Hepatic Venous Outflow Tract Obstruction Research Articles

Single-center study: evaluation of sonography in Budd-Chiari syndrome.

Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction. The study aimed to evaluate the diagnostic utility of ultrasound in confirming the diagnosis of BCS and to provide an overview of the clinical picture. In this retrospective single-center study, patients with an initial diagnosis of BCS were included. The files were analyzed concerning the ultrasound images and compared to computed tomography (CT) and magnetic resonance imaging (MRI). Main clinical signs of BCS were collected. Data of 25 patients were analyzed. Doppler sonography showed the highest sensitivity (78.9%) with the highest specificity 97.4 (%) in confirming the correct diagnosis of BCS. Main imaging signs were obstruction in the hepatic veins (68.0%, 17/25 thrombotic), collaterals (91.7%, 11/12 intrahepatic), inhomogeneous liver parenchyma (7/21), and a hypertrophied lobus caudatus (18/21) (p < 0.01). All imaging signs could be detected with sonography. Hypertrophied lobus caudatus was seen exclusively in BCS. Furthermore, portal hypertension (9/25), liver cirrhosis (9/25), and ascites (19/25) can be diagnosed as non-specific signs of BCS (p < 0.01).The main clinical findings were elevated γ-GT levels in the laboratory (92.0%, 23/25, p < 0.01) and esophageal varices in endoscopy (12/25 p < 0.01). An association with myeloproliferative neoplasia (MPN) was frequently seen (10/25) (p < 0.01). The present study demonstrates that sonography is an appropriate tool for the diagnosis of BCS and should be used as the first imaging procedure.

Hepatic Venous Outflow Tract Obstruction: Causes, Natural History, and Treatment.

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Budd-Chiari Sendromu ile Prezente Olan Ampulla Vateri Tümörü

Budd-Chiari syndrome is a rare clinical condition that characterized with hepatic venous outflow tract obstruction caused by thrombosis or phlebitis. Etiology includes diseases that disrupt venous return, conditions that can predispose to thrombosis, or causes of vasculitis. Adenocarcinoma of the ampulla of Vater is a rare tumor of gastrointestinal malignancies. It is very rare for tumor of the ampulla of Vater to cause and coexist with Budd-Chiari syndrome in its initial stage. In this article, we presented a case of Budd-Chiari syndrome associated with tumor of the ampulla of Vater which we were hospitalized due to ascites and icter.

Restenosis after recanalization for Budd-Chiari syndrome: Management and long-term results of 60 patients.

BACKGROUNDBudd-Chiari syndrome is defined as hepatic venous outflow tract obstruction. For Asian Budd-Chiari syndrome patients, the major treatment modality is recanalization (percutaneous transluminal angioplasty with or without stent implantation). The cumulative 1-, 5-, and 10-year primary patency rates and survival rates are reported to be excellent or satisfactory, but the long-term outcome of patients with restenosis (the most common complication after recanalization) is unknown.AIMTo explore the treatment strategy for restenosis in patients with Budd-Chiari syndrome after interventional therapy and to evaluate the long-term follow-up results.METHODSThe clinical data and follow-up results of 60 patients with restenosis after interventional therapy from November 1983 to December 2013 were retrospectively analyzed.RESULTSSixty patients with restenosis were retrospectively divided into a percutaneous transluminal angioplasty (PTA) group (40 patients) and a PTA + stent group (20 patients) according to the primary recanalization method. For the patients with restenosis in the PTA group, 13 refused treatment, and 27 received further treatment; among these patients, five had a second restenosis, two had a third restenosis, and one had a fourth restenosis. For the patients with restenosis in the PTA + stent group, nine refused treatment, ten received PTA alone, and the other received PTA + stent implantation. Among the patients who received further treatment, five had a second restenosis, three had a third restenosis, and one had a fourth restenosis. The 1-, 5-, 10-, 20-, and 25-year cumulative survival rates of the 38 patients who received further treatment after restenosis were 100%, 78.3%, 78.3%, 70.5%, and 70.5%, respectively; however, for the 22 patients who refused treatment, the survival rates were 72.7%, 45.9%, 30.6%, 10.2%, and unavailable, respectively (P < 0.001).CONCLUSIONLong-term follow-up after interventional therapy is very important. Active treatment for patients with restenosis can improve prognosis, and minimally invasive treatment strategies for restenosis allows to obtain satisfactory results.

