warfarin and Budd-Chiari-Syndrome

Budd-Chiari syndrome is a rare disorder characterised by hepatic venous outflow obstruction. It affects 1.4 per million people, and presentation depends upon the extent and rapidity of hepatic vein occlusion. An underlying myeloproliferative neoplasm is present in 50% of cases with other causes including infection and malignancy. Common symptoms are abdominal pain, hepatomegaly and ascites; however, up to 20% of cases are asymptomatic, indicating a chronic onset of hepatic venous obstruction and the formation of large hepatic vein collaterals. Doppler ultrasonography usually confirms diagnosis with cross-sectional imaging used for complex cases and to allow temporal comparison. Myeloproliferative neoplasms should be tested for even if a clear causative factor has been identified. Management focuses on anticoagulation with low-molecular-weight heparin and warfarin, with the new oral anticoagulants offering an exciting prospect for the future, but their current effectiveness in Budd-Chiari syndrome is unknown. A third of patients require further intervention in addition to anticoagulation, commonly due to deteriorating liver function or patients identified as having a poorer prognosis. Prognostic scoring systems help guide treatment, but management is complex and patients should be referred to a specialist liver centre. Recent studies have shown comparable procedure-related complications and long-term survival in patients who undergo transjugular intrahepatic portosystemic shunting and liver transplantation in Budd-Chiari syndrome compared with other liver disease aetiologies. Also, the optimal timing of these interventions and which patients benefit from liver transplantation instead of portosystemic shunting remains to be answered.

Topics: Abdominal Pain; Adult; Anticoagulants; Ascites; Budd-Chiari Syndrome; Heparin, Low-Molecular-Weight; Hepatomegaly; Humans; Liver Transplantation; Middle Aged; Portasystemic Shunt, Transjugular Intrahepatic; Prognosis; Ultrasonography, Doppler, Color; Warfarin

Relapsing polychondritis is a rare immune-mediated condition, characterized by episodic inflammation of the cartilaginous tissue, in particular the ears, nose, and eyes, and involvement of joints and respiratory tract. Nearly one third of patients showed other associated diseases, such as systemic vasculitides, connective tissue diseases, or myelodysplastic syndromes. Antiphospholipid antibodies can be found in relapsing polychondritis in patients with no clinical thrombotic disease. However, when antiphospholipid syndrome is present, its clinical manifestations can be severe and life threatening. We describe the case of a patient with relapsing polychondritis associated to Budd-Chiari syndrome due to antiphospholipid syndrome. The present clinical observations together with the updated review of the literature suggest a search for antiphospholipid antibodies in all patients with relapsing polychondritis.

Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Azathioprine; Budd-Chiari Syndrome; Comorbidity; Humans; Immune System; Immunologic Factors; Immunosuppressive Agents; Male; Middle Aged; Polychondritis, Relapsing; Prednisone; Treatment Outcome; Warfarin

Adamantiades-Behçet's disease (ABD) is a relapsing vasculitis of unknown aetiology and variable clinical manifestations. The syndrome can be presented in a myriad of ways and can involve nearly every organ. Although vascular involvement is not included among the ABD diagnostic criteria, it is a unique clinical manifestation in adults with a potentially devastating outcome. We report an ABD case, presenting with a thrombotic occlusion of the inferior vena cava. The authors review the recent literature, emphasizing the spectrum of vascular manifestations accompanying Behçet's disease.

Topics: Adult; Aged; Anticoagulants; Behcet Syndrome; Budd-Chiari Syndrome; Diagnosis, Differential; Fibrinolytic Agents; Humans; Male; Radiography; Vena Cava, Inferior; Warfarin

Topics: Budd-Chiari Syndrome; Diuretics; Drug Therapy, Combination; Humans; Hydroxyurea; Liver Transplantation; Warfarin

Topics: Adult; Blood Coagulation Tests; Budd-Chiari Syndrome; Catheterization; Female; Heparin; Humans; Labor, Obstetric; Pregnancy; Pregnancy Complications, Cardiovascular; Urokinase-Type Plasminogen Activator; Warfarin

Topics: Abdominal Pain; Adult; Anemia, Hemolytic; Antibodies, Monoclonal, Humanized; Anticoagulants; Budd-Chiari Syndrome; Complement Inactivating Agents; Drug Therapy, Combination; Ferritins; Hemoglobin A; Hemoglobinuria, Paroxysmal; Hemolysis; Hepatomegaly; Humans; Male; Tomography, X-Ray Computed; Treatment Outcome; Warfarin

