Hepatic Vein Occlusion Research Articles

Abstract No. 530 - Hepatic vein imaging findings associated with elevated lung shunt fraction in patients being considered for yttrium-90 radioembolization

No. 530 Hepatic vein imaging findings associated with elevated lung shunt fraction in patients being considered for yttrium-90 radioembolization O.G. Olorunsola, N. Fidelman, S. Behr, M. Kohi, K. Kolli, A.G. Taylor, R. Tong, J. LaBerge, R.K. Kerlan, Jr.; Radiology, University of California, San Francisco, San Francisco, CA Purpose: To identify hepatic vein (HV) morphologic and flow abnormalities associated with elevated lung shunt fraction (LSF) determined by Technetium-99m (Tc) macroaggregated albumin (MAA) imaging in patients being considered for yttrium-90 (Y) radioembolization. Materials and Methods: This single-center retrospective study included 152 consecutive patients who underwent mapping arteriography with Tc-MAA injection from 2009 to 2014 (50 with hepatocellular carcinoma [HCC], 102 with other tumors; 103 men, 49 women; median age 62). Each preprocedure imaging examination (CT or MRI) was assessed for HV tumor thrombus or HV occlusion from external compression by tumor. In addition, two readers separately evaluated the mapping arteriograms and the arterial phase from multiphase CTs (117 patients) for evidence of early HV opacification (relative to unaffected HVs), with consensus determined by a third reader. These factors were correlated with LSF categories (low [o10%], intermediate [10-20%], and high [420%]) determined by Tc-MAA imaging using uniand multivariate analysis. Results: Median LSF was 6.7% (range 0-71%; 74.3% low, 18.4% intermediate, 7.2% high). There was high interobserver agreement for determining early HV opacification (97%, kappa1⁄40.847). Early HV opacification at CT and angiography each were associated with higher LSF category (po.001 for both modalities). This finding at CT was 78% sensitive and 93% specific for predicting high (420%) LSF (64% sensitive and 95% specific at angiography). Of five cases of HV tumor thrombus (3 HCC, 2 other tumors), all had high (420%) LSF (po.001). HV occlusion due to external tumor compression was also associated with higher LSF category (p1⁄4.04). Multivariate analysis confirmed that early HV opacification at CT and either HV tumor thrombus or occlusion were associated with higher LSF category. Conclusion: Early HV opacification and HV tumor thrombus or occlusion on pre-procedure cross-sectional imaging are associated with elevated LSF, which may contraindicate or limit the dose delivered in Y radioembolization. This information could be helpful during patient counseling for anticipating the most appropriate mode of liver-directed therapy.

Intrahepatic Blood Flow Redistribution After Temporary Occlusion of the Middle Hepatic Vein During Right Lobe Liver Donation: Report of a Case

IntroductionOne of the critical factors that influence graft function after live donor liver transplantation is the presence or absence of global or sectorial liver congestion. Many authors advocate for routine middle hepatic vein (MHV) reconstruction because it is often difficult to determine when the MHV or one of its major branches have functional significance. Predictive tests to assess hemodynamic and functional significance of the MHV and its tributaries are still under study. Case ReportWe have described a novel intraoperative manipulation and Doppler ultrasonographic evaluation that led to the decision to include the MHV with the right lobe graft.

Laser speckle contrast imaging for intraoperative assessment of liver microcirculation: a clinical pilot study

