Transjugular intrahepatic portosystemic shunt: a case report of rescue management of unrestrainable variceal bleeding in a pregnant woman.

Liver cirrhosis complications in pregnant women are frequent and death rate secondary to variceal bleeding is relevant. Both sclerotherapy and banding ligation seem to be safe procedures in pregnancy; when bleeding is not arrested endoscopically an emergency transjugular intrahepatic portosystemic shunt should be considered, but data regarding pregnant cirrhotic women are scarce. We describe the case of a pregnant woman at 14 weeks of gestation who underwent management of acute variceal bleeding by transjugular intrahepatic portosystemic shunt. Transjugular intrahepatic portosystemic shunt may represent a rescue treatment for failed attempts of band ligation or sclerotherapy.     

Miscellaneous indications for transjugular intrahepatic portosystemic stent-shunt

This review will discuss the use of transjugular intrahepatic portosystemic stent-shunt in a number of relatively uncommon clinical situations. In particular, we will focus our paper on the use of transjugular intrahepatic portosystemic stent-shunt for hepatic hydrothorax, hepatopulmonary syndrome, veno-occlusive disease, portal hypertensive gastropathy and gastric antral vascular ectasia, before surgery and after liver transplantation.     

Transjugular intrahepatic portosystemic shunt as a treatment for protein-losing enteropathy caused by portal hypertension

A case of a 41-year-old man with diarrhea, hypoalbuminemia, and cryptogenic cirrhosis with features of portal hypertension is described. Protein-losing enteropathy was confirmed by analysis of whole-gut lavage fluid, and intestinal inflammation and infection were excluded. Distal duodenal biopsy specimens showed evidence of edematous villi with prominent submucosal vascular and lymphatic vessels. A transjugular intrahepatic portosystemic stent-shunt was inserted, resulting in resolution of both his diarrhea and elevated whole gut lavage fluid protein concentrations. His symptoms recurred and then again improved after shunt thrombosis and parallel shunt placement, respectively. Histological improvement of the villous edema was also noted. This is the first recorded case of protein-losing enteropathy caused by portal hypertension confirmed by successful treatment with transjugular intrahepatic portosystemic stent-shunt. (Gastroenterology 1996 Dec;111(6):1679-82)     

IS protein-losing enteropathy a significant complication of portal hypertension?: Stanley AJ,Gilmour HM,Ghosh S,et al.Transjugular Intrahepatic Portosystemic Shunt as a Treatment for Protein-Losing Enteropathy Caused by Portal Hypertension Gastroenterology 1996;111:1679–82

A 41-year-old man with diarrhea, hypoalbuminemia, and cryptogenic cirrhosis with features of portal hypertension is presented. Protein-losing enteropathy was confirmed by analysis of whole-gut lavage fluid. Intestinal inflammation and infection were excluded. Distal duodenal biopsy specimens showed edematous villi with prominent submucosal blood and lymphatic vessels. A transjugular intrahepatic portosystemic stent-shunt was inserted, resulting in reduction of both his diarrhea and elevated whole gut lavage fluid protein concentrations. His symptoms recurred after shunt thrombosis but improved after parallel shunt placement. Histological improvement of the villous edema was also noted. This is the first recorded case of protein-losing enteropathy caused by portal hypertension confirmed by successful treatment with transjugular intrahepatic portosystemic stent-shunt. If 40% of cirrhotic patients have portal hypertension and protein-losing enteropathy, this lesion is probably the most common complication of cirrhosis.     

Relationship Between Directly Measured Portal Pressure Gradient and Variceal Hemorrhage

Objective: It is commonly believed that variceal hemorrhage in patients with cirrhosis and portal hypertension does not occur below a portal pressure gradient (PPG) of 12 mm Hg. The aim of this study was to assess the relationship between directly measured portal pressure gradient and variceal hemorrhage.
Methods: The procedure of insertion of the transjugular intrahepatic portosystemic stent-shunt (TIPSS) for variceal hemorrhage provides access to the portal vein and allows direct measurement of the portal pressure. Right atrial, inferior vena caval, and portal pressure were recorded, and the PPG was calculated (portal pressure—inferior vena caval pressure) in 48 patients undergoing TIPSS for variceal hemorrhage.
Results: PPG was reduced from a mean of 21.4 (6.4) before TIPSS to 10.6 (3.1) mm Hg after the procedure. Seven patients (14.7%) had a baseline portal pressure gradient of <12 mm Hg.
Conclusion: The results of this study do not support the concept of a discrete bleeding threshold.     

