TEN-YEAR OUTCOMES OF TIPS FOR BUDD-CHIARI SYNDROME: SYSTEMATIC REVIEW AND META-ANALYSIS.

BACKGROUND: Budd-Chiari syndrome (BCS) results from the obstruction of the hepatic venous flow, usually at the level of the hepatic vein or inferior vena cava. When left untreated, it can progress with several complications, including liver cirrhosis. Transjugular intrahepatic portosystemic shunt (TIPS) appears to be effective in a subgroup of BCS patients. OBJECTIVE: To perform a systematic review and meta-analysis of TIPS effectiveness in BCS treatment, considering the survival rate, reduction in portosystemic pressure, need for liver transplantation, technical failure, and shunt dysfunction for up to 10 years of follow-up. METHODS: We evaluated 17 studies published in PubMed, Science Direct, Web of Science, and SCOPUS databases, which used TIPS as a treatment for BCS, comprising 618 subjects between 18 and 78 years old. We assessed the bias risk by the NOS, NHI, and JBI scales for cohort stu-dies, before-after studies, and case series, respectively. We conducted the meta-analyses by extracting the number of events and the total patients evaluated to perform the proportion meta-analyses using the R software ("meta" package - version 4.9-6). RESULTS: The pooled results (95%CI) showed a 19% (25.9-12.5%) rate of portosystemic pressure reduction, 6% (1-12%) rate for the need for liver transplants despite the use of TIPS, 2% (1-6%) technical failure rate, 30% (18-46%) shunt dysfunction rate, and 88% (81-93%) for the mean frequency of patients alive between 1 and 10 years after the procedure. We stratified survival rate and found an 86% (74-93%) prevalence of living subjects during less than five years, 92% (83-97%) at five years, and a 77% frequency (68-83%) of patients alive ten years after the TIPS placement. CONCLUSION: TIPS is an effective treatment for BCS, providing a high 10-year frequency of living patients and a significant decrease in portosystemic pressure. The need for liver transplants after TIPS and the technical failure rate is low.

Management and treatment of decompensated hepatic fibrosis and severe refractory Schistosoma mansoni ascites with transjugular intrahepatic portosystemic shunt.

This study aimed to report the first case of a patient with hepatosplenic schistosomiasis mansoni, refractory ascites and portal vein thrombosis treated with a transjugular intrahepatic portosystemic shunt (TIPS), at the Instituto de Radiologia, Hospital das Clinicas, Faculdade de Medicina, Universidade de Sao Paulo, Brazil. After the procedure, the patient recovered favorably and progressed with portal pressure reduction and no deterioration of the liver function. Endovascular shunt modification is a conservative medical approach that often helps in reducing symptoms significantly, making it a less invasive and a safer alternative to liver transplantation for the treatment of schistosomiasis with portal hypertension.

Tips for TIPS: A combined percutaneous and transjugular approach for intrahepatic portosystemic shunt placement after liver transplant.

A 39-year-old female, liver transplanted for Autosomic Dominant Polycystic Kidney Disease (ADPKD) developed refractory ascites early after surgery, with frequent need of large-volume paracentesis. This was associated with severe sarcopenia and kidney impairment. Liver biopsy showed a sinusoidal congestion with a significant enlargement of hepatic portal veins. This picture suggested the diagnosis of vascular obstructions. Due to an unfavorable passage through the piggy-back surgical anastomosis and the angle between the hepatic veins and the portal branches, a conventional placement of a transjugular portosystemic shunt (TIPS) was not feasible. An alternative approach was pursued with success, using a combined percutaneous-transjugular approach and achieving a complete recovery of ascites, sarcopenia and renal function.

Case Series Regarding Parastomal Variceal Bleeding: Presentation and Management.

Parastomal variceal bleeding (PVB) is a serious complication occurring in up to 27% of patients with an ostomy and concurrent cirrhosis and portal hypertension. The management of PVB is difficult and there are no clear guidelines on this matter. Transjugular intrahepatic portosystemic shunt (TIPS), sclerotherapy, and /or coil embolization are all therapies that have been shown to successfully manage PVB. We present a case series with five different patients who had a PVB at our institution. The aim of this case series is to report our experience on the management of this infrequently reported but serious condition. We also conducted a systemic literature review focusing on the treatment modalities of 163 patients with parastomal variceal bleeds. In our series, patient 1 had embolization and sclerotherapy without control of bleed and expired on the day of intervention due to hemorrhagic shock. Patient 2 had TIPS in conjunction with embolization and sclerotherapy and had no instance of rebleed 441 days after therapy. Patient 3 did not undergo any intervention due to high risk for morbidity and mortality, the bleed self-resolved and there was no further rebleed, this same patient died of sepsis 73 days later. Patient 4 had embolization and sclerotherapy and had no instance of rebleed 290 days after therapy. Patient 5 had TIPS procedure and was discharged five days post procedure without rebleed, patient has since been lost to follow-up.

