Budd-Chiari Patients Research Articles

15 Years Survival of Budd-Chiari Patients in a Single Center Study

Abstract Background Budd–Chiari syndrome (BCS) is a heterogeneous group of clinical conditions ranging from acute liver failure to completely asymptomatic patient. It is caused by hepatic venous outflow obstruction at any level from the small hepatic veins (HVs) to the junction of inferior vena cava (IVC) with the right atrium. Aim of the Work: The idea of this work was to report the result so favast clinical experience over 15 years presented by Tropical medicine department Ain Shams University through analysing the patient and disease characteristics, outcome, complications and innovations in therapeutic intervention. Ultimately this should throw lights on the understanding of the pathophysiology causes and clinical consequences of BCS among Egyptian patients. Patients and Methods 199 Egyptian patients with confirmed diagnoses of primary BCS were evaluated regarding their socio-demographic features, etiology, risk factors for BCS, clinical manifestations, management modalities and outcomes. The studied patients were enrolled from patients presented to Budd chiari study group clinic, Tropical Medicine Department ward, Ain Shams University hospitals, from January 2006 till December 2021. Results The commonest complaints in Budd Chiari patients were abdominal enlargement in 79.9% (159 patients) and abdominal pain in 77.9% (155 patients), Ascites was present in 75.4% (150), Hepatomegaly in 75.4% (150), Splenomegaly 61.8% (123). More than Seventy Seven percent (77.9%) of the enrolled patients were Chronic BCS patients, 12.6% were subacute BCS and 9.5% were acute BCS. In this study the leading cause of Budd-Chiari was FVLM 76 patients (38.2%). Several papers concluded that FVLM is the main or leading etiology to BCS but regarding the other papers that showed different results, these differences might possibly be due to the difference in sample size. In the current study the outcomes varied from one etiological factor to the other as in FVLM 78.7% of the BCS FVLM patients were compensated, 14.7% were decompensated and 6.7% died and these results were highly significant. Conclusion It seems that TIPS creation is an effective treatment for eligible BCS patients and has long-term survival benefit with satisfactory shunt patency and patient survival. The outcome of TIPS has improved over the years due to the increase in the operator experience and the availability of PTFE-covered stents.

Prevalence of Antiphospholipid Syndrome in Primary Budd Chiari patients and their intervention outcome

Abstract Background Budd-Chiari syndrome (BCS) is a rare but potentially life threatening hepatic disorder that results from obstruction of the hepatic venous outflow tract. Obstruction can occur at any level from the hepatic venules to the right atrium. According to the etiology, BCS can be classified as primary (due to intrinsic intraluminal thrombosis or webs) or secondary (due to intraluminal invasion by a parasite or malignant tumor or extraluminal compression by an abscess, cyst or solid tumor) the clinical presentation of BCS has a wide spectrum and ranges from asymptomatic cases to fulminant hepatic failure The classic triad of abdominal pain, ascites, and hepatomegaly is nonspecific. Aim of the Work To assess the prevelance of Antiphospholipid syndrome (APS) in primary Budd Chiari syndrome (BCS) patients and their management outcome. Patients and Methods Patients presented to BCS outpatient clinic, Tropical medicine department, Ain Shams University Hospitals were enrolled in the study from 2005 to 2020. Some patients with APS have no evidence of any definable associated disease, while, in other patients, APS occurs in association with SLE or another rheumatic or autoimmune disorder. Traditionally, these have been referred to as primary or secondary APS, respectively. Results Regarding laboratory data, Lupus Anticoagulant, ACL IgG antibodies and ACL IgM antibodies were positive in 60 (83.3%), 49 (68.1%) and 27 (37.5%) patients respectively. For 42 patients (58.3%), intervention was performed. TIPSS was performed on 33 (45.8%) patients, while Angioplasty with Stent was performed on 9 (12.5%) patients. There was no intervention in 30 (41.7%) of the patients. In the studied patients, rate of mortality of the total 72 patients was 48.6%. The mean cause of death was Hepatorenal syndrome (31.4%). Conclusion BCS, although infrequent, is not a rare complication in patients with APS and may be the first clinical manifestation of this syndrome. However, no predisposing factor to BCS has been identified in patients with APS. This supports the need for systematic screening for LA and aCL in all cases of hepatic vein thrombosis. In these patients, TIPS placement may be an effective treatment for portal hypertension, but in any case, long-term high intensity anticoagulation treatment is essential in order to prevent recurrent thrombosis. Lupus anticoagulant is much more common than anticardiolipin antibodies (83.3%, 68.1% respectively) among BCS patients due to antiphospholipid syndrome.

Liver transplantation in Budd–Chiari patients with history of portacaval shunt surgery

The paper presents the experience of successful treating a patient with diuretic-resistant ascites associated with Budd– Chiari syndrome. The authors provide a clinical observation of deceased donor liver transplantation to a patient with Budd–Chiari syndrome type 1, total hepatic vein occlusion, and diuretic-resistant ascites after portacaval shunt surgery. The post-transplantation period is observed as stable, with no signs of liver failure; ascites is reduced. The first experience of surgical treatment of a patient with Budd–Chiari syndrome, as well as the first experience of liver transplantation after portacaval shunting is appeared to be successful.