The compounding effects of hepatitis c infection with liver cirrhosis, hepatocellular carcinoma, and budd-chiari syndrome in fatal gastrointestinal haemorrhage

This case study reports a patient with hepatitis C infection with advanced liver cirrhosis, and undiagnosed hepatocellular carcinoma, who presented with acute Budd-Chiari syndrome and fatal gastrointestinal haemorrhage. Budd-Chiari syndrome is defined as hepatic venous outflow tract obstruction, and can present with signs of acute liver failure, including nausea and vomiting, abdominal distension, jaundice, coagulopathy, and abnormal liver function tests, and may progress to multiorgan failure and death. Budd-Chiari syndrome can arise in the setting of malignancy, via direct compression or invasion of vascular structures, with hepatocellular carcinoma most commonly described. The majority of hepatocellular carcinomas arise in the setting of liver cirrhosis, with chronic viral hepatitis (hepatitis B and C) a leading cause. Liver cirrhosis may result in portal hypertension, which promotes the development of oesophageal and gastric varices. Liver disease can also affect the production of clotting factors, which may increase the risk of potential haemorrhage. This case highlights the combined effects of potentially reduced clotting factors, oesophageal varices, and disseminated intravascular coagulation, and how their overlapping aetiologies may increase the risk of developing potentially fatal spontaneous gastrointestinal haemorrhage.

Pediatric Hepatic Venous Outflow Tract Obstruction: Experience from a Transplant Center

We performed a review of case records of children diagnosed with hepatic venous outflow tract obstruction at our center in last 10 years. Out of 11 cases identified, 6 had variable blocks in the hepatic venous system and 4 had combined hepatic venous and inferior vena cava (IVC) block. One child with paroxysmal nocturnal hemoglobinuria (PNH) had isolated IVC involvement. Angioplasty was attempted in 3 patients; among them 2 had successful outcome. Seven children with advanced liver disease underwent transplantation, which was successful in six. With availability of modalities like interventional radiology and transplantation, the overall prognosis of hepatic venous outflow tract obstruction seems to be good when managed in a well-equipped center.

2209 Abdominal Arterial Aneurysms in a Patient With Budd-Chiari Syndrome

INTRODUCTION: Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow tract obstruction. Chronic BCS eventually leads to post-sinusoidal hypertension resulting in the symptoms of the disease. There have been case reports of BCS leading to intra-abdominal bleeding from ectopic varices, however, these are cases of venous bleeding. Here, we report a unique case of a Chinese woman with BCS presenting with bleeding from arterial aneurysms. CASE DESCRIPTION/METHODS: A 61-year-old woman with BCS requiring multiple prior porto-systemic shunting procedures was brought by EMS for coffee ground emesis. At the ED, she was stuporous and developed bloody emesis and hypotension. She was intubated and transferred to the ICU. Exam was remarkable for abdominal distention (AD) with a fluid wave. Labs showed leukocytosis; prolonged coags, elevated troponins, lactate, LDH and decreased fibrinogen and haptoglobin. FAST showed intra-abdominal free fluid. EGD showed grade II varices in the esophagus that were banded. However, the patient continued to deteriorate with increasing AD. CT-AP revealed a cirrhotic liver with occluded left and middle hepatic veins and extravasation of contrast near the greater curvature of the stomach into a LUQ hematoma (Figure 1). IR embolized the left gastric artery and identified aneurysms along a branch of the SMA (Figure 2) in addition to an aneurysm at the celiac artery origin (Figure 3). On the 2nd post-op day, the patient again developed hypotension with AD. Attempted embolization of the SMA branch was unsuccessful. On the 5th post-op day, the family agreed to withdrawal of life support and the patient was extubated and vasopressors were stopped. The patient passed away in a few hours. DISCUSSION: Chronic BCS presents with complications of cirrhosis; i.e., ascites and rupture of esophageal varices, like in our patient. On CTA, both the hepatic veins and part of the IVC were occluded. In fact, 31% of BCS patients have outflow occlusion at both these locations. However, in our patient, CTA revealed aneurysms in the celiac artery and distal portions of an SMA branch along with left gastric artery bleeding. Multiple arterial aneurysms have been associated with disorders such as PAN, Behcet's, Ehler-Danlos etc. By comparison, there have not been many reports of multiple idiopathic arterial aneurysms in adults. Several of these possible causes were unable to be worked up for due to the acute presentation, although our patient’s history was not typical for these diseases.