Splanchnic vein thrombosis (SVT) is an uncommon but potentially life-threatening disease usually related to different underlying clinical conditions. The risk of SVT recurrences is high over time in patients with an underlying permanent prothrombotic condition. Vitamin K antagonists (VKA) represent the mainstay of treatment for SVT. Data about the efficacy and safety of direct oral anticoagulants (DOACs) are reported in the literature for the treatment of acute SVT, but less is known about their application for the secondary prophylaxis of venous thromboembolism (VTE). The aim of this study was to assess the efficacy and safety of long-term DOACs therapy in patients at high-risk of thrombosis, compared to VKA.. This is a retrospective single-centre study including 70 patients with SVT on long-term anticoagulant treatment with VKA followed-up at our Units between January 2017 and December 2019. All the patients were at high thrombotic risk defined as the presence of a permanent prothrombotic condition requiring long-term anticoagulation. During follow-up, 28 patients were shifted to DOACs and their clinical outcomes were compared to those of the patients who continued VKA therapy. All the arterial and venous thrombotic events of the splanchnic and extra-splanchnic districts as well as the haemorrhagic adverse events occurring during follow-up were recorded.. Of the seventy patients enrolled in the study, 36 patients (51.4%) had a single-segment involvement thrombosis (28.5% of portal vein, 7.1% of superior mesenteric vein, 4.3% of splenic vein, 11.5% of hepatic veins) and 34 patients (48.6%) had multi-segment involvement at the time of diagnosis. 42 patients (60%) continued VKA therapy and 28 (40%) were switched to DOACs. Median follow-up was 6 years (range 2-8) during VKA and 1.9 years (range 1-5.2) during DOACs. The incidence of thrombotic events was similar between patients on VKA and those on DOACs. Patients on VKA developed deep vein thrombosis (DVT), and of the patients on DOACs 1 developed NSTEMI and 1 DVT. No major haemorrhagic events occurred. Minor bleedings occurred in 26% of patients on VKA and in none of the DOACs patients (P: 0.09).. Our results highlight that DOACs could represent an effective and safe alternative to the VKA for secondary prophylaxis in SVT patients at high risk of thrombosis.

Topics: Acenocoumarol; Adult; Anticoagulants; Budd-Chiari Syndrome; Duration of Therapy; Factor Xa Inhibitors; Female; Hemorrhage; Humans; Male; Mesenteric Ischemia; Middle Aged; Portal Vein; Pyrazoles; Pyridines; Pyridones; Rivaroxaban; Secondary Prevention; Thiazoles; Venous Thrombosis; Warfarin

Anticoagulation is universally recommended in Budd-Chiari syndrome [BCS]. Vitamin K epoxide reductase complex 1 (VKORC1) and CYP2C9 are involved in the metabolism of warfarin. The present study was done to assess whether these mutations are associated with the risk of bleeding in patients with BCS receiving warfarin.. Patients diagnosed with BCS underwent genotyping for three single nucleotide polymorphisms [SNPs]-two for the CYP2C9 and one for the VKORC1 haplotype. The patients were followed up for at least 12 months and all bleeding episodes were recorded. Patients with and without mutations were compared for bleeding complications and a crude odds ratio [crude OR] was derived for the association between bleeding and presence or absence of mutant alleles.. Eighty patients [mean (SD) age 27.47 (8.93) years, 35 male] with BCS underwent genetic testing. 37/80 (46.2%) patients had mutation of CYP2C9 and/or VKORC1; 22/80 (27.5%) had either of the mutant alleles of CYP2C9 and, similarly, 22/80 (27.5%) had the VKORC mutation. Over a median follow-up of 20 (range 12-96) months, 21/80 (26.3%) patients had bleeding complications. Patients with mutant SNPs had a higher risk of bleeding than those without [14/37 vs. 7/43, p = 0.04, crude OR (95% CI) 3.13 (1.1-8.9)].. The presence of mutations in VKORC1 or CYP2C9 is associated with increased risk of bleeding in patients with BCS on warfarin. Such patients with SNPs of CY2C9 or VKORC1 haplotype should be monitored intensively while receiving warfarin.