BackgroundLiver microcirculation can be affected by a wide variety of causes relevant to liver transplantation and resectional surgery. Intraoperative assessment of the microcirculation could possibly predict postoperative outcome. The present pilot study introduces laser speckle contrast imaging (LSCI) as a new clinical method for assessing liver microcirculation.MethodsLSCI measurements of liver microcirculation were performed on ten patients undergoing liver resection. Measurements were made during apnea with and without liver blood inflow occlusion. Hepatic blood flow was assessed by subtracting zero inflow signal from the total signal. Zero inflow signal was obtained after hepatic artery and portal vein occlusion. Perfusion was expressed in laser speckle perfusion units, and intraindividual and interindividual variability in liver perfusion was investigated using the coefficient of variability.ResultsHepatic microcirculation measurements were successfully made in all patients resulting in analyzable speckle contrast images. Mean hepatic blood flow was 410±36 laser speckle perfusion units. Zero inflow signal amounted to 40%±4% of the total signal. Intraindividual and interindividual coefficients of variability in liver perfusion were 25% and 28%, respectively.ConclusionUnder the conditions of this pilot study, LSCI allows rapid noncontact measurements of hepatic blood perfusion over wide areas. More studies are needed on methods of handling movement artifacts.

Acute Budd-Chiari Syndrome with Suspicious Behçet’s Disease: Successful Catheter-Directed Thrombolysis after the Failure of Anticoagulation

Budd-Chiari syndrome (BCS) with occlusion of the major hepatic veins is a rare and serious complication of Behçet’s disease. We report the case of a 38-year-old female who presented with abdominal pain recently. Computerized tomography of the abdomen revealed acute thrombus in the supra- to intrahepatic IVC and thrombotic occlusion of the middle hepatic vein, which is a cardinal sign of acute BCS. The patient had suffered from recurrent oral ulcers, multiple arthralgia, and erythema nodosum, but was not diagnosed with Behçet’s disease based on the international diagnostic criteria. We also tested for other autoimmune diseases, hereditary thrombophilia, antiphopholipid syndrome, myeloproliferative neoplasm and paroxysmal nocturnal hematuria, the results of which were all negative. Therefore, Behçet’s disease was suspected clinically and was also thought to be the cause of BCS. She was treated with heparin for a week, but edema of lower extremities and the abdominal wall were aggravated. We performed catheter- directed thrombolysis and thrombectomy followed by insertion of a stent. Abdominal distension and edema of the legs were improved immediately after the procedure. The patient has exhibited no signs of recurred BCS during 3 months of follow-up with warfarin treatment. Keywords: Acute Budd-Chiari syndrome, Behçet’s disease, Catheter-directed, Thrombolysis

Prevalence of thrombotic risk factors in Chinese Budd–Chiari syndrome patients

Thrombotic risk factors, such as myeloproliferative neoplasms, factor V Leiden mutation, prothrombin G20210A mutation, paroxysmal nocturnal hemoglobinuria, etc., are common in western patients with Budd–Chiari syndrome (BCS) [1,2]. However, recent observational studies have shown that these risk factors are rarely observed in Chinese BCS patients [3–5]. More recently, this discrepancy has been further confirmed by other studies [6,7]. However, considering that the current evidence is limited, a prospective study has been carried out to further validate these previous findings. Between 1 January 2012 and 30 June 2012, a total of 26 consecutive patients who were diagnosed with BCS and first admitted to our department were eligible for this study. However, one patient with concomitant hepatocellular carcinoma was excluded. Thus, 25 patients were finally included. According to the location of occlusion, BCS was classified as pure hepatic vein occlusion (n = 4), pure inferior vena cava occlusion (n = 0), and combined occlusion of hepatic vein and inferior vena cava (n = 21). Demographic data (male: 14; female: 11; mean±SD of age: 35.7±13.2) and laboratory tests are shown in Supplementary Table 1. The results of thrombotic risk factors are presented in Table 1. The JAK2 V617F mutation, the JAK2 exon 12 mutation, and the MPL W515 L/K mutation were negative in all patients. Neither factor II G20210A mutation nor the factor V Leiden mutation was found in any patient. One patient was diagnosed with paroxysmal nocturnal hemoglobinuria because of both CD55 and CD59 deficiencies on granulocytes. Serum erythropoietin level below the reference range was not found in any patients. The heterozygous and the homozygous MTHFR mutation were found in 14 and four patients, respectively. Anticardiolipin IgG antibodies were negative in all patients.

