Radiological interventions in HVOTO--practical tips

Hepatic venous outflow tract obstruction (HVOTO) comprises of constellation of disorders causing obstruction of hepatic venous outflow or suprahepatic inferior vena cava (IVC) or both and leading to increased hepatic sinusoidal pressure and portal hypertension. Clinical presentation in HVOTO includes both acute onset or chronic insidious onset of the disease and predominant clinical manifestations consist of ascites, hepatomegaly, and portal hypertension. IVC/hepatic vein (HV) web or thrombosed hepatic veins replaced by fibrotic constriction or thrombus in suprahepatic IVC is encountered as the pathogenic process at such obstructions. Due to advances in radiologic techniques there has been a changes in the management protocol of HVOTO with surgery or liver transplantation reserved for patients not suitable for radiological interventions or requiring liver transplantation. The present article reviews the techniques of various radiological interventions in HVOTO and their efficacy.     

Redefining Budd-Chiari syndrome:A systematic review

AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.     

Radical surgery under genuine direct vision for the treatment of Budd-Chiari syndrome

BACKGROUND: Budd-Chiari syndrome (BCS) develops with complete or incomplete obstruction of the hepatic veins (HV), the super hepatic inferior vena cava (IVC), or both. Various methods have been reported regarding the treatment of BCS. In this article, we present our preliminary experience with radical surgery in the treatment of Budd-Chiari syndrome under genuine direct vision. METHODS: In 13 patients aged from 17 to 48 years, the disease lasted from 3 months to 5 years. Membranous obstruction of the inferior vena cava (IVC) was observed in 3 patients, right hepatic venous (HV) membrane in 1, IVC membrane with distal thrombosis in 6, long-segment thrombosis of the IVC in 2, and IVC thrombosis caused by retroperitoneal tumor extending to the right atrium in 1. RESULTS: All lesions were successfully resected. Extracorporeal circulation was used in one patient, and the cell saver in 2 patients. No blood transfusion was given except for 3 patients receiving blood transfusion of 2000, 400, and 400 ml, respectively. One patient died of renal failure during the postoperative period. Signs and symptoms disappeared after the operation in the remaining patients. CONCLUSION: This new radical surgery gives access to the lesions under clear direct vision in further facilitating the correction needed.     

Inferior vena cava thrombosis at its hepatic portion (obliterative hepatocavopathy)

The Budd-Chiari syndrome was primarily described as hepatic vein thrombosis within the liver, but it now includes inferior vena cava (IVC) thrombosis and other conditions that cause hepatic vein outflow obstruction. This author and several others maintain that primary hepatic vein thrombosis and primary IVC thrombosis represent two different clinical disorders. Primary thrombosis of the IVC most commonly occurs in its hepatic portion, which seems to be predisposed to thrombosis and has been called membranous obstruction of IVC, because the thrombus organizes into a fibrous and frequently membranous occlusion of the IVC. The hepatic vein orifices are affected to varying degrees, resulting in congestive liver damage. The cause of IVC thrombosis may be a hypercoagulable state such as coagulation factor deficiency and myeloproliferative disorders, but is more often idiopathic. In Nepal, it is endemic with a suspected association with infections. To consider IVC thrombosis and the congestive liver damage as a disease entity, this author proposes the term obliterative hepatocavopathy, separate from hepatic vein thrombosis. Clinically obliterative hepatocavopathy is less severe in its acute phase compared with hepatic vein thrombosis, but it aggravates occlusion of hepatic vein orifices with recurrent thrombosis. Primary hepatic vein thrombosis and obliterative hepatocavopathy display different hemodynamics of the hepatic veins, IVC, and portal vein; dilatation of the subcutaneous veins in the body trunk is more pronounced in obliterative hepatocavopathy because the ascending lumbar vein becomes the major collateral route. Congestive liver cirrhosis develops after a long clinical course that may be complicated by hepatocellular carcinoma.     

Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

AIM： To evaluate patterns of obstruction, etiological spectrum and non-surgical treatment in patients with Budd-Chiari syndrome in India.
METHODS： Forty-nine consecutive cases of Budd- Chiari syndrome （BCS） were prospectively evaluated. All patients with refractory ascites or deteriorating liver function were, depending on morphology of inferior vena cava （IVC） and/or hepatic vein （HV） obstruction, triaged for radiological intervention, in addition to anticoagulation therapy. Asymptomatic patients, patients with diuretic-responsive ascites and stable liver function, and patients unwilling for surgical intervention were treated symptomatically with anticoagulation.
RESULTS： Mean duration of symptoms was 41.5 ± 11.2 （range = 1-240） too. HV thrombosis （HVT） was present in 29 （59.1%）, IVC thrombosis in eight （16.3%）, membranous obstruction of IVC in two （4%） and both IVC-HV thrombosis in 10 （20.4%） cases. Of 35 cases tested for hypercoagulability, 27 （77.1%） were positive for one or more hypercoagulable states. Radiological intervention was technically successful in 37/38 （97.3%）： IVC stenting in seven （18.9%）, IVC balloon angioplasty in two （5.4%）, combined IVC-HV stenting in two （5.4%）, HV stenting in 11 （29.7%）, transjugular intrahepatic portosystemic shunt （TIPS） in 13 （35.1%） and combined TIPS-IVC stenting in two （5.4%）. Complications encountered in follow-up： death in five, re-stenosis of the stent in five （17.1%）, hepatic encephalopathy in two and hepatocellular carcinoma in one patient. Of nine patients treated medically, two showed complete resolution of HVT.
CONCLUSION： IN our series, HVT was the predominant cause of BCS. In the last five years with the availability of sophisticated tests for hypercoagulability, etiologies were defined in 85.7% of cases. Non-surgical management was successful in most cases.     

Budd–Chiari syndrome: Focus on surgical treatment

Budd–Chiari syndrome (BCS) is caused by an obstruction in the hepatic venous outflow tract at various levels from small hepatic veins to the inferior vena cava (IVC) due to thrombosis or fibrous sequelae. This rare disease mainly affects young adults. Risk factors have been identified and patients often have multiple risk factors. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients in Europe and North America countries. Multistep management is required for such patients. Interventional revascularization and transjugular intrahepatic portosystemic shunt procedure are indicated after initial anticoagulation therapy, whereas IVC plasty using a patch graft is indicated for obstruction of the IVC. Liver transplantation (LT) is usually indicated as a treatment for liver failure despite various treatments. The outcomes of LT are good, with a 5‐year survival after LT of nearly 70%.     

Nuclear medicine dynamic investigations in the diagnosis of Budd-Chiari syndrome

AIM:To investigate the hepatic hemodynamics in the Budd-Chiari syndrome(BCS) using per-rectal portal scintigraphy(PRPS) and liver angioscintigraphy(LAS).METHODS:Fourteen consecutive patients with BCS were evaluated by PRPS between 2003 and 2012.Ten of them underwent LAS and liver scan(LS) with Tc-99m colloid.Eleven patients had clinical manifestations and three were asymptomatic,incidentally diagnosed at PRPS.The control group included 15 healthy subjects.We used new parameters at PRPS,the liver transit time of portal inflow and the blood circulation time between the right heart and liver.PRPS offered information on the hepatic areas missing venous outflow or portal inflow,length and extent of the lesions,open portosystemic shunts(PSS),involvement of the caudate lobe(CL) as an intrahepatic shunt and flow reversal in the splenic vein.LAS was useful in the differential diagnosis between the BCS and portal obstructions,highlightingthe hepatic artery buffer response and reversed portal flow.LS offered complementary data,especially on the CL.RESULTS:We described three hemodynamic categories of the BCS with several subtypes and stages,based on the finding that perfusion changes depend on the initial number and succession in time of the hepatic veins(HVs) obstructions.Obstruction of one hepatic vein(HV) did not cause opening of PSS.The BCS debuted by common obstruction of two HVs had different hemodynamic aspects in acute and chronic stages after subsequent obstruction of the third HV.In chronic stages,obstruction of two HVs resulted in opening of PSS.The BCS,determined by thrombosis of the terminal part of the inferior vena cava,presented in the acute stage with open PSS with low speed flow.At least several weeks are required in the obstructions of two or three HVs for the spontaneous opening of dynamically efficient PSS.The CL seems to have only a transient important role of intrahepatic shunt in several types of the BCS.CONCLUSION:Dynamic nuclear medicine investigations assess the extent and length of hepatic venous obstructions,open collaterals,areas without portal inflow,hemodynamic function of the CL and reverse venous flow.     