Topics: Adolescent; Adult; Alleles; Anticoagulants; Budd-Chiari Syndrome; Cytochrome P-450 CYP2C9; Female; Haplotypes; Hemorrhage; Humans; Male; Middle Aged; Mutation; Polymorphism, Single Nucleotide; Severity of Illness Index; Vitamin K Epoxide Reductases; Warfarin; Young Adult

Topics: Abdominal Pain; Abscess; Adalimumab; Adrenal Cortex Hormones; Anti-Inflammatory Agents; Anticoagulants; Behcet Syndrome; Budd-Chiari Syndrome; Female; Fever; Humans; Liver Abscess; Splenic Diseases; Suction; Tomography, X-Ray Computed; Treatment Outcome; Warfarin; Young Adult

We describe the case of a patient with Budd-Chiari syndrome who presented with an unusual membranous obstruction of the inferior vena cava complicated by massive portal vein thrombosis (PVT). The patient underwent percutaneous transluminal balloon angioplasty through the right groin and was prescribed oral warfarin for 6 months. Treatment resulted in the complete disappearance of the PVT. This therapeutic strategy should be considered in the management of other cases of this rare, complex disease.

Topics: Administration, Oral; Angioplasty, Balloon; Anticoagulants; Budd-Chiari Syndrome; Computed Tomography Angiography; Humans; Male; Middle Aged; Multidetector Computed Tomography; Phlebography; Portal Vein; Treatment Outcome; Vena Cava, Inferior; Venous Thrombosis; Warfarin

If they do not respond to other treatments, patients with Budd-Chiari syndrome are potential candidates for a liver transplant. Timing for transplant is controversial; however, before other systems deteriorate, early intervention in relatively stable patient may improve the outcome and survival of these patients.. Six patients (2 women and 4 men) had Budd-Chiari syndrome (1.2%) among 475 patients who had undergone a liver transplant at our center between 2001 and 2012. Imaging modalities including duplex ultrasound, abdominal computed tomography angiography, and hematologic evaluation were part of our routine diagnostic work-up. Although we perform mostly living-donor liver transplants, these patients received a liver transplant from a deceased donor, because there was not enough evidence to justify a living-donor liver transplant. We thought that not replacing the caval vein might negatively influence the outcome. Postoperatively, these recipients were started on a heparin infusion and triple therapy immunosuppression; only then was warfarin introduced as long-term anticoagulant.. Two patients died, 1 from uncontrollable bleeding and disseminated intravascular coagulopathy, and the other died in the intensive care unit after 5 months because of multiorgan failure and sepsis. One patient had portal vein thrombosis 9 months after the liver transplant; the other patient needed a liver retransplant after 5 years owing to liver failure, secondary to chronic rejection. Graft survival rate was 75%, and patient survival rate was 66.6%.. This is the first article from Saudi Arabia to describe the outcome of a liver transplant in this subgroup of patients with Budd-Chiari syndrome. Treatment of Budd-Chiari syndrome follows a therapeutic algorithm that should start with anticoagulation and may end up with liver transplant; however, it should be considered early if other treatments fail.

Topics: Adult; Algorithms; Anticoagulants; Budd-Chiari Syndrome; Critical Pathways; Diagnostic Imaging; Female; Graft Survival; Hematologic Tests; Heparin; Humans; Immunosuppressive Agents; Liver Transplantation; Male; Middle Aged; Reoperation; Saudi Arabia; Time Factors; Treatment Outcome; Warfarin

Topics: Adult; Anticoagulants; Budd-Chiari Syndrome; Catheterization; Female; Graft Occlusion, Vascular; Humans; Male; Middle Aged; Portal Vein; Portasystemic Shunt, Transjugular Intrahepatic; Stents; Tomography, X-Ray Computed; Ultrasonography, Interventional; Vascular Patency; Vena Cava, Inferior; Warfarin

In recent years there have been increasing evidence associating liver disease with hypercoagulability, rather than bleeding. The aim of the study was to evaluate the haemostatic potential in patients with liver disease.. We measured thrombin generation in the presence and absence of thrombomodulin in patients with portal vein thrombosis (PVT, n=47), Budd-Chiari syndrome (BCS, n=15) and cirrhosis (n=24) and compared the results to those obtained from healthy controls (n=21). Fifteen patients with PVT and 10 patients with BCS were treated with warfarin and were compared to an equal number of patients with atrial fibrillation matched for prothrombin time-international normalized ratio. We assessed resistance to thrombomodulin by using ratios [marker measured in the presence/absence of thrombomodulin].. There were no differences in thrombin generation between patients on warfarin treatment and their controls. Cirrhotic patients generated more thrombin in the presence of thrombomodulin and exhibited thrombomodulin resistance compared to controls [p=0.006 for endogenous thrombin potential (ETP) and p<0.001 for peak thrombin and both ratios ETP and peak] and patients with non-cirrhotic PVT (p=0.001, p=0.006, p<0.001, p<0.001 for ETP, peak, ratio ETP, ratio peak, respectively). The patients with cirrhotic PVT exhibited higher ETP (p=0.044) and peak (p=0.02) in the presence of thrombomodulin than controls, as well as thrombomodulin resistance (ETP and peak ratios: p=0.001).. Hypercoagulability and thrombomodulin resistance in patients with cirrhosis were independent of the presence of splanchnic vein thrombosis. The hypercoagulability in patients with cirrhotic PVT could have implications for considering longer or more intensive treatment with anticoagulants in this group.