Strategy and Long-term Outcomes of Endovascular Treatment for Budd–Chiari Syndrome Complicated by Inferior Vena Caval Thrombosis

The aim of this study was to evaluate the strategy and long-term outcomes of endovascular treatment of Budd-Chiari syndrome (BCS) complicated by inferior vena cava (IVC) thrombosis. The treatment strategy and outcomes of BCS complicated by IVC thrombosis were retrospectively evaluated in a single-center study. The treatment was aimed at the IVC thrombus, not hepatic vein occlusion. All 133 patients with BCS complicated by IVC thrombosis from February 2003 to March 2013 underwent endovascular treatment. For the fresh thrombus group (n=75) recanalization was performed after transcatheter thrombolysis with urokinase. For the mixed thrombus group (n=19) a small balloon pre-dilation of the IVC was performed first, followed by transcatheter thrombolysis using urokinase and a large balloon dilation of the IVC. For the old thrombus group (n=39) a large balloon dilation or/and stent placement was performed directly. Pre- and post-treatment follow-ups were recorded. The endovascular treatment was successful in 131 out of 133 patients (98.5%). Thirty seven patients had synchronous hepatic vein occlusion. The incidence of serious complications was 4.5% (6/133). Symptomatic pulmonary embolism occurred in three cases, cerebral hemorrhage in two, and cardiac tamponade in one. The cumulative 1-, 5-, and 10-year primary patency rate was 96.3%, 84.0%, and 64.6%, respectively. The cumulative 1-, 5-, and 10-year secondary patency rate was 99.0%, 96.1% and 91.3%, respectively. Segmental occlusion of the IVC and duration of anticoagulant therapy less than 6 months were independent risk factors for reocclusion. For patients with BCS complicated by IVC thrombosis, an individualized treatment strategy based on the property of the thrombus can result in excellent long-term patency.

Initial Imaging Analysis of Budd-Chiari Syndrome in Henan Province of China: Most Cases Have Combined Inferior Vena Cava and Hepatic Veins Involvement

AimTo evaluate the type of venous involvement in Chinese Budd-Chiari syndrome (BCS) patients and the relative diagnostic accuracy of the different imaging modalities.MethodsUsing digital subtraction angiography (DSA) as a reference standard, color Doppler ultrasound (CDUS), computed tomography angiography (CTA), and magnetic resonance angiography (MRA) were performed on 338 patients with BCS. We analyzed the course of the main and any accessory hepatic veins (HVs) and the inferior vena cava (IVC) to assess the etiology of obstructed segments and diagnostic accuracy of CDUS, CTA and MRA.ResultsAmong the 338 cases, there were 8 cases (2.4%) of isolated IVC membranous obstruction, 45 cases (13.3%) of isolated HV occlusion, and 285 cases (84.3%) with both IVC membranous obstruction and HV occlusion. Comparing with DSA, CDUS, CTA had a diagnostic accuracy of 89.3% and 80.2% in detecting BCS, and 83.4% of cases correctly correlated by MRA.ConclusionIn Henan Province, most patients with BCS have complex lesions combining IVC and HV involvement. The combination of CDUS and CTA or MRI is useful for diagnosis of BCS and guiding therapy.

Hepatic Sinusoidal Obstruction Syndrome Caused by Herbal Medicine: CT and MRI Features