Safety and efficacy of angiographic occlusion of duodenal varices as an alternative to TIPS: review of 32 cases.

UNLABELLED: BACKGROUND/RATIONALE OF STUDY: Analyze safety and efficacy of angiographic-occlusion-with-sclerotherapy/embolotherapy-without-transjugular-intrahepatic-portosystemic-shunt (TIPS) for duodenal varices. Although TIPS is considered the best intermediate-to-long term therapy after failed endoscopic therapy for bleeding varices, the options are not well-defined when TIPS is relatively contraindicated, with scant data on alternative therapies due to relative rarity of duodenal varices. Prior cases were identified by computerized literature search, supplemented by one illustrative case. Favorable clinical outcome after angiography defined as no rebleeding during follow-up, without major procedural complications. RESULTS: Thirty-two cases of duodenal varices treated by angiographic-occlusion-with-sclerotherapy/embolotherapy- without-TIPS were analyzed. Patients averaged 59.5 ± 12.2 years old (female = 59%). Patients presented with melena-16, hematemesis & melena-5, large varices-5, growing varices-2, ruptured varices-1, and other- 3. Twenty-nine patients had cirrhosis; etiologies included: alcoholism-11, hepatitis C-11, primary biliary cirrhosis- 3, hepatitis B-2, Budd-Chiari-1, and idiopathic-1. Three patients did not have cirrhosis, including hepatic metastases from rectal cancer-1, Wilson's disease-1, and chronic liver dysfunction-1. Thirty-one patients underwent esophagogastroduodenoscopy before therapeutic angiography, including fifteen undergoing endoscopic variceal therapy. Therapeutic angiographic techniques included balloon-occluded retrograde-transvenous-obliteration (BRTO) with sclerotherapy and/or embolization-21, DBOE (double-balloon-occluded-embolotherapy)-5, and other-6. Twenty-eight patients (87.5%; 95%-confidence interval: 69-100%) had favorable clinical outcomes after therapeutic angiography. Three patients were therapeutic failures: rebleeding at 0, 5, or 10 days after therapy. One major complication (Enterobacter sepsis) and one minor complication occurred. CONCLUSIONS: This work suggests that angiographic-occlusion-with sclerotherapy/ embolotherapy-without-TIPS is relatively effective (~90% hemostasis-rate), and relatively safe (3% major-complication-rate). This therapy may be a useful treatment option for duodenal varices when endoscopic therapy fails and TIPS is relatively contraindicated.

Right atrial pressure may impact early survival of patients undergoing transjugular intrahepatic portosystemic shunt creation.

PURPOSE: To elucidate the impact of right atrial (RA) pressure on early mortality after transjugular intrahepatic portosystemic shunt (TIPS). MATERIAL AND METHODS: In this single institution retrospective study, 125 patients (M:F = 75:50, mean age 55 years) who underwent TIPS with recorded intra-procedural RA pressures between 1999-2012 were studied. Demographic (age, gender), liver disease (Child-Pugh, Model for End Stage Liver Disease or MELD score), and procedure (indication, urgency, Stent type, portosystemic gradient or PSG reduction, baseline and post-TIPS RA pressure) data were identified, and the influence of these parameters on 30- and 90-day mortality was assessed using binary logistic regression. RESULTS: TIPS were created for variceal hemorrhage (n = 55) and ascites (n = 70). Hemodynamic success rate was 99% (124/125) and mean PSG reduction was 13 mmHg. 30- and 90-day mortality rates were 18% (19/106) and 28% (29/106). Baseline and final RA pressure were significantly associated with 30- (12 vs. 15 mmHg, P = 0.021; 18 vs. 21 mmHg, P = 0.035) and 90-day (12 vs. 14 mmHg, P = 0.022; 18 vs. 20 mmHg, P = 0.024) survival on univariate analysis. Predictive usefulness of RA pressure was not confirmed in multivariate analyses. Area under receiver operator characteristic (AUROC) curve analysis revealed good pre- and post-TIPS RA pressure predictive capacity for 30- (0.779, 0.810) and 90-day (0.813, 0.788) mortality among variceal hemorrhage patients at 14.5 and 21.5 mm Hg thresholds. CONCLUSION: Intra-procedural RA pressure may have predictive value for early post-TIPS mortality. Pre-procedure consideration and optimization of patient cardiac status may enhance candidate selection, risk stratification, and clinical outcomes, particularly in variceal hemorrhage patients.