Treatment of refractory stomal variceal haemorrhage with embolisation and sclerosis

Stomal variceal bleeding is a rare but life‐threatening complication of cirrhosis. As it is an uncommon condition, there is little evidence on the optimum treatment. We report a case of parastomal variceal bleeding in a cirrhotic and haemodynamically unstable patient. The bleeding had failed to respond to local therapy and was not amenable to transjugular intrahepatic portosystemic shunting. The varix was successfully treated under radiological guidance embolisation in conjunction with Fibrovein (STD Pharmaceuticals, UK) sclerosis. We propose that Fibrovein sclerosis through angiography should be considered as an initial treatment option in patients with parastomal variceal bleeding who are not candidates for transjugular intrahepatic portosystemic shunting.     

Transjugular intrahepatic portosystemic stent-shunt in the management of gastric and ectopic varices

Uncertainty exists about the ideal therapy for gastric and ectopic varices owing to relatively few controlled studies. Endoscopic therapy with tissue adhesives and thrombin appear promising. Transjugular intrahepatic portosystemic stent-shunt has a role in patients with refractory gastric variceal bleeding in the presence of a patent portal vein. The addition of coil embolization may be particularly useful for ectopic varices, as these can continue to bleed despite successful portal pressure reduction. The high efficacy of transjugular intrahepatic portosystemic stent-shunt has to be balanced against the potential for increased encephalopathy. Balloon occluded retrograde transvenous obliteration is a recent technique for patients with gastro-renal shunts and large gastric varices. Early results are promising, and balloon occluded retrograde transvenous obliteration may be valuable in patients who bleed at lower portal pressures, in the encephalopathic patient, or where the portal vein is not patent. Its use may be limited by availability or lack of technical expertise, and caution is required in patients with large oesophageal varices.     

Efficacy and safety of transjugular intrahepatic portosystemic shunt in the treatment of nongastric extraesophageal variceal bleeding

OBJECTIVES: Although esophageal varices are the most common site of variceal bleeding, extraesophageal varices cause up to 30% of variceal bleeding. Unlike esophageal variceal bleeding, the experience in management of extraesophageal variceal bleeding, especially nongastric extraesophageal variceal bleeding is limited, and there are no established guidelines for treatment of nongastric extraesophageal variceal bleeding. This study aims to provide experience in treatment of nongastric extraesophageal variceal bleeding with transjugular intrahepatic portosystemic shunt in a tertiary medical center. METHODS: We retrospectively reviewed all cases, admitted or transferred to Emory University Hospital, with extraesophageal variceal bleeding who had transjugular intrahepatic portosystemic shunt as the final resolution to control bleeding over a period of 4 years, from January 1999 to January 2003. We also compared the outcomes after transjugular intrahepatic portosystemic shunt for bleeding from gastric varices and nongastric extraesophageal varices. RESULTS: Forty-one patients (33 gastric varices and 8 nongastric extraesophageal varices) with extraesophageal variceal bleeding who had transjugular intrahepatic portosystemic shunt performed were identified in this study period. Bleeding was controlled immediately in 90% (37/41) of those patients. The mortality was 7% (3/41). The rebleeding rate was 10% (4/41). Encephalopathy occurred in 24% (10/41) of the patients. Patients with gastric varices bleeding appeared to have more advanced liver disease than patients with nongastric extraesophageal varices bleeding. The outcomes after transjugular intrahepatic portosystemic shunt for bleeding from gastric varices and nongastric extraesophageal varices were similar. CONCLUSIONS: Transjugular intrahepatic portosystemic shunt is an effective and safe treatment of extraesophageal variceal bleeding, including bleeding from gastric varices and nongastric extraesophageal varices.     

Recurrent thrombotic occlusion of a transjugular intrahepatic portosystemic stent-shunt due to activated protein C resistance

The transjugular intrahepatic portosystemic stent-shunt (TIPS) has successfully been used in the management of refractory variceal bleeding and ascites in patients with portal hypertension. Major drawbacks are the induction of hepatic encephalopathy and shunt dysfunction. We present a 59-year-old woman with alcoholic liver cirrhosis who received a TIPS because of recurrent bleeding from esophageal varices. Stent occlusion occurred 4 mo after placement of the TIPS. Laboratory testing revealed resistance to activated protein C (APC). Combination therapy with low-dose enoxaparin and clopidogrel could not prevent her recurrent stent occlusion. Finally, therapy with high-dose enoxaparin was sufficient to prevent further shunt complications up to now (follow-up period of 1 year). In conclusion, early occlusion of a TIPS warrants testing for thrombophilia. If risk factors are confirmed, anticoagulation should be intensified. There are currently no evidence-based recommendations regarding the best available anticoagulant therapy and surveillance protocol for patients with TIPS.     