ObjectiveTo describe the CT and MRI features of hepatic sinusoidal obstruction syndrome (HSOS) caused by herbal medicine Gynura segetum.Materials and MethodsThe CT and MRI features of 16 consecutive Gynura segetum induced HSOS cases (12 men, 4 women) were analyzed. Eight patients had CT; three patients had MRI, and the remaining five patients had both CT and MRI examinations. Based on their clinical presentations and outcomes, the patients were classified into three categories: mild, moderate, and severe. The severity of the disease was also evaluated radiologically based on the abnormal hepatic patchy enhancement in post-contrast CT or MRI images.ResultsAscites, patchy liver enhancement, and main right hepatic vein narrowing or occlusion were present in all 16 cases. Hepatomegaly and gallbladder wall thickening were present in 14 cases (87.5%, 14/16). Periportal high intensity on T2-weighted images was present in 6 cases (75%, 6/8). Normal liver parenchymal enhancement surrounding the main hepatic vein forming a clover-like sign was observed in 4 cases (25%, 4/16). The extent of patchy liver enhancement was statistically associated with clinical severity classification (kappa = 0.565).ConclusionAscites, patchy liver enhancement, and the main hepatic veins narrowing were the most frequent signs of herbal medicine induced HSOS. The grade of abnormal patchy liver enhancement was associated with the clinical severity.

Effects of percutaneous transhepatic interventional treatment for symptomatic Budd-Chiari syndrome secondary to hepatic venous obstruction.

Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder characterized by hepatic venous outflow obstruction. The management of BCS includes anticoagulation and thrombolysis, percutaneous transhepatic stent angioplasty (PTSA), and transjugular intrahepatic portosystemic shunt (TIPS), but the effect of these approaches varies greatly. The aim of our study was to retrospectively evaluate the medium-term effects of PTSA and TIPS of BCS secondary to hepatic venous outflow obstruction and to determine the critical factors affecting the efficacy. From June 2007 to June 2012, 18 patients (15 males and 3 females; mean age, 36± 9 years) with BCS (obstruction of the hepatic veins) treated by PTSA (n= 15) and TIPS (n= 3) were studied retrospectively. Clinical records were analyzed with respect to underlying disease, therapeutic interventions, complications, quality of life, and overall outcome. Percutaneous transhepatic interventional treatment was technically successful in all patients. In PTSA group, the primary and secondary stent patency rates were 80% and 86.7%, respectively. In the TIPS group, ascites resolved completely, and liver congestion and function were relieved greatly in all three patients. Hemodynamic features and clinical symptoms inpatients with successful treatment improved significantly. Physical aspects evaluated by SF-36 were improved greatly at the end of follow-up. For segmental stenosis or occlusion of hepatic vein caused by thrombosis or membranous webs, PTSA should be recommended as the first choice. TIPS should be applied for diffuse stenosis or occlusion in all the hepatic veins and branches. Standard anticoagulation may promote stent patency. Quality of life after interventional treatment was improved partially, and the mental aspects need to be further investigated.

Hirmi Valley liver disease: A disease associated with exposure to pyrrolizidine alkaloids and DDT

Hirmi Valley liver disease was first reported in 2001 in Tigray, Ethiopia. 591 cases, including 228 deaths, were reported up to December 2009. The pyrrolizidine alkaloid acetyllycopsamine was detected in stored grain and residents reported adding the pesticide DDT (dichlorodiphenyldichloroethylene) directly to their food stores. We aimed to characterise the clinical features of the disease, and explore the role of these chemicals in its aetiology. 32 cases were examined and full clinical histories taken. Nine cases underwent liver biopsy in hospitals. Serum and urine samples were collected from cases and controls. Urine was analysed for acetyllycopsamine by UPLC-MS. Total DDT in serum was measured by ELISA. Hepatotoxicity of DDT and acetyllycopsamine alone or in combination was explored in C57BL/6J mice. Clinical presentation included epigastric pain, abdominal swelling, bloody diarrhoea, hepatomegaly, splenomegaly, and ascites. Histology revealed acute injury characterised by centrilobular necrosis or chronic injury with bile ductular reaction, cytomegaly and fibrosis but no hepatic vein occlusion. Acetyllycopsamine was detected in urine samples taken in the affected area with significantly greater concentrations in 45 cases than in 43 controls (p=0.02). High levels of DDT (>125 ppb) were detected in 78% of serum samples. In mice, DDT (3 × 75 mg/kg) significantly increased the hepatotoxicity (plasma ALT, p=0.0065) of acetyllycopsamine (750 mg/kg), and in combination induced liver pathology similar to Hirmi Valley liver disease including centrilobular necrosis and cytomegaly. This novel form of disease appears to be caused by co-exposure to acetyllycopsamine and DDT.