Topics: Adult; Aged; Aged, 80 and over; Anticoagulants; Blood Coagulation Tests; Budd-Chiari Syndrome; Female; Hemostasis; Humans; Liver; Liver Cirrhosis; Male; Middle Aged; Splanchnic Circulation; Thrombin; Thrombomodulin; Venous Thrombosis; Warfarin; Young Adult

To evaluate the safety and clinical efficacy of warfarin anticoagulation after balloon dilation alone for the treatment of Budd-Chiari syndrome (BCS) complicated by old inferior vena cava (IVC) thrombosis.. From January 2008 to November 2013, 19 BCS patients complicated with old IVC thrombosis were treated with balloon dilation followed by oral administration of anticoagulant warfarin. Follow-up was performed at 1 week, then 1, 2, 3, 6, and 12 months after balloon dilation, and then annually thereafter. IVC patency and morphologic changes of the old thrombus were examined by ultrasound, and clinical symptoms and signs were determined by clinical examinations during follow-up.. Successful IVC balloon dilation was achieved in the 19 patients (100%). Inferior vena cavography demonstrated the patency of IVC lumen, and the size of the old thrombus was not altered. The mean pressure gradient between IVC and the right atrium was reduced from 27.5 ± 3.0 cm H2O (range, 22-35) before treatment to 5.4 ± 1.3 cm H2O (range: 2-7) after treatment (t = 41.6, P < 0.05; 1 cm H2O = 0.098 kPa). Patients were followed up as outpatients for an average of 15.9 ± 14.4 months (range, 3-66). Anticoagulation with warfarin was well tolerated in all patients after balloon dilation alone. Of the 19 patients, complete resolution of the old thrombus was achieved in 12 patients and partial resolution was achieved in 7 patients. Color Doppler ultrasound showed that 17 patients had IVC lumen patency, and 2 patients had IVC reocclusion. None of the patients had recurrence of thrombosis, symptomatic pulmonary embolism, and bleeding complications throughout the follow-up period.. Our results indicate that warfarin anticoagulation after balloon dilation alone is a safe and effective therapy for BCS patients with old IVC thrombosis.

Topics: Adult; Aged; Angioplasty, Balloon; Anticoagulants; Budd-Chiari Syndrome; Combined Modality Therapy; Diagnostic Imaging; Female; Humans; Male; Middle Aged; Retrospective Studies; Treatment Outcome; Vascular Patency; Vena Cava, Inferior; Venous Thrombosis; Warfarin

Pregnancy management is a crucial issue in women with Budd-Chiari Syndrome (BCS) and there are no established guidelines on the management.. To report our experience of pregnancy outcome with BCS.. We report outcome of 13 pregnancies in three women, with favourable outcome after the diagnosis of the condition and its treatment using intervention to bypass obstruction and anticoagulant therapy during pregnancy.. Three women had a total of 13 pregnancies; three after the diagnosis and decompressive treatment of the disease. Disease was diagnosed during index pregnancy in two women. Anticoagulation was given in all the three pregnancies (Robertson et al., Br J Haematol, 132:171-196, 2006). Pregnancies prior to diagnosis and treatment resulted in a live birth.. Pregnancy does not seem to be a contraindication in well treated and controlled BCS. Maternal outcome is good with close multidisciplinary surveillance. Foetal outcome, however, may still be poor due to underlying prothrombotic condition.