Splanchnic vein thrombosis: clinical presentation,risk factors and treatment

The term splanchnic vein thrombosis encompasses Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO), and mesenteric vein thrombosis; the simultaneous involvement of additional regions is frequent, and clinical presentations and risk factors may be shared. The annual incidence of BCS and isolated mesenteric vein thrombosis is less than one per million individuals, while the incidence of EHPVO is about four per million; autopsy studies, however, suggest higher numbers. Current advances in non-invasive vascular imaging allow for the identification of chronic or asymptomatic forms. Risk factors can be local or systemic. A local precipitating factor is rare in BCS, while it is common in patients with portal vein thrombosis. Chronic myeloproliferative neoplasms (MPN) are the leading systemic cause of splanchnic vein thrombosis, and are diagnosed in half the BCS patients and one-third of the EHPVO patients. The molecular marker JAK2 V617F is detectable in a large majority of patients with overt MPN, and up to 40% of patients without overt MPN. Inherited thrombophilia is present in at least one-third of the patients, and the factor V Leiden or the prothrombin G20210A mutations are the most common mutations found in BCS or EHPVO patients, respectively. Multiple factors are present in approximately one-third of the patients with BCS and two-thirds of the patients with portal vein thrombosis. Immediate anticoagulation with heparin is used to treat patients acutely. Upon clinical deterioration, catheter-directed thrombolysis or transjugular intrahepatic portosystemic shunt is used in conjunction with anticoagulation. Long-term oral anticoagulation with vitamin K-antagonists (VKA) is recommended in all BCS patients, and in the patients with a permanent prothrombotic state associated with an unprovoked EHPVO. In patients with an unprovoked EHPVO and no prothrombotic conditions, or in those with a provoked EHPVO, anticoagulant treatment is recommended for a minimum of 3–6 months.     

Membranous obstruction of the inferior vena cava in the United States

The pathogenesis of membranous obstruction of the inferior vena cava (MOVC) is unclear. Although the lesion is rare in the United States compared to Japan, India, and black South Africa, it has been responsible for 23% of cases of hepatic outflow obstruction we have encountered in the ethnically heterogeneous indigent population of Los Angeles. Most patients with MOVC are male. In contrast, recent series of patients with Budd-Chiari Syndrome (BCS) have demonstrated a female predominance. Compared to BCS without involvement of the inferior vena cava (IVC), patients with MOVC have more chronic symptoms. Large truncal collaterals, particularly on the back, strongly suggest MOVC. In patients without this sign, a high index of diagnostic suspicion is required. Chronic hepatitis B infection occurs with increased frequency in these patients. Chest radiograph may show an enlarged azygous shadow. Liver-spleen scan is not helpful, and the liver biopsy is frequently nondiagnostic. A useful screening procedure for hepatic outflow block is transhepatic portal pressure measurement demonstrating aberrant hepatic veins with pressures higher than in the portal vein and, occasionally, hepatofugal portal flow. Transcardiac membranotomy appears to be symptomatically effective in patients with MOVC and at least one patent hepatic vein. It is not known whether this operation will prolong life and prevent the development of hepatocellular cancer, which may occur in up to 48% of these patients. The correct therapeutic approach has not been established for those patients whose lesion is not amenable to surgery because of extensive IVC occlusion or absence of patent hepatic veins.     

Successful treatment with stent angioplasty for Budd-Chiari syndrome in Behçet’s disease

Budd-Chiari syndrome (BCS) is a very rare vascular complication of Behçets disease (BD) which often leads to death as a result of portal hypertension and liver failure. We report a 45-year-old BD patient who presented with BCS. Diagnosis was confirmed with CT scan and contrast-enhanced MR angiography which showed ascites, short-segment stenosis of the inferior vena cava (IVC), and middle and left hepatic venous thrombosis. Percutaneous transluminal angioplasty (PTA) of the obstructed segment in the IVC was performed and resulted in dramatic reduction of portal venous pressure. Our experience indicates that PTA may be a safe and effective therapeutic modality for BCS in BD which is caused by short segmental obstruction of the IVC.     