Celiac disease with Budd–Chiari syndrome: A rare association

Budd–Chiari syndrome is characterized by hepatic venous outflow tract obstruction. We describe an 18-year-old female of known celiac disease presented with progressive abdomen distention and shortness of breath for the last 1 month. Computed tomography of abdomen revealed hepatic vein obstruction. The patient was diagnosed with Budd–Chiari syndrome. Coagulation profile showed an increased homocysteine level. Serum folate level was also decreased. The patient was put on oral anticoagulant with a gluten-free diet. After 4 weeks, the patient showed significant improvement with decreased ascites. The association of Budd–Chiari syndrome with Celiac disease has not yet been fully understood. There have been few reports that described this rare association. Budd–Chiari syndrome should be considered as an important differential in a patient with unexplained ascites and celiac disease.

Budd-Chiari syndrome: imaging review.

Budd-Chiari syndrome (BCS), also known as hepatic venous outflow tract obstruction includes a group of conditions characterized by obstruction to the outflow of blood from the liver secondary to involvement of one or more hepatic veins (HVs), inferior vena cava (IVC) or the right atrium. There are a number of conditions that lead to BCS-ranging from hypercoagulable states to malignancies. In up to 25% patients, no underlying disorder is identified. Diagnosis of BCS is based on a combination of clinical and imaging features. A major part of the literature in BCS has been devoted to interventions; however, a detailed description of various imaging manifestations of BCS is lacking. In this review, we highlight the importance of various imaging modalities in the diagnosis of BCS.

Heparin-Induced Thrombotic Thrombocytopenia in Budd-Chiari Syndrome: Anticoagulation of Choice?

Budd Chiari Syndrome (BCS) is characterized by hepatic venous outflow tract obstruction leading to post sinusoidal portal hypertension. If untreated, BCS is often fatal. Guidelines recommend heparin and its derivatives for anticoagulation (AC) in BCS. A 23 year-old Caucasian female with no medical history presented with a ten day history of abdominal pain and worsening distension. She reported occasional alcohol use and a remote history of oral contraceptive use 2 years back. She denied a personal and family history of liver disease and hypercoagulable disorders. Laboratory tests showed Hgb of 16.7 gm/dl, WBC of 18.9K, platelet of 525K, AST of 733 U/L, ALT of 889 U/L, AP of 162 U/L, total bilirubin of 3.6 mg/dl, and INR of 1.42. Doppler ultrasound (US) of the liver showed occlusion of the hepatic veins, hepato-splenomegaly and ascites. Contrast-enhanced computed tomography (CECT) confirmed these findings and showed patent but dilated (1.4 cm) portal vasculature and collaterals suggestive of portal hypertension. She was started on intravenous heparin and underwent Transjugular Intrahepatic Portosystemic Shunt (TIPS) placement with a final gradient of 5mmHg. Her liver enzymes initially improved, but began to worsen 8 days later. She developed marked thrombocytopenia and abdominal ecchymosis with eschar (Figure 1). A repeat US Doppler showed decreased flow within TIPS (Figure 2) and new portal vein thrombosis. Positive immunoassay for heparin antibodies confirmed Heparin Induced Thrombotic Thrombocytopenia (HITT). Anticoagulation was changed to bivalirudin and she underwent TIPS revision with thrombectomy. Hypercoagulable work up revealed JAK2-V617F mutation suggestive of myeloproliferative processes- Polycythemia Vera (PCV) and Essential Thrombocythemia (ET). The incidence of BCS is 0.2-0.8 per million/year, however the mortality in untreated BCS is 80-90%. Most BCS cases are acquired and up to 84% of cases have thrombotic diathesis, the most common being PCV and/or ET. Aside from TIPS and liver transplantation, initial AC with heparin is the standard of care. The overall prevalence of HITT from all use is low at 0.2-5.2%. However, in a retrospective study, 9/32 (28%) patients with BCS developed HITT. Our patient initially responded to heparin, but later had extension of thrombosis. This raises the question of alternative anticoagulation such as Direct Thrombin inhibitors in this patient population.Figure: Abdominal eschar due to HITT.Figure: Clotted TIPS - pre and post IR revision.Figure: US doppler showing clotted TIPS.