Topics: Adult; Angioplasty; Anticoagulants; Budd-Chiari Syndrome; Combined Modality Therapy; Female; Follow-Up Studies; Heparin; Humans; Pregnancy; Pregnancy Complications, Cardiovascular; Pregnancy Outcome; Prenatal Care; Tertiary Care Centers; Treatment Outcome; Warfarin

Protein C and S are vitamin K-dependent natural anticoagulants. They play a major role in hemostasis by degrading the activated factor V and factor VIII. Deficiencies of protein C and protein S are associated with increased risk of thrombotic events. The combined occurrence of protein C and S deficiency has been rarely reported. We report a 6-year-old boy with Budd-Chiari syndrome due to combined protein C and protein S deficiency. He was managed with low molecular weight heparin and discharged on long-term warfarin therapy.

Topics: Anticoagulants; Budd-Chiari Syndrome; Child; Humans; Male; Protein C Deficiency; Protein S Deficiency; Warfarin

Essential thrombocytosis is extremely rare in children. However, when present, it is associated with increased prevalence of antiphospholipid antibodies and thrombo-hemorrhagic complications. The authors report here a child with Budd-Chiari Syndrome resulting from essential thrombocytosis and associated antiphospholipid antibodies. A 13- y-old boy presented with microcytic hypochromic anemia, hepatosplenomegaly and thrombocytosis. CT scan demonstrated calcified thrombus in inferior vena cava (IVC). Diagnosis of essential thrombocytosis was considered in view of persistent thrombocytosis, antiphospholipid antibodies, bone marrow showing increased number, clusters and giant forms of megakaryocytes and IVC thrombosis. He was started on warfarin prophylaxis and did not have thrombotic recurrence on follow up.

Topics: Adolescent; Anticoagulants; Antiphospholipid Syndrome; Budd-Chiari Syndrome; Chronic Disease; Developing Countries; Diagnosis, Differential; Humans; Male; Thrombocythemia, Essential; Tomography, X-Ray Computed; Warfarin

To evaluate the initial clinical safety and feasibility of anticoagulation using warfarin for Budd-Chiari syndrome (BCS) with chronic inferior vena cava (IVC) thrombosis.. Between October 2005 and June 2009, a total of 16 consecutive BCS patients with chronic IVC thrombosis were treated with warfarin. Warfarin was administered orally at 2.5 mg/d for approximately 3-12 months. Transluminal balloon dilatation of the IVC with a 30-mm balloon catheter was applied for the patients with complete resolution of the thrombus. Data relating to the technical success, angiographic results, complications, and final clinical outcome were collected retrospectively and follow-ups were performed at 1, 3, 6, and 12 months after the stent placement, and annually thereafter.. Warfarin was successfully used for anticoagulation in all patients without any complications. Patients were followed up as outpatients for 6.43 ± 2.19 months, and in 14 cases, complete disappearance of the thrombosis was achieved with successful treatment by balloon dilation. In two patients with partial resolution of the thrombosis, Z-stent placement was initiated to compress the thrombus to prevent migration of the thrombosis, followed by dilation of the IVC. During the follow-up for 20.94 ± 10.31 months after the procedure, all the IVC remained patent without complications or pulmonary embolus, and all patients were alive with resolution of symptoms at the time of this study.. The use of warfarin for anticoagulation proved to be simple, safe, and feasible for BCS with chronic IVC thrombosis.

Topics: Administration, Oral; Adult; Anticoagulants; Budd-Chiari Syndrome; Catheterization; China; Chronic Disease; Drug Administration Schedule; Female; Humans; Male; Middle Aged; Phlebography; Retrospective Studies; Stents; Time Factors; Tomography, X-Ray Computed; Treatment Outcome; Vascular Patency; Vena Cava, Inferior; Warfarin; Young Adult

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.

Topics: Adolescent; Adult; Anticoagulants; Aspirin; Budd-Chiari Syndrome; Child; Female; Fibrinolytic Agents; Follow-Up Studies; Graft Survival; Hepatic Artery; Humans; Hydroxyurea; Liver Transplantation; Male; Middle Aged; Platelet Aggregation Inhibitors; Portal Vein; Postoperative Complications; Thrombosis; Warfarin; Young Adult

The Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction involving the hepatic veins, inferior vena cava, or both. BCS has occasionally been reported in the literature as a very rare complication of ulcerative colitis. However, association of Crohn's disease (CD) and BCS is extremely rare with only a single case reported in the world literature to date. We report a case of a young woman with chronically active, therapy-resistant CD who developed massive ascites, elevation of liver enzymes, and coagulopathy in the course of her disease. She was subsequently diagnosed with BCS for which a successful liver transplantation was performed. Chronically active therapy resistant CD and methylenetetrahydrofolate reductase gene mutation have been identified as possible risk factors for development of BCS in this patient.