P9.02: Percutaneous Transhepatic TIPS for Budd-Chiari Syndrome As a Bridge to Liver Transplantation

Purpose: To evaluate the effectiveness of percutaneous transhepatic direct simultaneous puncture of portal vein and inferior vena cava in Budd-Chiari syndrome and the clinical outcomes. Material and Methods: From January 2006 to January 2022, we performed TIPS in 65 consecutive patients (mean age, 33 years) with BCS (8 patients with acute BCS and 57 patients with subacute and chronic BCS). Doppler US was performed first day, one week, one month and then 3 months apart. Portography and pressure measurements were performed every year if no shunt dysfunction was detected before. Mean follow-up of 52 months (range, 6 months -119 months). Results: TIPS procedure was technically successful in all patients. In all patients, bare stents were used. Patients were anticoagulated with warfarin after TIPS procedure. Early thrombosis (in one week) was diagnosed in 13 (20%) patients and TIPS revision was required. One year primary patency was 65.2%. Clinical success was achieved in 60 patients. 5 patients required liver transplantation because of liver function deterioration but only 3 of them had liver transplantation(7months, 1.5 year and 2 year later from TIPS) other 2 patients died while in the waiting list. Conclusion: Percutaneous direct puncture of portal vein and inferior vena cava is safe and effective in patients with Budd-Chiari syndrome. This procedure may provide an effective alternative for the management of Budd-Chiari patients that necessitates TIPS. Close follow-up and second intervention is necessary to get good clinical outcome.

4:21 PMAbstract No. 435 - Survival benefit of Budd-Chiari patients who undergo TIPS as a bridge to transplant: a single center experience

4:21 PMAbstract No. 435 - Survival benefit of Budd-Chiari patients who undergo TIPS as a bridge to transplant: a single center experience

Hepatic caudate vein in Budd-Chiari syndrome: Depiction by using magnetic resonance imaging

To study the visibility of the caudate vein and its diameter on MR imaging in healthy people and in patients with Budd-Chiari syndrome. In this study there were 14 patients with Budd-Chiari syndrome and 54 healthy subjects without hepatic lesion or liver disease, all of whom had upper abdominal enhanced MRI. The visibility of the caudate vein and its diameter on MR images was compared between Budd-Chiari patients and healthy subjects, and among Budd-Chiari patients, the correlation between the visibility of caudate vein and extrahepatic collaterals were compared. Caudate vein was noted in 64% of patients with Budd-Chiari syndrome and in 7% of healthy subjects (P=0.000). The diameter of the caudate vein visualized on MR imaging in Budd-Chiari syndrome was significantly larger than that in healthy group (7.3±3.9 mm vs 2.6±0.6 mm, P=0.037). Among Budd-Chiari patients, both caudate vein and extrahepatic collateral veins were noted in 9 patients, only extrahepatic collateral veins were noted in 4 patients and neither caudate vein nor extrahepatic collateral veins were noted in 1 patient. No correlation was found between the visibility of caudate vein and that of extrahepatic collateral vein in patients with Budd-Chiari (P=0.375). Gadolinium enhanced dynamic MR imaging can visualize hepatic caudate vein frequently. The visibility and dilation of hepatic caudate veins on MR imaging in Budd-Chiari syndrome were more frequent than in control subjects. MR depiction of a caudate vein may help differentiate Budd-Chiari from cirrhosis.

Abstract No. 204: Percutaneous Sonographic Guided Direct Simultaneous Puncture of the Portal Vein and Inferior Vena Cava for TIPS in Budd-Chiari Patients

Abstract No. 204: Percutaneous Sonographic Guided Direct Simultaneous Puncture of the Portal Vein and Inferior Vena Cava for TIPS in Budd-Chiari Patients

Frequency of JAK2 Exon 12 Mutations in JAK2 Exon 14 V617F-Negative Patients: High Frequency in ET Patients.