Long-term follow-up after successful transjugular intrahepatic portosystemic shunt placement in a pediatric patient with Budd-Chiari syndrome.

Orthotopic liver transplantation is the standard of care in patients with Budd-Chiari syndrome (BCS), and transjugular intrahepatic portosystemic shunt (TIPS) has become an important adjunct procedure while the patient is waiting for a liver. No long-term follow up of TIPS in BCS patients has been published in children. We report successful 10-year follow-up of a child with BCS and iatrogenic TIPS dysfunction caused by oral contraceptive use.

Massive hematuria due to portal hypertension in a child with bladder augmentation for vesical exstrophy.

We report a case of a 17-year-old patient with severe hematuria from an ileocystoplasty-augmented bladder for vesical exstrophy, with associated cirrhosis secondary to autoimmune hepatitis, diagnosed years later. Varix development and further bleeding from ectopic varices, related to cirrhosis and portal hypertension, are a rare cause of hematuria in patients with surgically augmented bladder volume. This child was successfully treated with a transjugular intrahepatic portosystemic shunt procedure as a bridge to hepatic transplantation.

The use of TIPS in chronic liver disease.

The development of cirrhosis and portal hypertension in the natural history of chronic liver disease is associated with many complications. A transjugular intrahepatic portosystemic stent shunt (TIPS) is a metal prosthesis that has been shown to be very effective in lowering sinusoidal portal pressure, and therefore is effective in the management of complications of cirrhosis, especially those related to portal hypertensive bleeding and sodium and water retention. In patients with acute variceal bleeding not responding to pharmacologic and endoscopic treatments, a reduction of the hepatic venous pressure gradient to < 12 mmHg or by > 20% with TIPS has been shown to be effective in controlling the acute bleed and in preventing rebleeding. For stable patients whose acute variceal bleed is controlled, TIPS is equal to combined beta-blocker and band ligation in the prevention of recurrent variceal bleed. TIPS is also more effective than large volume paracentesis in the control of refractory ascites, and may confer a survival advantage over repeated large volume paracentesis. TIPS has also been used in the management of other complications related to portal hypertension including ectopic varices, hepatic hydrothorax, and hepatorenal syndrome with some success, but experience is still rather limited. Miscellaneous uses include treatment of Budd Chiari Syndrome, portal hypertensive gastropathy and hepatopulmonary syndrome. Careful patient selection is vital to a successful outcome, as patients with severe liver dysfunction tend to die post-TIPS despite a functioning shunt. All patients who require a TIPS for treatment of complications of cirrhosis should be referred for consideration of liver transplant.

Hepatorenal syndrome.

Hepatorenal syndrome is complication of advanced cirrhosis characterized by renal failure, changes in systemic blood pressure, and increased activity of endogenous vasoactive systems. Renal failure is due to severe renal vasoconstriction developing in the late stages of cirrhosis. The pathogenesis of hepatorenal syndrome is the result of an extreme underfilling of the arterial circulation secondary to an arterial vasodilation located in the splanchnic circulation. This underfilling triggers a compensatory response with activation of vasoconstrictor systems. The diagnosis of hepatorenal syndrome is based on established diagnostic criteria aimed at excluding nonfunctional causes of renal failure. The prognosis of patients with hepatorenal syndrome is very poor. Liver transplantation is the best option in selected patients, but it is not always applicable due to the short survival expectancy and donor shortage. Pharmacological therapies based on the use of vasoconstrictor drugs (terlipressin, midodrine, octreotide or noradrenline) are the most promising in aims of successfully offering a bridge to liver transplantation. Prevention of hepatorenal syndrome with albumin infusion is recommended in patients with spontaneous bacterial peritonitis and with pentoxifylline in patients with acute alcoholic hepatitis.

[TIPS - Transjugular intrahepatic portosystemic shunt. A review]. / TIPS - anastomose portossistêmica intra-hepática transjugular. Revisão.

At the present time several therapeutic options are used for the treatment of bleeding esophageal varices in patients with portal hypertension. We will review the main medical publications on transjugular intrahepatic portosystemic shunt (TIPS), a procedure seldom used among us. TIPS works as a portocaval side-to-side shunt and decreases the risk of esophageal bleeding through lowering of the portal system pressure and a decrease of the portal hepatic pressure gradient. TIPS consists in the percutaneous insertion, through the internal jugular vein, of a metallic stent under fluoroscopic control in the hepatic parenchyma creating a true porta caval communication. There are several studies demonstrating the efficacy of TIPS, although only a few of them are randomized and control-matched to allow us to conclude that this procedure is safe, efficient and with a good cost benefit ratio. In this review, we search for the analysis of the TIPS utilization, its techniques, its major indications and complications. TIPS has been used in cases of gastroesophageal bleeding that has failed with pharmacologic or endoscopic treatment in patients Child-Pugh B and C. It can be used also as a bridge for liver transplantation. Others indications for TIPS are uncontrolled ascites, hepatic renal syndrome, and hepatic hydrothorax. The main early complications of TIPS using are related to the insertion site and hepatic encephalopathy and the stent occlusion is the chief late complication.