布-加综合征疑难病例的介入治疗

介入治疗已成为布一加综合征首选治疗方法。回顾了布-加综合征的影像学诊断以及6种疑难类型,即肝静脉阻塞、下腔静脉长段闭塞、下腔静脉闭塞合并血栓形成、下腔静脉闭塞近心端盲端缺如或很短、广泛肝静脉闭塞和下腔静脉支架放置后阻塞肝静脉的诊治经验。介绍了此6种类型的介入治疗技术要点和注意事项。分析表明,只要充分评估病情,选择合理的治疗方案,将有助于提高疑难布一加综合征病例的介入治疗成功率,进一步提高我国布一加综合征的整体诊疗水平。     

Hypereosinophilia,JAK2V617F,and Budd‐Chiari syndrome: Who is responsible for what?

Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction, which sometimes may be life threatening, with the development of fulminant hepatic failure. In cases of this kind, the most frequent underlying cause of BCS, myeloproliferative neoplasms (MPN), should always be excluded first, and molecular analysis of the Janus Kinase 2 (JAK2) mutation must always be performed [1]. While the association of BCS with polycythemia vera, essential thrombocythemia, and idiopathic myelofibrosis is well documented, hypereosinophilia has only been described in sporadic cases [2–7]. Furthermore, Jak2 mutation in association with hypereosinophilia has been reported very rarely and its prevalence in this disorder still requires further investigation [8,9]. To the best of our knowledge, cases with the above association occurring together with BCS have not been reported until now. Here, we describe a young woman presenting with idiopathic eosinophilia, JAK2 mutation, and BCS. We also elaborate briefly on the biological mechanism and clinical features of this rare entity. In our opinion, this case supports the formal inclusion of hypereosinophilic syndrome (HES) in the WHO MPN category and also raises the possible pathogenetic contribution of eosinophils, or their products, in MPN-associated splanchnic vein thrombosis.     

A case of Budd–Chiari syndrome with Behcet’s disease and oral contraceptive usage

We present a case of Budd–Chiari syndrome (BCS) having two risk factors, Behcet’s disease (BD) and oral contraceptive (OC) usage. A 33-year-old woman with BD was admitted to the Emergency Unit with nausea, vomiting, abdominal pain, abdominal distention, and confusion started 12 days ago before admission. Since the patient was in a shock state, she was taken to the Intensive Care Unit (ICU) with the suspicion of abdomen-originated sepsis. Abdominal ultrasound showed massive hepatosplenomegaly and moderate ascites. Abdominal MRI revealed an inferior vena cava (IVC) obstruction starting above the renal veins and diffuse thrombosis of the right and medial hepatic veins. An extensive thrombosis of the IVC and the hepatic veins (BCS) which led to shock was diagnosed. In addition to BD, the unnotified OC usage for a year by the patient without her doctor’s knowledge was recognized as possible precipitating factor of BCS. Pulse methylprenisolone was started for three consecutive days to treat active BD-induced vasculitis. IVC digital subtraction angiography (DSA) showed occlusion of the IVC below the hepatic veins with extensive collateral circulation originating at the occlusion level suggesting that obliteration had a subacute or chronic course. Since intralesional thrombolytic therapy failed, the patient was transferred to a liver transplantation center. While waiting for an appropriate donor, the patient died due to hepatic failure. Since BCS is mortal and deemed multi-factorial, every patient with a thrombotic risk factor such as BD should be questioned for other possible causes of thrombosis.     