Budd-Chiari syndrome/hepatic venous outflow tract obstruction.

Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction (HVOTO). Recent literature has been analyzed for this narrative review. Primary BCS/HVOTO is a result of thrombosis. The same patient often has multiple risk factors for venous thrombosis and most have at least one. Presentation and etiology may differ between Western and certain Eastern countries. Myeloproliferative neoplasms are present in 40% of patients and are usually associated with the V617F-JAK2 mutation in myeloid cells, in particular peripheral blood granulocytes. Presentation and symptoms vary, thus this diagnosis must be considered in any patient with acute or chronic liver disease. Doppler ultrasound, computed tomography, or magnetic resonance imaging of the hepatic veins and inferior vena cava usually successfully provide noninvasive identification of the obstruction or its consequences in the collaterals of hepatic veins or the inferior vena cava. The reported life expectancy in these patients is 3 years after the first symptoms. The therapeutic strategy includes first, anticoagulation, correction of risk factors, diuretics, and prophylaxis for portal hypertension, then angioplasty for short-length venous stenosis followed by transjugular intrahepatic portosystemic shunt (TIPS) and finally liver transplantation. The progression of treatment is based on the response to therapy at each step. This strategy results in a 5-year survival rate of nearly 85%. The medium-term prognosis depends upon the severity of liver disease, and the long-term outcome can be jeopardized by transformation of underlying conditions and hepatocellular carcinoma. BCS/HVOTO hepatic manifestations of BCS/HVOTO can be controlled in most patients with medical or radiological interventions. Underlying disease has become the major determinant of patient outcome.

Radiological Management of Hepatocellular Carcinoma Associated With Hepatic Venous Outflow Tract Obstruction

Background and Aim: Hepatocellular carcinoma (HCC) associated with hepatic venous outflow tract obstruction (HVOTO) is an unusual entity which has recently gained recognition. Treatment of both entities is essential in prolonging survival. Interventional radiologic techniques are the established therapeutic options for HVOTO and HCC and thus treatment becomes challenging. We illustrate two representative cases treated successfully treated at our centre. Methods: Patients with clinical diagnosis of HVOTO were evaluated by ultrasound Doppler, multiphase CT liver (MPCT)/dynamic MRI. HVOTO was confirmed by the presence of intrahepatic venous obstruction on Doppler and the treatment was based on the site/type of venous obstruction and included inferior vena cava(IVC) angioplasty or hepatic vein angioplasty (both with or without stenting) and trans-jugular intrahepatic port-systemic shunt (TIPS)/direct intrahepatic port-systemic shunt. On MPCT, HCC diagnosis was based on the modified EASL criteria. Staging and treatment options were according to the Barcelona clinic liver cancer (BCLC) staging. Results: Case one was diagnosed with absent all three hepatic veins with BCLC B stage HCC on MPCT and Doppler. TIPS was performed for HVOTO and subsequently TACE was undertaken for HCC. Follow up Doppler confirmed normal flow through the shunt and HCC depicted complete response. Case two was diagnosed with stenosis of all hepatic veins and IVC stenosis at cavo-atrial junction with intrahepatic collaterals. He also had BCLC B stage HCC on MPCT. IVC angioplasty was performed following which there was persistent stenosis on Doppler assessment, IVC stenting was subsequently performed which restored the normal calibre and flow in IVC. TACE was done for HCC and the residual tumor was completed treated by acetic acid ablation. Conclusion: Radiological techniques play a key role in the diagnosis and management of HVOTO-HCC. Both the entities need to be treated and this makes the treatment very challenging. The authors have none to declare.