TIPS--anastomose portossistêmica intra-hepática transjugular. Revisao / TIPS - Transjugular intrahepatic portosystemic shunt. A review

At the present time several therapeutic options are used for the treatment of bleeding esophageal varices in patients with portal hypertension. We will review the main medical publications on transjugular intrahepatic portosystemic shunt (TIPS), a procedure seldom used among us. TIPS works as a portocaval side-to-side shunt and decreases the risk of esophageal bleeding through lowering of the portal system pressure and a decrease of the portal hepatic pressure gradient. TIPS consists in the percutaneous insertion, through the internal jugular vein, of a metallic stent under fluoroscopic control in the hepatic parenchyma creating a true porta caval communication. There are several studies demonstrating the efficacy of TIPS, although only a few of them are randomized and control-matched to allow us to conclude that this procedure is safe, efficient and with a good cost benefit ratio. In this review, we search for the analysis of the TIPS utilization, its techniques, its major indications and complications. TIPS has been used in cases of gastroesophageal bleeding that has failed with pharmacologic or endoscopic treatment in patients Child-Pugh B and C. It can be used also as a bridge for liver transplantation. Others indications for TIPS are uncontrolled ascites, hepatic renal syndrome, and hepatic hydrothorax. The main early complications of TIPS using are related to the insertion site and hepatic encephalopathy and the stent occlusion is the chief late complication.

Evaluación de las derivaciones porto sistémicas por vía transyugular (TIPS) por medio de ultrasonido Doppler en color, Doppler de poder y ecorrealzadores / Color doppler, power doppler and enhanced doppler sonography evaluation of transjugular intrahepatic portosystemic shunts

Los autores estudiaron 10 pacientes con hipertensión portal tratados con derivaciones portosistémicas por vía transyugular (TIPS) en los que evaluaron el flujo en el interior de la prótesis por medio de ultrasonido Doppler en color, Doppler de potencia y análisis espectral, antes y después de la aplicación de ecorrealzadores. Dos pacientes tuvieron flujos normales demostrados con el Doppler en color y con reconstrucciones en tercera dimensión. En seis pacientes encontraron signos de estenosis con reducción del flujo, uno en la porción proximal, tres en la porción media y dos en la distal; el empleo de ecorrealzadores en estos permitió mejorar el estudio del flujo y delimitar mejor el sitio y la morfología de la estenosis. En un paciente no se demostró flujo, debido a obstrucción.

Tratamiento de la ascitis refractaria mediante derivación transyugular intrahepática portosistémica: caso clínico / Treatment of refractory ascites using a transjugular intrahepatic portosystemic shunt TIPS: clinical case

Approximately, 10 percent of cirrhotic patients with ascites become refractory to medical treatment, a condition that reduces overall survival. TIPS is a therapeutic alternative for the management of refractory ascites. It corrects sinusoidal portal hypertension, becoming a rational and relatively safe therapeutic approach. The most frequent complications of TIPS are the development of hepatic encephalopathy and a high incidence of shunt stenosis or occlusion after one year of follow-up. We report a 43 years old alcoholic cirrhotic male with ascites refractory to medical treatment, that required multiple large volume paracentesis, whose clinical condition deteriorated progressively. A TIPS was successfully placed with a rapid and complete resolution of ascites and marked improvement of his clinical condition. During the ensuing six months the patient remained in excellent conditions, but was lost from follow-up and resumed excessive alcohol intake. Eight months after TIPS placement he had an upper gastrointestinal bleeding and died

Derivación portosistémica intrahepática percutánea transyugular: presentación de 1 caso y revisión de la literatura / Transjugular percutaneous intra-hepatic portosystemic shunt: presentation of one case and review of the literature

La hemorragia digestiva alta por varices esofágicas secundarias a hipertensión portal, sigue siendo un problema de dificil tratamiento. Han surgido diferentes alternativas para el control de la hipertensión portal. En este articulo se presenta una alternativa que, si bien no es nueva, ha tenido exito recientemente gracias al desarrollo de la protesis expandible de Palmaz que permite hacer una derivación portosistemica intrahepatica percutanea transyugular, con exito, manteniendo la permeabilidad y con buenos resultados hasta el momento. Se presenta un caso y se hace una revision de la literatura.