The identification of the G to T point (V617F) mutation in exon 14 of the JH2 domain of the Janus kinase 2 gene (JAK2) has recently simplified the diagnosis of many patients affected with a variety of myeloproliferative disorders (MPDs). More recently JAK2 exon 12 mutations have been described in these disorders; although the role of these mutations is as yet unclear. There have been only two investigations which have identified the presence of exon 12 mutations in clinical diagnosis among polycythaemia vera (PV) (Scott et al NEJM 2007, Pardanani et al Leukaemia 2007). A total of 725 patients diagnosed clinically with a variety of MPDs were investigated for both the frequently occurring exon 14 V617F and for the rarer exon 12 mutations.Methods: Peripheral blood samples from all patients were screened for the V617F mutation by gene-scaning on an ABI 3130 fragment analyser. 478 of these patients were further screened for the exon 12 mutant allele by denaturing high performance liquid chromatography (Transgenomic WAVE®) and direct sequencing.Results: Of 725 patients screened for the V617F mutation 204 (28%) were found to be heterozygous (HET) and 30 (4%) homozygously (HM) mutated while 491 (68%) were wildtype (WT). 478 were further tested for the exon 12 mutation and eight were found to carry mutations. Two of the patients had previously described mutations while three novel mutations were described in the remaining six patients. Only one of these was in the PV group while the majority of exon 12 mutations clustered in the essential thrombocythemia (ET) (4/8) and Budd Chiari patients (2/8). All exon 12 mutations were identified at very low level.Conclusions: Combined rapid gene-scanning and WAVE analysis allows for the characterisation of a large cohort of patients diagnosed clinically to have a variety of MPDs. This helps with the correct diagnosis of a frequently difficult group of patients. Detection of exon 12 mutations should be used in patients who are WT for V617F, including patients in the ET and the Budd Chiari syndrome groups. Both V617F and exon 12 mutation analysis tests should be included in primary screening in the routine management of MPD patients.DiagnosisTotalWT for V617F (mutant exon 12)HET for V617F (mutant exon 12)HM for V617F (mutant exon 12)Budd Chiari/PVT6042 (2)16 (0)2 (0)Polycythemia Vera (PV)187112 (1)63 (0)12 (0)Reactive PV2828 (0)0 (none tested)0 (none tested)ET198123 (4)71 (none tested)4 (none tested)MPD12387 (1)29 (none tested)7 (none tested)Inspecified diagnosis12999 (none tested)25 (none tested)5 (none tested)TOTAL725491 (8)204 (0)30 (0)Results of Exon 14 V617F and exon 12 mutation screening

O41 What is the best treatment strategy for Budd-Chiari patients with JAK2 mutation?

O41 What is the best treatment strategy for Budd-Chiari patients with JAK2 mutation?

Flow cytometric analysis of autonomous growth of erythroid precursors in liquid culture detects occult polycythemia vera in the Budd-Chiari syndrome

Background/Aims: Hepatic vein thrombosis (Budd-Chiari syndrome) is associated with various hypercoagulable states, such as polycythemia vera (PV), presence of the lupus anticoagulant, paroxysmal nocturnal hemoglobinuria (PNH) and deficiencies of antithrombin III, protein C and protein S. In recent years, it has become evident that patients with the Budd-Chiari syndrome may have more than one risk factor that may cause a state of hypercoagulability. The aim of the current study was to assess the prevalence of occult PV in patients with Budd-Chiari syndrome using a novel method for the detection of spontaneous erythroid growth. Methods: Twenty-two patients with Budd-Chiari syndrome were evaluated. As controls, we studied normal donors and four patients with liver cirrhosis and five patients with right-side heart failure, two conditions that in part mimic Budd-Chiari syndrome. The presence of PV was determined by flow cytometric analysis of autonomous growth of erythroid precursors. Patients were considered as having occult PV if they had spontaneous erythroid cell growth in the absence of erythropoietin and with no features of overt PV. Results: Cells from ten patients with Budd-Chiari syndrome demonstrated spontaneous erythroid cell growth; eight patients (32%) were found to have occult PV and two patients had overt PV. None of the controls had spontaneous erythroid growth. Of the eight Budd-Chiari patients with occult PV, six had one or more additional recognized hypercoagulable states. Seven patients (32%) had protein C deficiency, six patients (27%) had activated protein C resistance, five (23%) had anti-cardiolipin antibodies, five (23%) had antithrombin III deficiency, and four patients (18%) had protein S deficiency. Three patients (14%) were homozygous to methyltetra hydrofolate reductase and ten (45.5%) were heterozygous. One patient had PNH. Overall, in 12 patients there were two or more combined risk factors. Conclusions: Using a flow cytometric analysis of autonomous growth of erythroid precursors we found a clear correlation between Budd-Chiari syndrome and occult PV.

Effect of Portosystemic Shunting on PGI2 and Glucagon Levels in Humans

This study determined if the proposed mediators of splanchnic blood flow, prostacyclin and glucagon, were elevated in patients with portal hypertension undergoing portal systemic shunts. Chronic portal hypertension results in increased portal venous pressure and increased splanchnic blood flow. Animal studies have suggested prostacyclin or glucagon, potent vasodilators, as potential mediators of this increased flow. Correlative clinical studies have been difficult to perform due to the wide variation in degree of portal-systemic shunting and the frequent association of parenchymal liver disease in patients with cirrhosis. The authors measured portal and systemic hemodynamics in PGI2 and glucagon levels in patients with portal hypertension due to cirrhosis (partial portal systemic shunt) and Budd-Chiari syndrome (complete portal systemic shunt) undergoing portal systemic shunts and in porto normotensive patients undergoing exploratory laparotomies. PGI2 levels in portal hypertension were significantly increased over normal, and prostacyclin in Budd-Chiari patients were increased significantly over patients with cirrhosis. Both PGI2 and portal venous pressure decreased significantly after portal systemic shunting, and prostacyclin levels correlated directly with portal venous pressure (R = 0.37, p < 0.05). This is the first evidence in humans supporting the hypothesis that PGI2 is elevated in portal hypertension and is related to both the degree of portal venous obstruction and portal pressure. PGI2 probably has a role in the abnormal splanchnic hemodynamics of human portal hypertension.