Fulminate intracardiac thrombosis associated with Budd-Chiari-syndrome and inferior vena cava thrombosis

The most common cause of edema of the legs and dyspnea is congestive heart failure. Further differential diagnosis such as renal or hepatic failure have to be considered. We report the case of a previous healthy 65-year-old woman who developed dyspnea and massive edema of the legs followed by acute hepatic and renal failure. Imaging studies showed a thrombosis of the inferior vena cava (IVC) caused by a tumor between the right kidney and the IVC. Histological examination revealed a leiomyosarcoma of the IVC. Hepatic failure due to venous outflow obstruction (Budd-Chiari syndrome, BCS) was diagnosed. Coagulation profile showed a complex disorder due to acute hepatic failure. Factor V Leiden and prothrombin gene mutation G20210A could be excluded. The thrombosis extended from the femoral veins up to the right atrium. After 11 days of anticoagulation with heparin platelet counts decreased by more than 50%. Suspecting a heparin-induced thrombocytopenia the patient was placed on recombinant hirudin (lepirudin) for anticoagulation. Hepatic venogram showed a thrombosis of the hepatic vein orifices but not of the hepatic veins. The tumor and the thrombi were removed surgically. When the cardiopulmonary bypass was terminated new intracardiac thrombi occurred. Despite immediate surgical intervention the patient finally died due to right ventricular failure caused by the fulminate intracardiac thrombosis. In conclusion, thrombosis of the IVC may mimic congestive heart failure and may cause BCS. Neoplasms and coagulation disorders may cause thrombosis of the IVC.     

Membranous obstruction of the inferior vena cava (obliterative hepatocavopathy, Okuda)

Confusion prevails throughout the world regarding the definition and classification of the Budd-Chiari syndrome. The original patients (Budd and Chiari) described had hepatic vein thrombosis, but this syndrome now encompasses various hepatic venous outflow blocks, of which membranous obstruction of the inferior vena cava (IVC) is the most common. This author has been suggesting that the classical Budd-Chiari syndrome or hepatic vein thrombosis and membranous obstruction of IVC or primary thrombosis of IVC at its hepatic portion are epidemiologically, pathologically and clinically different, and that they should be treated as two clinical entities that are not to be mixed. The two diseases have a different onset, different clinical manifestations and a different natural history. Whereas hepatic vein thrombosis is a severe disease with an acute onset, IVC thrombosis presents mildly at onset, but it recurs and eventually turns into a fibrous occlusion of IVC of varying thickness or stenosis of a various degree. The fibrous IVC occlusion is found as a mysterious thin membrane, but is more often much thicker than a membrane, and therefore 'membrane' is a misnomer. Although the genesis is not established, formation of a thin membrane may be an outcome of recurrent thrombosis. The past congenital vascular malformation theory no longer holds, because the disease occurs mostly in adulthood, and transformation of thrombosis into a membrane has now been well documented pathologically as well as clinically. This author suggests that a term 'obliterative hepatocavopathy' replace membranous obstruction of IVC, the term 'Budd-Chiari syndrome' be abandoned, and that primary hepatic venous outflow block be divided into primary hepatic vein thrombosis and primary IVC thrombosis (obliterative hepatocavopathy).     

The transjugular intrahepatic portosystemic stent-shunt (TIPS) as rescue therapy for complete Budd-Chiari syndrome and portal vein thrombosis

We present a 40-year-old female patient with epigastric pain, ascites, and progressive liver failure, caused by Budd-Chiari syndrome (BCS) with thrombotic occlusion of the right and middle hepatic veins. As underlying diseases, essential thrombocythemia and resistance to activated protein C (APC) due to heterozygote factor V Leiden were found. Initial therapy with heparin caused thrombocytopenia (HIT) type II culminating in thrombosis of the last patent left hepatic vein and further deterioration of liver function. The decision against a surgical shunt and liver transplantation by our surgeons on the basis of the risks involved, prompted us to insert a transjugular intrahepatic portosystemic stent-shunt (TIPS). There was no measurable flow signal in the doppler sonography of the portal vein presumably due to thrombosis. A further evaluation with magnetic resonance tomography and angiography was impossible due to movement artefacts. TIPS initially served as a diagnostic tool allowing direct angiography-diagnosed thrombosis of the portal vein, the superior mesenteric and the splenic vein respectively. However, insertion of the TIPS shunt and subsequent fragmentation led to an effective hepatic decompression and full recanalisation of the portal vein. In the present case TIPS simultaneously allowed the diagnosis of portal vein thrombosis and served as rescue therapy of complicated Budd-Chiari syndrome. The potential development of HIT type II should be kept in mind when heparin is given, especially to patients with thrombophilia.     