Topics: Adrenal Cortex Hormones; Ascites; Budd-Chiari Syndrome; Crohn Disease; Female; Hepatic Veins; Humans; Immunosuppressive Agents; Liver; Liver Transplantation; Methylenetetrahydrofolate Reductase (NADPH2); Mutation; Phlebography; Risk Factors; Tacrolimus; Tomography, X-Ray Computed; Vena Cava, Inferior; Warfarin; Young Adult

Topics: Aged; Anticoagulants; Budd-Chiari Syndrome; Female; Granuloma, Plasma Cell; Humans; Hypertrophy; Liver Diseases; Tomography, X-Ray Computed; Warfarin

Outcome of patients with hepatic venous outflow tract obstruction (HVOTO) has improved with newer treatments, including anticoagulants, radiological interventions and liver transplant. In India, however, liver transplant and radiological interventions are costly and have limited availability. Hence, patients often opt for anticoagulation alone. We followed up a group of such patients to determine the clinical outcome with such treatment.. Consecutive patients with HVOTO, treated with oral anticoagulation and supportive medical therapy but no radiological or surgical intervention, were followed up for at least 12 months. Diagnosis of HVOTO was based on color Doppler, and either angiography or magnetic resonance venography. Warfarin dose was adjusted to maintain international normalized ratio (INR) between 2.0 and 3.0. Patients with secondary HVOTO and those with baseline INR > or = 2.0 were excluded. Response was defined as absence of ascites and/or encephalopathy, normal AST/ALT, bilirubin <1.5 mg/dL, and no portal hypertension related bleed after starting therapy.. Of 43 patients (mean [SD] age=28.7 [8.4] years; 20 men), 26 (61%) had a response during a median follow up of 23 (range 15-33) months. The response first appeared within 2 months of the start of treatment in 18 patients and between 2 and 5 months from the start of treatment in eight patients. Seven patients died of progressive liver failure (6 patients) or GI bleed (1 patient). Nine patients had anticoagulation-related complications. On univariate analysis, short duration of symptoms, high serum albumin, low baseline INR, and low baseline Child-Pugh's (CP) or Clichy scores predicted response. Presence of hepatic encephalopathy, portal vein thrombosis, obstruction of all hepatic veins, low albumin, high INR, high serum bilirubin, high baseline CP score, Murad score and adverse Clichy index were associated with higher mortality rate. However, on multivariate analysis, only low CP score was associated with response, and no factor was found to predict death.. More than half of patients with HVOTO show response with only supportive medical therapy and anticoagulants. This occurs more often in patients with low CP score. Some patients may have delayed response.

Topics: Administration, Oral; Adult; Angiography; Anticoagulants; Budd-Chiari Syndrome; Chi-Square Distribution; Female; Follow-Up Studies; Humans; India; International Normalized Ratio; Logistic Models; Magnetic Resonance Angiography; Male; Statistics, Nonparametric; Treatment Outcome; Ultrasonography, Doppler, Color; Warfarin

Pulmonary embolism (PE) is one of the major complications after percutaneous balloon angioplasty (PTBA) for Budd-Chiari's syndrome (BCS). The purpose of this study was to investigate the role of warfarin pre-treatment in the prevention of PE after PTBA in patients with large inferior vena cava (IVC) thrombus.. From October 2002 to December 2009, 16 patients with symptomatic membranous or segmental IVC occlusion and large thrombus were treated with warfarin before PTBA. Eleven patients were men and 5 were women. The median age was 36 years, ranging from 21 to 52 years. The median duration of warfarin treatment before PTBA was 7 months, ranging from 3 to 12 months. Fourteen patients had membranous IVC occlusion and 2 had segmental occlusion. All 16 patients had significant thrombi underneath the obstructive lesions. PE diagnosis was based on clinical presentation and pulmonary computerized tomographic angiogram, if indicated.. In 14 of 16 patients, IVC thrombus was completely or near-completely resolved based on follow-up cavogram and PTBA was performed. In the other 2 patients, residual thrombus was demonstrated by cavogram at 12 months. PTBA and stent placement were carried out. IVC patency in the 16 patients was confirmed by completion cavogram. No major bleeding complication during warfarin pre-treatment aimed to keep international normalized ratio (INR) 2 to 3. There was no clinically significant PE or death in this group during follow-up, ranging from 6 to 40 months (median 21 months).. Spontaneous fibrinolysis of IVC thrombus occurs within 1 year in the majority of the patients treated with warfarin. Pre-treatment with warfarin prevents PE after PTBA in the patients with BCS with IVC membranous or segmental occlusion and large thrombus.