A Case of Budd-Chiari Syndrome Associated with Alveolar Echinococcosis

Although alveolar echinococcosis (AE) can cause a serious disease with high mortality and morbidity similar to malign neoplasms. A 62-year-old woman admitted to a hospital located in Sivas, Turkey, with the complaints of fatigue and right upper abdominal pain. On contrast abdominal CT, a 54×70×45 mm sized cystic lesion was detected in the left lobe of the liver that was seen to extend to the posterior mediastinum and invade the diaphragm, esophagus, and pericardium. The cystic lesion was seen to be occluding the inferior vena cava and left hepatic vein at the level where the hepatic veins poured into the inferior vena cava. Bilateral pleural effusion was also detected. We discussed this secondary Budd-Chiari Syndrome (BCS) case, resulting from the AE occlusion of the left hepatic vein and inferior vena cava, in light of the information in literature.

Thrombotic Storm in a Teenager With Previously Undiagnosed Ulcerative Colitis

Venous thrombosis can complicate inflammatory bowel diseases, both in adult and pediatric patients, and a few adult cases of thrombotic storm, ie, thrombosis at multiple sites occurring over a period of a few days to a few weeks, have been described. However, venous thrombosis as the first manifestation of an inflammatory bowel disease is extremely rare. We report the case of a 14-year-old girl presenting with ascites and marked hypertransaminasemia resulting from hepatic vein occlusion (Budd-Chiari syndrome). Despite anticoagulant therapy, in the following days she developed criteria suggestive of thrombotic storm to include cerebral vein, right atrial thrombosis, and bilateral pulmonary embolism. Thrombolytic treatment with recombinant-tissue plasminogen activator was started, with resolution of all venous thromboses and without bleeding complications. Additional examinations revealed a severely active ulcerative pancolitis, which did not respond to medical treatment and required surgery. No thrombophilia abnormality nor other risk factors for thrombosis were detected. We conclude that an underlying inflammatory state, such as ulcerative colitis, should be suspected in pediatric patients with venous thrombosis storm.

Infantile Budd-Chiari and response to balloon dilatation

Budd-Chiari Syndrome (BCS) is a rare hepatic disease caused by occlusion of the hepatic venous outflow. Percutaneous hepatic vein angioplasty (PHVA) may also achieve symptomatic relief in patients with BCS caused by short length hepatic vein stenosis or occlusion. However, effectiveness of PHVA in infants with BCS is rarely reported. We report two children with infantile Budd-Chiari who responded to balloon dilatation.

Laser speckle contrast imaging for assessment of liver microcirculation

ObjectiveLaser speckle contrast imaging (LSCI) is a novel technique for microcirculation imaging not previously used in the liver. The aim of the present experimental study was to evaluate the use of LSCI for assessing liver microcirculation. Materials and methodsIn six male Wistar rats, the median liver lobe was exposed through a midline laparotomy. Liver blood perfusion was measured simultaneously with LSCI and sidestream dark-field (SDF) imaging at baseline and during sequential temporary occlusions of the portal vein, hepatic artery, and total blood inflow occlusion. Both the inter-individual variability associated with perfusion sampling area and comparisons in perfusion measurements between both imaging techniques were investigated and validated for the application of LSCI in the liver. ResultsOcclusion of the hepatic artery, portal vein, and total inflow occlusion resulted in a significant decrease in LSCI signal to 74.7±6.4%, 15.0±2.3%, and 10.4±0.5% respectively (p<0.005 vs. baseline). The LSCI perfusion units correlated with sinusoidal blood flow velocity as measured with SDF imaging (Pearson's r=0.94, p<0.001). In a 10mm diameter region of interest, as measured with LSCI, baseline inter-individual variability measured by the coefficient of variability was 13%. ConclusionAlterations in LSCI signal during sequential inflow occlusions were in accordance with previously published results on hepatic hemodynamics in the rat and correlated well with our SDF imaging-derived sinusoidal blood flow velocity measurements. We found that LSCI was able to produce reproducible real-time blood perfusion measurements of hepatic microcirculation. Compared to established techniques for liver blood perfusion measurements LSCI holds the advantages of non-contact measurements over large surfaces with a high speed of data acquisition.