Transjugular intrahepatic portosystemic stent-shunt: technical factors and new developments

The introduction of expandable metal stents in the mid 1980s led to the development of transjugular intrahepatic portosystemic stent-shunt (TIPSS) as we know it today. Short-lived detrimental effects on the hyperdynamic circulation in cirrhosis accompany the acute reduction in portal pressure following TIPSS creation. Caution is needed in patients with cardiac dysfunction or pulmonary hypertension. With increasing expertise and careful patient selection, fatal procedural complications are rare and TIPSS can even be safely used as a bridge to liver transplantation. Shunt insufficiency and hepatic encephalopathy are more common following TIPSS. Currently, however, novel approaches to tackling both these limitations exist. These include the combination of uncovered TIPSS with variceal band ligation, and the introduction of polytetrafluoroethylene covered stents. Despite the lack of controlled studies, covered stents are now widely used and have the potential to drastically reduce shunt insufficiency, the need for long-term shunt surveillance and even hepatic encephalopathy.     

Using transjugular intrahepatic portosystemic shunts to control variceal bleeding before liver transplantation.

OBJECTIVE: To determine the safety and efficacy of transjugular intrahepatic portosystemic shunts (TIPS) in controlling bleeding from esophageal varices in patients awaiting liver transplantation. DESIGN: Prospective, uncontrolled trial. SETTING: University medical center with an active liver transplant program. PATIENTS: Thirteen patients referred for liver transplantation with either active variceal hemorrhage or recurrent variceal hemorrhage despite sclerotherapy; four patients had been previously treated with surgical portosystemic shunts. INTERVENTION: An intrahepatic portosystemic shunt created via a transjugular approach to the hepatic veins using expandable, flexible metallic stents. MEASUREMENTS: Portal pressures before and after the creation of the shunt, the direction of portal blood flow at differing diameters of the shunts, procedure-related complications, and outcome in terms of survival, liver transplantation, and recurrent variceal bleeding. MAIN RESULTS: The transjugular intrahepatic portosystemic shunt was placed successfully in 13 patients, and bleeding was controlled acutely in all 13. After the procedure, the mean portal pressure decreased from 34 +/- 8.9 cm H2O to 22.4 +/- 5.4 cm H2O (P less than 0.001). No complications were associated with the procedure; however, two patients died of causes unrelated to the procedure. Seven patients subsequently underwent liver transplantation and are doing well, and three patients are being managed conservatively. Bleeding recurred in one patient 102 days after the procedure secondary to shunt occlusion caused by neointimal proliferation. CONCLUSION: Placement of a transjugular intrahepatic portosystemic shunt is apparently safe and effective therapy for variceal hemorrhage in patients referred for liver transplantation.     

TIPSS for variceal hemorrhage after living related liver transplantation： A dangerous indication

The introduction of transjugular intrahepatic portal-systemic stent-shunt(TIPSS)has been a majorbreakthrough in the treatment of portal hypertension,which has evolved to a large extent,into a routineprocedure.A 21-year-old male patient with progressivegraft fibrosis/cirrhosis requiring TIPSS for varicealhemorrhage in the esophagus due to portal hypertensionwas unresponsive to conventional measures twoyears after living related liver transplantation(LDLT).Subsequently,variceal hemorrhage was controlled,however,liver function decreased dramatically withconsecutive multi organ failure.CT scan revealedsubstantial necrosis in the liver.The patient underwentsuccessful"high urgent"cadaveric liver transplantationand was discharged on postoperative d 20 in a stablecondition.     

Emerging issues in the use of transjugular intrahepatic portosystemic shunt (TIPS) for management of portal hypertension: Time to update the guidelines?