Topics: Adult; Angioplasty, Balloon; Anticoagulants; Budd-Chiari Syndrome; China; Drug Administration Schedule; Female; Humans; Male; Middle Aged; Phlebography; Pulmonary Embolism; Stents; Time Factors; Tomography, X-Ray Computed; Treatment Outcome; Vena Cava, Inferior; Venous Thrombosis; Warfarin; Young Adult

We describe an unusual case of a 54-year-old woman who was diagnosed with a subacute Budd-Chiari syndrome due to membranous venous obstruction in the inferior vena cava. The unusual feature of this case was that she had been diagnosed with pulmonary emboli a few years earlier and was on lifelong warfarin with a therapeutic international normalised ratio. She was effectively treated by venoplasty.

Topics: Anticoagulants; Budd-Chiari Syndrome; Female; Humans; Middle Aged; Pulmonary Embolism; Vena Cava, Inferior; Warfarin

Porto-caval hemitransposition (PCH) in liver transplantation allows revascularization of the liver when the porto-mesenteric axis is thrombosed. We, here, review our experience over an 11-year period. A total of 23 patients underwent liver transplantation using PCH. Immunosuppression was based on tacrolimus, with sirolimus used in case of renal insufficiency. Most common diagnoses were hepatitis C, Laennec's, Budd-Chiari and cryptogenic cirrhosis. Six patients needed splenectomy prior to transplant, 5 during transplant, 1 post-transplant, 11 had no splenectomy. Overall survival was 60% at 1 year and 38% at 3 years, with 10 of 23 patients currently alive and the longest survivor at 9.3 years. Most common cause of death was sepsis/multisystem organ failure, followed by pulmonary embolism. A total of 7/23 patients experienced post-operative gastrointestinal bleeding episodes, 6/23 patients developed thrombosis of the vena cava (median 162 days post-op). Post-operative ascites was noted in almost all patients. Renal dysfunction was commonly seen even after the first month post-transplant. PCH offers a feasible option for liver transplantation in those patients with complex thrombosis of the mesenteric and portal circulation.

Topics: Adult; Anticoagulants; Budd-Chiari Syndrome; Female; Follow-Up Studies; Humans; Immunosuppression Therapy; Kidney; Liver Diseases; Liver Transplantation; Longitudinal Studies; Male; Middle Aged; Portacaval Shunt, Surgical; Portal Vein; Survival Rate; Warfarin

Topics: Adult; Anticoagulants; Budd-Chiari Syndrome; Female; Humans; Labor, Induced; Nadroparin; Portasystemic Shunt, Surgical; Pregnancy; Pregnancy Complications, Hematologic; Premature Birth; Protein C Deficiency; Uterus; Warfarin

Topics: Adult; Ascites; Budd-Chiari Syndrome; Factor V; Hemoglobinuria; Hemoglobinuria, Paroxysmal; Heterozygote; Humans; International Normalized Ratio; Male; Methylenetetrahydrofolate Dehydrogenase (NAD+); Mutation; Prothrombin; Spleen; Thrombophilia; Time Factors; Tomography, X-Ray Computed; Warfarin

We report a patient with Budd-Chiari syndrome (BCS) and extrahepatic portal obstruction (EHO) associated with congenital antithrombin (AT) III deficiency. A 35-year-old man was admitted to Nishi Kobe Medical Center for evaluation of abnormal intrahepatic veins. By various imaging modalities, BCS and EHO were diagnosed. Laboratory data revealed parallel decreases in activity and antigen concentration of AT III despite normal liver function. Taken together, the etiology of both BCS and EHO was considered to be thrombosis, associated with congenital AT III deficiency. Two years after beginning warfarin therapy, the patient has no symptoms and his liver function remains normal. Anticoagulant therapy is considered useful for preventing progression of the disease.

Topics: Adult; Anticoagulants; Antithrombin III Deficiency; Budd-Chiari Syndrome; Humans; Japan; Male; Portal System; Warfarin

We have treated a 33-year-old Budd-Chiari patient (due to antiphospholipid syndrome) with a history of myocardial infarction by placing a vascular stent in the inferior vena cava and performing a portorenal shunt with three objectives: (1) to perform a shunt operation on a Budd-Chiari patient with good hepatic functional reserve, (2) to avoid a thoracotomy and manipulation of the heart in a patient with a cardiac thrombus and a history of myocardial infarction and (3) to avoid a synthetic graft in a patient with antiphospholipid syndrome. Vena cava stenting and portorenal shunt make a useful combination which should be included in the armamentarium of the hepatobiliary surgeon.

