8). Vitamin K-antagonists (VKA) were prescribed in 85% of patients and the recurrence rate was 3.9 per 100 pt-years, whereas in the small fraction (15%) not receiving VKA more recurrences (7.2 per 100 pt-years) were reported. Intracranial and extracranial major bleeding was recorded mainly in patients on VKA and the corresponding rate was 2.0 per 100 pt-years. In conclusion, despite anticoagulation treatment, the recurrence rate after SVT in myeloproliferative neoplasms is high and suggests the exploration of new avenues of secondary prophylaxis with new antithrombotic drugs and JAK-2 inhibitors.

Percutaneous Transluminal Angioplasty for Complete Membranous Obstruction of Suprahepatic Inferior Vena Cava: Long-Term Results.

PURPOSE: To determine the long-term results of percutaneous transluminal angioplasty (PTA) for a complete membranous obstruction of the suprahepatic inferior vena cava. METHODS: Patients (n = 65) who were referred to the interventional unit for PTA for a complete membranous obstruction of the suprahepatic inferior vena cava between January 2006 and October 2014 were included in the study. Thirty-two patients (18 males, 14 females, mean age 35 ± 10.7, range 20-42 years) were treated. The patients presented with symptoms of ascites (88 %), pleural effusion (53 %), varicose veins (94 %), hepatomegaly (97 %), abdominal pain (84 %), and splenomegaly (40 %). Transjugular liver access set and re-entry catheter were used to puncture and traverse the obstruction from the jugular side. PTA balloon dilations were performed. The mean follow-up period was 65.6 ± 24.5 months. The objective was to evaluate technical success, complications, primary patency, and clinical improvement in the symptoms of the patients. RESULTS: The technical success rate was 94 %. In two patients, obstruction could not be traversed. These patients underwent cavoatrial graft bypass surgery. There were no procedure-related complications. Clinical improvements were achieved in all patients within 3 months. The primary patency rate at 4 years was 90 %. There was no primary assisted patency. There was no need for metallic stent deployment in the cohort. The secondary patency rate at 4 years was 100 %. CONCLUSIONS: Percutaneous transluminal angioplasty for a complete membranous obstruction of the suprahepatic inferior vena cava is safe and effective, and the long-term results are excellent.

Outcome of the Z-expandable metallic stent for Budd-Chiari syndrome and segmental obstruction of the inferior vena cava.

OBJECTIVE: Treatment of segmental obstruction of the inferior vena cava (SOIVC) with Z-expandable metallic stents (Z-EMS) is controversial and data on long-term follow-up are lacking. We aimed to evaluate the long-term outcomes of the use of a Z-EMS for Budd-Chiari syndrome (BCS) patients with SOIVC. MATERIALS AND METHODS: Between August 2004 and December 2014, 37 consecutive BCS patients with SOIVC were referred for Z-EMS treatment and subsequently underwent follow-up in our department. Data were collected retrospectively and follow-up observations were made 1, 2, 2-5, and 5-10 years postoperatively. RESULTS: Percutaneous transluminal balloon angioplasty and Z-EMS placement were technically successful in all patients. Major procedure-related complications occurred in four of 37 patients (10.81%). Follow-up for 61.89±41.45 months in 37 patients indicated portal hypertension in one patient 4 months after stent placement and symptoms were resolved by transjugular intrahepatic portosystemic stent shunting. Hepatocellular carcinoma was observed in four patients and five patients died during follow-up. Reocclusion of the inferior vena cava occurred in four patients (10.81%, 4/41) and all reocclusions were managed by percutaneous transluminal balloon angioplasty. Cumulative 1-, 2-, 2-5-, and 5-10-year primary patency rates were 94.60% (35/37), 93.33% (28/30), 88.89% (24/27), and 85.0% (17/20), respectively. Cumulative 1-, 2-, 2-5-, and 5-10-year secondary patency rates were 100% at all time-points. CONCLUSION: These data suggest that Z-EMS implantation is an efficacious, safe, and curative approach for BCS with SOIVC because satisfactory long-term outcomes were achieved. Long-term follow-up is required to ascertain stent patency and hepatocellular carcinoma occurrence.

[Experience in treating portal thromboses in patients with chronic myeloproliferative diseases].

Patients with myeloproliferative diseases (MPD) are noted to be at high risk for portal thromboses. This problem gives rise to disability if it is untimely treated or resistant to therapy. The paper gives the experience of the Outpatient Department of the Hematology Research Center, Ministry of Health of the Russian Federation, in using antithrombin III in MPD patients (3 patients with primary myelofibrosis, 3 with essential thrombocythemia) and acute and subacute portal vein thromboses resistant to therapy with direct anticoagulants. In all 5 cases, the use of antithrombin III in combination with low-molecular-weight heparin showed a positive clinical effect as rapid relief of pain syndrome and comparatively early (3-week to 1.5-2-month) recanalization of thrombosed vessels. Three clinical cases are described in detail.