Since its first introduction in the 1980s, transjugular intrahepatic portosystemic shunt has played an increasingly important role in the management and treatment of the complications of portal hypertension. In 2005, the American Association for the Study of Liver Diseases published the Practice Guidelines for the use of transjugular intrahepatic portosystemic shunt in the management of portal hypertension. Since then, technical advances and new interesting data on transjugular intrahepatic portosystemic shunt have been presented in the literature. The present review focusses on the applications of transjugular intrahepatic portosystemic shunt and examines more recent studies on this topic; the current guidelines on the use of transjugular intrahepatic portosystemic shunt are also discussed. From the data presented in the most recent publications, it has become increasingly clear that the recommendations stemming from the current guidelines need to be reviewed and updated in several points. Changes in the American Association for the Study of Liver Diseases Practice Guidelines are needed for both common indications (variceal bleeding and refractory ascites) as well as uncommon ones (i.e., Budd-Chiari syndrome and portal cavernoma). In addition, a relevant technical advance has been the introduction of the polytetrafluoroethylene-covered stents, which greatly improved the patency and clinical efficacy of transjugular intrahepatic portosystemic shunt. Consequently, new studies are required to re-assess the role of transjugular intrahepatic portosystemic shunt performed with new covered stents as compared with other strategies in the management of portal hypertension.     

Transjugular intrahepatic portosystemic shunt (TIPS) for budd-chiari syndrome

Summary The transjugular intrahepatic portosystemic shunt (TIPS) is widely used in the treatment of variceal hemorrhage and portal hypertension associated with cirrhosis. Its potential as a therapy for Budd-Chiari syndrome is less well-known. We report a case of a 15-year-old girl with Budd-Chiari syndrome who was successfully treated with a TIPS, leading to resolution of her ascites and improvement in liver function.     

Successful hemostasis of intractable rectal variceal bleeding using variceal embolization

Portal hypertension causes portosystemic shunting along the gastrointestinal tract,resulting in gastrointestinalvarices.Rectal varices and their bleeding is a rare complication,but it can be fatal without appropriate treatment.However,because of its rarity,no established treatment strategy is yet available.In the setting of intractable rectal variceal bleeding,a transjugular intravenous portosystemic shunt can be a treatment of choice to enable portal decompression and thus achieve hemostasis.However,in the case of recurrent rectal variceal bleeding despite successful transjugular intravenous portosystemic shunt,alternative measures to control bleeding are required.Here,we report on a patient with liver cirrhosis who experienced recurrent rectal variceal bleeding even after successful transjugular intravenous portosystemic shunt and was successfully treated with variceal embolization.     

Transjugular intrahepatic portosystemic shunt versus distal splenorenal shunt--a comparative study

BACKGROUND/AIMS: No general consensus exists regarding the proper surgical management of recurrent variceal bleeding due to hepatic cirrhosis. Transjugular intrahepatic portosystemic shunt and distal splenorenal shunt are increasingly being performed in the management of these patients. The present study was undertaken to compare the efficacy, complications and survival rate of these two procedures. METHODOLOGY: Sixty-seven patients with alcoholic liver cirrhosis of Child-Pugh's class A (n = 22) and class B (n = 45) with recurrent variceal bleeding not controlled by conservative means underwent either transjugular intrahepatic portosystemic shunt placement (n = 35) or a distal splenorenal shunt operation (n = 32). These patients were followed for a mean of 887 +/- 189 days. Both groups were compared with respect to the rates of survival, recurrence of gastrointestinal bleeding, encephalopathy, ascitis, shunt blockade and other relevant biochemical parameters. RESULTS: Patients who underwent a distal splenorenal shunt operation had lower rates of recurrence of gastrointestinal bleeding (6.25% vs. 25.71%), encephalopathy (18.75% vs. 42.86%) shunt blockade (6.25% vs. 68.57%) and lower mean fasting blood ammonia levels (56.70 +/- 7.10 mumol/L vs. 61.70 +/- 5.70 mumol/L). However the rate of ascitis was higher amongst these patients (40.63% vs. 11.43%). There was no significant difference in the midterm survival rates between these groups (81.25% vs. 80.00%). Both procedures were effective in controlling functional renal failure, splenomegaly and features of hypersplenism. CONCLUSIONS: Distal splenorenal shunt operation is a better therapeutic option than transjugular intrahepatic portosystemic shunt placement for control of recurrent variceal bleeding due to hepatic cirrhosis.     