Topics: Adult; Anticoagulants; Budd-Chiari Syndrome; Constriction, Pathologic; Female; Follow-Up Studies; Humans; Portacaval Shunt, Surgical; Stents; Vena Cava, Inferior; Warfarin

Membranous obstruction of the inferior vena cava is a rare cause of hepatic venous outflow obstruction in Caucasians. There has been much debate in the literature about its aetiology.. We describe a Caucasian with hepatic venous outflow obstruction due to an inferior vena cava web, who was found to have hypercoagulability due to factor V Leiden. Following balloon rupture of the membrane and anticoagulation, his symptoms resolved and he has remained well for a year.. The age at presentation in this patient, the presence of hypercoagulability and the excellent response to membrane rupture and anticoagulation suggest that in this case the membrane may have been derived from organised thrombus. Balloon rupture of the membrane and anticoagulation appears to be an effective treatment in such cases.

Topics: Anticoagulants; Blood Coagulation Disorders; Budd-Chiari Syndrome; Catheterization; Factor V; Follow-Up Studies; Hepatic Veno-Occlusive Disease; Humans; Male; Middle Aged; Protein C; Vena Cava, Inferior; Warfarin

Topics: Budd-Chiari Syndrome; Developing Countries; Female; Hematologic Tests; Humans; Middle Aged; Radiography; Ultrasonography; Vena Cava, Inferior; Warfarin

Hepatic venous thrombosis (HVT) should be recognized as a distinct and highly lethal thrombotic complication of paroxysmal nocturnal hemoglobinuria. In a patient with fulminant onset prompt recognition of a triad of clinical, laboratory and liver scan findings facilitated early, aggressive and prolonged heparinization followed by coumadin maintenance, all with good results. Additionally a case of asymptomatic, smoldering HVT was unearthed by liver scan survey and confirmed by hepatic venogram; the patient was started on a regimen of Coumadin (crystalline sodium warfarin, Endo).

Topics: Budd-Chiari Syndrome; Emergencies; Hemoglobinuria, Paroxysmal; Heparin; Humans; Male; Middle Aged; Warfarin

Although venous thrombosis (thrombophlebitis) is well known, there are uncommon manifestations which are seen infrequently, discussed rarely, and documented poorly. Experiences with 38 patients in seven categories are discussed in terms of our results and the pertinent reports of others. Pulmonary necrosis after embolic pulmonary infarction (six patients) may require tube thoracotomy and/or lung resection and contraindicate further heparin therapy. Iliac and/or femoral vein thrombosis occasionally fails to recanalize. Long-standing occlusion (18 patients) may be benefited by a cross-over saphenous vein graft. Left iliac venous occlusion secondary to pressure from the crossing right iliac artery (four patients) may indicate repair or bypass. Budd-Chiari syndrome (thrombosis of the hepatic venous outflow) was, in a single patient, carried past a critical period by a long Dacron tube shunt graft from the umbilical vein to the azygos vein. Subclavian and axillary venous thrombosis due to thoracic outlet pressure syndrome (three patients) often responds to heparin but may require thrombectomy; later resection of the first rib is indicated. Phlegmasia cerulea dolens (blue phlebitis) with tissue gangrene (three patients) requires immediate venous thrombectomy and subsequent heparinization. The occluded inferior vena cava (three patients) remains a challenging unsolved problem.

Topics: Adult; Aged; Arteries; Blood Pressure; Blood Vessel Prosthesis; Budd-Chiari Syndrome; Child; Female; Femoral Vein; Gangrene; Heparin; Humans; Iliac Vein; Male; Middle Aged; Necrosis; Pulmonary Embolism; Subclavian Vein; Thoracic Outlet Syndrome; Thrombophlebitis; Transplantation, Autologous; Veins; Vena Cava, Inferior; Warfarin

Topics: Adult; Animals; Antibodies; Budd-Chiari Syndrome; Complement Fixation Tests; Complement System Proteins; Female; Guinea Pigs; Hemoglobinuria, Paroxysmal; Hemolysis; Humans; Inulin; Male; Middle Aged; Prednisolone; Rabbits; Sheep; Snakes; Sucrose; Venoms; Warfarin