An unusual cause of refractory ascites with cirrhosis

Reports of chronic constrictive pericarditis presenting with hepatomegaly, cirrhosis and ascites but no cardiac symptoms are rare. A 10 year old child presented with refractory ascites and firm hepatomegaly of 2 years duration which partially responded to diuretics. Doppler study showed dilated hepatic veins and inferior vena cava and ruled out hepatic venous outflow tract obstruction. Chest X ray showed pericardial calcification and 2D Echo was suggestive of chronic constrictive pericarditis. A diagnosis of chronic calcified constrictive pericarditis with cirrhosis and refractory ascites was made. Based on previous series which showed that tuberculosis is the most common cause of constrictive pericarditis, tuberculosis as the cause of constrictive pericarditis was kept. Anti tuberculous treatment as per Revised National Tuberculosis Control Program guidelines was started along with oral steroids and diuretics. Child showed response to the treatment and was later referred to cardiac surgery department for further management (pericardectomy).

Low frequency of V617F mutation in JAK2 gene in Indian patients with hepatic venous outflow obstruction and extrahepatic portal venous obstruction.

Hepatic venous outflow tract obstruction (HVOTO) and extrahepatic portal venous obstruction (EHPVO) are important causes of portal hypertension and related complications in India. Both these conditions result from splanchnic venous thrombosis. In recent years, a V617F somatic mutation in Janus kinase 2 (JAK2) gene which is highly specific for myeloproliferative disorders has been detected in 40 % to 50% and 30 % to 35% of Western patients with HVOTO and EHPVO, respectively. However, data on this mutation in these conditions from Asian countries are limited. We looked for JAK2 V617F mutation in Indian patients with HVOTO (n = 40, median age 31 [range 17-51] years, 21 female) and EHPVO (n = 50, median age 23 [15-70] years, 25 female) by using two separate methods. Both the methods involved polymerase chain reaction using allele-specific primers. Positive results on one or both of these techniques were confirmed using DNA sequencing. None of the 40 patients with HVOTO and only 1 of 50 patients with EHPVO was found to have JAK2 V617F mutation. In the one patient who was found to have this mutation, both the PCR methods and DNA sequencing showed positive results. Hypercoagulability associated with JAK2 V617F mutation and associated chronic myeloproliferative disorders was not a major cause of HVOTO and EHPVO in this population.

Increased prevalence of heparin-induced thrombocytopenia in patients with Budd-Chiari syndrome: a retrospective analysis.

Budd-Chiari syndrome (BCS) is a rare, potentially fatal disease characterized by hepatic venous outflow tract obstruction. Heparin-induced thrombocytopenia (HIT) is an immune-mediated complication of heparin therapy, with mortality approaching 10%. The reported prevalence of HIT in adults is 0.2-5.2%. Expert consensus through case reports is the only existing evidence of HIT in BCS. To our knowledge, this is the first study to formalize this anecdotal evidence. A retrospective analysis was carried out of patients presenting at a tertiary liver centre with acute liver failure because of BCS or BCS as the primary indication for liver transplantation between 2000 and 2013. The prevalence of HIT in the study group was compared with the highest reported prevalence in adult medical patients receiving heparin (5.2%). Mortality, length of stay and liver transplantation rates were also studied. Of 32 BCS patients, 9 (28.1%) developed HIT, significantly higher than the previously reported prevalence of HIT in medical patients (5.2%) (P<0.0001). There was no difference in mortality (P=0.66), length of stay (P=0.58) and liver transplantation rate (P=0.39) between HIT-positive and HIT-negative patients. The prevalence of HIT (28.1%) in our cohort of BCS patients is significantly higher than that in the general population (0.2-5.2%). Although this study was not powered to detect outcome differences, as heparin is the mainstay of acute BCS treatment, this represents a significant risk. We recommend a high index of suspicion for HIT in patients with BCS and thrombocytopenia, an appropriate HIT-testing strategy and consideration of direct thrombin inhibitors.