Hepatic Venous Occlusion During Cardiopulmonary Bypass in Patients With Heterotaxy Syndrome: A Safe but Underutilized Option

Direct intracardiac drainage of separate right and left hepatic veins remote from each other (independent hepatic veins) in heterotaxy patients complicates procedures requiring cardiopulmonary bypass (CPB). Temporary occlusion of such independent hepatic veins during CPB is an alternative to cannulation but is rarely used because of concerns about acute congestive hepatopathy. Consequently, temporary single hepatic venous occlusion has not been well described as a safe and simple alternative to hepatic venous cannulation during CPB. We report 2 patients with the polysplenia variant of heterotaxy in whom independent hepatic veins were safely occluded for 55 and 86 minutes, respectively, in the course of intracardiac repair. Temporary hepatic venous occlusion simplified the CPB technique and minimized clutter of the operative field. The intrahepatic hemodynamics during CPB using temporary hepatic venous occlusion is illustrated.

A prospective randomized controlled trial to compare two methods of selective hepatic vascular exclusion in partial hepatectomy

Background and aimSelective hepatic vascular exclusion (SHVE) has not been widely used because of difficulty in extrahepatic isolation of hepatic veins. This study aims to compare the results of SHVE using tourniquets or Satinsky clamps on major hepatic veins in partial hepatectomy for liver tumors involving the roots of hepatic veins. MethodsBetween June 2008 and March 2012, a randomized controlled trial was performed on patients undergoing liver resection to compare selective hepatic vascular exclusion using tourniquets or Satinsky clamps in partial hepatectomy. In the tourniquet group, the hepatic veins were completely isolated and occluded with tourniquets. In the Satinsky clamp group, the hepatic veins were dissected on the anterior and side walls only and they were clamped directly by Satinsky clamps. ResultsThe time for dissecting hepatic veins was significantly shorter in the Satinsky clamp group (7.5 ± 6.6 min vs 21.3 ± 7.4 min) than the tourniquet group. In the tourniquet group, 5 hepatic veins could not be completely isolated and encircled. In 4 additional patients the hepatic vein was slightly torn during dissection. These 9 patients received successful occlusion using Satinsky clamps. In the Satinsky group, all occlusion of the hepatic vein was successful. There was a significant difference in the success rate in hepatic vein occlusion using the Satinsky and the tourniquet groups 60/60 vs 51/60, P = 0.0018. ConclusionsBoth techniques of hepatic vein occlusion were safe and efficacious. As the use of Satinsky clamps is safer, easier and took less time, it is recommended.

Hepatic and Abdominal Carbon Dioxide Measurements Detect and Distinguish Hepatic Artery Occlusion and Portal Vein Occlusion in Pigs