Variceal hemorrhage

Opinion statement Reducing morbidity and mortality from esophageal varices remains a challenge for physicians managing patients with chronic liver disease. For patients who have never bled from varices, prophylactic therapy with nonselective beta-blockers reduces the risk of initial variceal bleeding and bleeding-related death. Thus, patients with newly diagnosed cirrhosis should be considered for endoscopic variceal screening. All patients with Child’s class B and C cirrhosis should be offered endoscopic screening, whereas those with Child’s class A with evidence of portal hypertension (eg, platelet count less than 140,000 per milliliter, portal vein diameter larger than 13 mm, evidence of splenic varices on ultrasound) should be screened. The principal risk factors for variceal bleeding are variceal size, the presence of color changes on the variceal wall (indicative of decreased wall thickness), and degree of liver dysfunction. Patients with moderate or large sized varices and those with varices exhibiting color changes (eg, red wale marks, cherry red spots) should be treated with beta-blockers. Individuals without varices and those with small varices should undergo repeat endoscopy at approximately 2-year intervals. Patients unwilling or unable to take beta-blockers do not need to be screened. For patients with acute variceal bleeding, the combination of pharmacologic therapy plus endoscopic therapy is superior to either therapy alone. Octreotide is the drug most often used as initial therapy in the United States. Terlipressin is the preferred agent; however, it is not available in the United States. Endoscopy is performed as early as possible, and endoscopic injection sclerotherapy or endoscopic variceal band ligation is employed if variceal bleeding is confirmed or suspected. Endoscopic therapy should be repeated until the varices are obliterated completely. The addition of beta-blockers to endoscopic sclerotherapy or ligation may decrease the rate of rebleeding compared with receiving endoscopic treatment alone. Patients with bleeding refractory to combined medical plus endoscopic therapy should be considered for transjugular intrahepatic portosystemic shunts or shunt surgery.     

Surgical portosystemic shunts in the era of TIPS and liver transplantation are still relevant

BackgroundThe surgical portosystemic shunts (PSS) are a time-proven modality for treating portal hypertension. Recently, in the era of liver transplantation and the transjugular intrahepatic portosystemic shunts (TIPS), use of the PSS has declined.ObjectivesThis study was conducted to evaluate changes in practice, referral patterns, and short- and longterm outcomes of the use of the surgical PSS before and after the introduction of the Model for End-stage Liver Disease (MELD).MethodsA retrospective analysis of 47 patients undergoing PSS between 1996 and 2011 in a single university hospital was conducted.ResultsSubgroups of patients with cirrhosis (53%), Budd–Chiari syndrome (13%), portal vein thrombosis (PVT) (26%), and other pathologies (9%) differed significantly with respect to shunt type, Child–Pugh class, MELD score and perioperative mortality. Perioperative mortality at 60 days was 15%. Five-year survival was 68% (median: 70 months); 5-year shunt patency was 97%. Survival was best in patients with PVT and worst in those with Budd–Chiari syndrome compared to other subgroups. Patency was better in the subgroups of patients with cirrhosis and other pathologies compared with the PVT subgroup. Substantial changes in referral patterns coincided with the adoption of the MELD in 2002, with decreases in the incidence of cirrhosis and variceal bleeding, and increases in non-cirrhotics and hypercoagulopathy.ConclusionsAlthough the spectrum of diseases benefiting from surgical PSS has changed, surgical shunts continue to constitute an important addition to the surgical armamentarium. Selected subgroups with variceal bleeding in well-compensated cirrhosis and PVT benefit from the excellent longterm patency offered by the surgical PSS.     

Transjugular intrahepatic portosystemic shunt in the management of Budd Chiari syndrome

Budd Chiari syndrome presents with a wide range of severity and duration of symptoms. Transjugular intrahepatic portosystemic shunt has been used to treat selected Budd Chiari syndrome patients for several years. The technique of transjugular intrahepatic portosystemic shunt may be more challenging than in cirrhosis because of hepatic vein occlusion. Covered transjugular intrahepatic portosystemic shunt stents have reduced the requirement for follow-up interventions. Transjugular intrahepatic portosystemic shunt has been a successful bridge to liver transplant for Budd Chiari syndrome but is the definitive treatment in many cases. Patient selection is important to determine who will benefit from transjugular intrahepatic portosystemic shunt or other treatments such as hepatic vein recanalization or liver transplant.     

Hepatic venous pressure gradient measurement before TIPS for acute variceal bleeding

Hepatic venous pressure gradient (HVPG) is an independent predictor of variceal rebleeding in patients with cirrhosis. After pharmacological and/or endoscopic therapy, the use of a transjugular intrahepatic portosystemic shunt (TIPS) may be necessary in HVPG non-responders, but not in responders. Thus, HVPG measurement may be incorporated into the treatment algorithm for acute variceal bleeding, which further identifies the candidates that should undergo early insertion of TIPS or maintain the traditional pharmacological and/or endoscopic therapy. The potential benefits are to reduce the cost and prevent TIPS-related complications.