Budd-Chiari syndrome： Diagnosis with three-dimensional contrast-enhanced magnetic resonance angiography

AIM: To evaluate the role of three-dimensional contrast-enhanced magnetic resonance angiography (3D CE MRA) in the diagnosis of Budd-Chiari syndrome (BCS). METHODS: Twenty-three patients with BCS underwent 3D CE MRA examination, in which 13 cases were secondary to either hepatocellular carcinoma (11 cases), right adrenalcarcinoma (1 case) or thrombophlebitis (1 case) and 10 suffered from primary BCS. The patency of the inferior vena cava (IVC), hepatic and portal veins as well as the presence of intra- and extrahepatic collaterals, liver parenchymal abnormalities and porto-systemic varices were evaluated. Inferior vena cavography was performed in 10 cases. The diagnosis of IVC obstruction by 3D CE MRA was compared with that demonstrated by inferior vena cavography.RESULTS: The major features of BCS could be clearly displayed on 3D CE MRA. Positive hepatic venous signs included tumor thrombosis (9 cases), tumor compression (2 cases), nonvisualization (4 cases) and focal stenosis (2 cases). Positive IVC findings were noted as severe stenosis or occlusion (10 cases), tumor invasion (2 cases), thrombosis (3 cases), thrombophlebitis (1 case) and septum formation (3 cases). Intrahepatic collaterals were shown in 9 patients,2 of them with “spider web“ sign. The displayed extrahepatic collaterals included dilated azygos and hemi-azygos veins (13 cases) and left renal-inferior phrenic-pericardiophrenic veins (2 cases). The occlusion of the left intrahepatic portal veins was found in 2 cases. Porto-systemic varices were detected in 10 patients. Liver parenchymal abnormalities displayed by 3D CE MRA were enlargement of the caudate lobe (7 cases), heterogenous enhancement (18 cases) and complicated tumors (13 cases). Compared with the inferior vena cavography performed in 10 cases, the accuracy of 3D CE MRA was 100 % in the diagnosis of IVC obstruction.CONCLUSION: 3D CE MRA can display the major features of BCS and provide an accurate diagnosis.     

Obliterative hepatocavopathy-inferior vena cava thrombosis at its hepatic portion

Budd-Chiari syndrome was formerly described as hepatic vein thrombosis within the liver, but it now includes inferior vena cava (IVC) thrombosis and other conditions that cause hepatic vein outflow obstruction. The author consider that primary hepatic vein thrombosis and primary IVC thrombosis represent two different clinical disorders.     

3种类型布-加综合征的临床特点分析

[目的]探讨3种类型布-加综合征的临床特征.[方法]回顾性分析3种类型共57例布-加综合征患者的临床资料,对其临床表现、实验室生化检查结果及影像学结果等进行比较.[结果]57例中男38例,女19例,发病率男女之比为2∶1.3种类型中以下腔静脉型最多26例(45.6％)、肝静脉型14例(24.6％)、混合型17例(29....     

Direct intrahepatic portocaval shunt for treatment of portal thrombosis and Budd-Chiari syndrome

Budd-Chiari syndrome (BCS) refers to hepatic venous outflow obstruction that in severe cases can lead to acute liver failure prompting consideration of revascularization or transplantation. Here, a 22 year old female with angiographically proven BCS secondary to JAK2/V617F positive Polycythemia vera on therapeutic warfarin presented with acute liver failure (ALF). Imaging revealed a new, near complete thrombotic occlusion of the main portal vein with extension into the superior mesenteric vein. An emergent direct intrahepatic portocaval shunt (DIPS) was created and liver function promptly normalized. She has been maintained on rivaroxaban since that time. Serial assessment over 1 year demonstrated continued shunt patency and improved flow in the mesenteric vasculature on ultrasound as well as normal liver function. DIPS is a viable alternative in the treatment of ALF from BCS when standard recanalization is not feasible. Improved blood flow may also improve portal/mesenteric clot burden. While further investigation is needed, new targeted anticoagulants may be viable as a long term anticoagulation strategy.