Hepatic artery (HA) occlusion and portal vein (PV) occlusion are the most common vascular complications after liver transplantation with an impact on mortality and retransplantation rates. The detection of severe hypoperfusion may be delayed with currently available diagnostic tools. Hypoperfusion and anaerobically produced lactic acid lead to increases in tissue carbon dioxide. We investigated whether the continuous assessment of the intrahepatic and intra-abdominal partial pressure of carbon dioxide (PCO(2) ) could be used to detect and distinguish HA and PV occlusions in real time. In 13 pigs, the HA and the PV were fully occluded (n = 7) or gradually occluded (n = 6). PCO(2) was monitored intrahepatically and between loops of small intestine. The hepatic and intestinal metabolism was assessed with microdialysis and PV as well as hepatic vein blood samples, and the results were compared to clinical parameters for the systemic circulation and blood gas analysis. Total HA occlusion led to significant increases in hepatic PCO(2) and lactate, and this was accompanied by significant decreases in the partial pressure of oxygen and glucose. PV occlusion induced a significant increase in intestinal PCO(2) (but not hepatic PCO(2) ) along with significant increases in intestinal lactate and glycerol. Gradual HA occlusion and PV occlusion caused steady hepatic and intestinal PCO(2) increases, respectively. Systemic clinical parameters such as the blood pressure, heart rate, and cardiac output were affected only by PV occlusion. In conclusion, even gradual HA occlusion affects liver metabolism and can be reliably identified with hepatic PCO(2) measurements. Intestinal PCO(2) increases only during PV occlusion. A combination of hepatic and intestinal PCO(2) measurements can reliably diagnose the affected vessel and depict the severity of the occlusion, and this may emerge as a potential real-time clinical monitoring tool for the postoperative course of liver transplantation and enable early interventions.

Systemic thrombolysis for acute, severe Budd-Chiari syndrome

Budd-Chiari syndrome (BCS) or hepatic venous outflow obstruction, is a rare and complex clinical entity, with diverse aetiology and a wide range of presentations. Its rarity and heterogeneity create significant challenges for evidence-based clinical decision-making, particularly in the even more infrequent setting of acute BCS presentation. Here we present the case of a young woman with acute, severe BCS, where treatment alternatives were limited due to a significant IVC thrombus burden. A clearly defined acute onset allowed the prompt use of systemic thrombolysis, conducted in consultation with a multi-disciplinary team of clinicians, with close monitoring for both treatment response and complications. Her treatment resulted in resolution of hepatic vein occlusion and prevention of the potentially significant clinical sequelae. We review the literature and discuss current opinion in the management of BCS.

Abstract No. 442: Management of thrombotic occluded hepatic veins after liver transplantation

Abstract No. 442: Management of thrombotic occluded hepatic veins after liver transplantation

Budd–Chiari Syndrome Revisited: 38 Years’ Experience with Surgical Portal Decompression

ObjectiveTen years ago, we reported our results with what remains as the largest clinical experience with surgical portal decompression for Budd–Chiari syndrome (BCS) in the West. Since then, our series has expanded to 77 patients, and there has been an explosion of interest in and publications about BCS. The objectives of this study are to assess the validity of our observations and conclusions regarding BCS reported 10 years ago by expansion of our series of patients and observations of outcomes over an additional decade of close follow-up. MethodsSeventy-seven patients with BCS were allocated to three groups: group I, 39 had hepatic vein occlusion alone, treated by side-to-side portacaval shunt (SSPCS); group II, 26 had inferior vena cava occlusion treated by mesoatrial shunt in eight and combined SSPCS and cavoatrial shunt (CAS) in 18; and group III, 12 had decompensated cirrhosis too late for portal decompression who were listed for liver transplantation (LT). An extensive diagnostic workup included angiography with pressure measurements and needle liver biopsy. Follow-up was 100%, lasting 5–38 years. ResultsIn group I, long-term survival is 95% with 36 free of ascites, leading lives of good quality 5–38 years post-SSPCS. In group II, mesoatrial shunt was discontinued after 1990 because of a high failure rate, but combined SSPCS-CAS has resulted in 100% survival for 5–25 years. In group III, six patients (50%) are alive and well for more than 5 years post-LT. Serial liver biopsies following portal decompression have shown long-term reversal of the lesions of BCS. ConclusionsLong-term survival following portal decompression of BCS in the West has not been equaled by any other form of therapy, medical or surgical. It is imperative to perform surgical portal decompression early in the course of BCS in order to avoid irreversible liver damage.