Thromboelastography (TEG) does not Predict Thrombophilia in Hepatic Venous Outflow Tract Obstruction (HVOTO)

Thromboelastography (TEG) does not Predict Thrombophilia in Hepatic Venous Outflow Tract Obstruction (HVOTO)

Hepatic venous outflow tract obstruction: treatment outcomes and development of a new prognostic score.

Results of endovascular interventions in hepatic venous outflow tract obstruction (HVOTO) have been reported from limited studies. Treatment outcomes and prognostic scores need further validation. To evaluate treatment outcomes and prognostic scores for hepatic venous outflow tract obstruction in an Indian population. Consecutive patients with hepatic venous outflow tract obstruction diagnosed at a tertiary centre were included. Technical success and clinical response after endovascular interventional therapy were documented. Predictors of survival were assessed with Cox-proportional model. A new score was derived from the factors significant on multivariate analysis and compared with Child-Turcotte-Pugh, model for end-stage liver disease (MELD), Rotterdam prognostic index (PI) and Budd-Chiari syndrome-transjugular intrahepatic portosystemic shunt ( BCS-TIPSS) PI. Three hundred and thirty-four patients (56.6% males), median age 24 (3-62) years were included. Hepatic vein was the commonest site of block-isolated hepatic vonous block in 48%, combined hepatic venous-inferior vena cava block in 46%. Endovascular interventional therapy was performed in 233/334 (70%) with 90% technical success. Clinical response was complete in 166 (71.2%), partial in 58 (24.9%) and no response in nine (3.9%). Majority of cases with HV block did not require TIPSS and could be treated with angioplasty (with/without stenting). On Cox-proportional multivariate analysis, Child class C and response to intervention were independent predictors of outcome and used to derive the All India Institute of Medical Sciences (AIIMS) hepatic venous outflow tract obstruction score. The 5-year survival was 92% (95% CI, 81-97%) for score ≤3, 79% (95%CI, 63-88%) for score >3 and ≤4, and 39% (95% CI, 21-57%) for score >4. The performance of AIIMS hepatic venous outflow obstruction score was superior to other prognostic indices. Advanced Child class and no response to intervention are associated with poor outcomes. The All India Institute of Medical Sciences hepatic venous outflow tract obstruction score predicts survival better than other prognostic scores.

Collateral Pathways in Budd Chiari Syndrome- MDCT Depiction

Budd Chiari syndrome (BCS) is a condition arising from hepatic venous outflow tract obstruction (HVOTO) arising at the level of hepatic veins, inferior vena cava, right atrium or a combination of these. It can be classified as primary or secondary depending upon the underlying pathophysiology. Regardless of the cause of HVOTO, there is lack/complete absence of hepatic venous drainage leading to constellation of symptoms pertaining to portal hypertension with radiologically visible intrahepatic and extrahepatic porto-systemic collaterals. The presence of these collateral systems provide imaging diagnostic clue in chronic cases with cirrhosis labelled as chronic liver disease and thereby establishing the primary cause of cirrhosis to be BCS. Current essay aims to provide in depth knowledge of collateral pathways of BCS.

SAT-001 - Hepatic Venous Outflow Tract Obstruction: Treatment Outcomes and Utility of Prognostic Scores

SAT-001 - Hepatic Venous Outflow Tract Obstruction: Treatment Outcomes and Utility of Prognostic Scores

Sarcoidosis Presenting as Acute on Chronic Liver Failure-Report of the First Case

Sarcoidosis is a multi system disease with different clinical presentations. Asymptomatic presentation is the commonest, but others include jaundice with chronic cholestasis, cirrhosis, portal hypertension, hepatic venous outflow tract obstruction and extrahepatic biliary obstruction. Cirrhosis and portal hypertension are the rarest manifestation of hepatic sarcoid and represent less than 1% of all cases. Acute on chronic liver failure as a presentation of sarcoidosis has never been reported before. Here we present a patient of sarcoidosis presenting with acute on chronic liver failure, and in whom, steroid therapy improved liver failure with a good outcome.