Transjugular portosystemic shunting (TIPS): Indications and technique


Introduction

Besides the well-established role of transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of portal hypertension, additional indications in noncirrhotic, presurgical, and oncologic patients, as well as portal vein thrombosis, have evolved over the past several years. In presurgical patients, TIPS has been used before major interventions such as colectomy in efforts to reduce the risk of intraoperative bleeding. In anticipation of liver or multivisceral transplant TIPS has become a tool to reestablish physiologic portal venous flow, especially in cases of portal vein thrombosis, to avoid organ hypoperfusion and improve outcomes. Portal vein recanalization–TIPS (PVR-TIPS) may achieve recanalization in patients even with complete portal vein thrombosis. Bureau et al. demonstrated in a randomized controlled trial that polytetrafluoroethylene (PTFE)–coated TIPS led to an increase in transplant-free survival in patients with cirrhosis and recurrent ascites, widening the possibilities to not only bridge patients but use TIPS as definitive therapy. Berry et al. reported from an analysis of the UNOS (United Network for Organ Procurement) database the benefit of TIPS to reduce the mortality rate in the waiting list.

Currently, with the advance of locoregional therapies to bridge or downstage liver tumors, it is not uncommon to face the need of treating patients with TIPS requiring other therapies such as transarterial chemoembolization (TACE), yttrium-90, or ablation. This makes it necessary to have extensive multidisciplinary evaluation for correct selection and to prevent complications that could affect the quality of life and expectancy. The concomitant use of TIPS and intraarterial chemoembolization has been reported as safe and effective in patients with hepatocellular carcinoma (HCC) undergoing liver transplantation. Technically, TIPS is still a challenging intervention, requiring, along with the conventional approach, the development of technologic tools to aid portal venous access and decompression, including intravascular ultrasound (IVUS) guidance, cone beam computed tomography (CT), and new control expandable stents.

Indications

In 2009 the American Association for the Study of Liver Diseases (AASLD) published practice guidelines that include the primary and secondary roles of TIPS for portal hypertension (see Chapters 74 , 77 , and 78 ). Since then, multiple updates have been written, including the use of PTFE-covered stents to improve patency, the use of TIPS for Budd-Chiari syndrome (see Chapter 86 ), and specific algorithms as a second-line therapy for the management of ascites. , The European Association for the Study of the Liver (EASL) produced clinical guidelines for the management of patients with decompensated cirrhosis, using evidence from PubMed and Cochrane database searches before March 2018 and guided by a panel of experts. The value of TIPS versus a surgical shunt in the prevention of variceal rebleeding in patients who have failed medical therapy, as well as the cost analysis, has been clarified by the publication of a controlled trial comparing TIPS versus distal splenorenal shunt (DSRS). It was concluded that TIPS was slightly more cost effective than DSRS at year 5, but these two approaches were of equal efficacy in the prevention of variceal rebleeding. , Current indications from multiple clinical trials are listed in Box 85.1 .

BOX 85.1
Transjugular Intrahepatic Portosystemic Shunt (TIPS): Current Indications

  • Uncontrollable esophageal or gastric variceal hemorrhage

  • Current or prior variceal hemorrhage that is not amenable to initial or continued endoscopic therapy

  • Prophylaxis against recurrent variceal bleed in high-risk patients (5 days from initial bleeding)

  • Recurrent bleeding from ectopic varices or stomas, for which nonselective beta-blockers and/or endoscopic treatment fails

  • Portal hypertensive gastropathy or enteropathy (transfusion dependent and not responding to medical therapy)

  • Refractory ascites

  • Hepatic hydrothorax

  • Portal vein thrombosis

  • Budd-Chiari syndrome

  • Hepatorenal syndrome

  • Decompression of portosystemic collaterals before abdominal surgical procedures

  • Portal hypertension associated with malignancies (palliation)

  • Hepatopulmonary syndrome * in study

* In the hepatopulmonary syndrome, the role of TIPS is still unclear, and it is hypothesized that it can be as a result of lessening of intrapulmonary vascular dilations (IPVDs). However, it has been shown to improve hypoxemia in some patients and can be safely performed for the treatment of other complications of portal hypertension in the presence of hepatopulmonary syndrome and reasonably used as a bridge toward liver transplant.

Authors have identified predictor scales, including the Model for End-stage Liver Disease (MELD), Child-Turcotte-Pugh (CTP), Apache II, and more recently the Garcia-Pagan score, this last one to predict the survival of patients with Budd-Chiari syndrome undergoing TIPS (see Chapter 4 ). Despite multiple attempts to develop new predictive models, MELD and CTP are still the best predictors of post-TIPS survival. In 2010 a randomized trial described that early preemptive TIPS, performed within 72 hours of an acute episode of variceal bleeding, demonstrated an increased survival benefit for patients who were Child-Pugh class C and with no increase in the risk of hepatic encephalopathy.

Acute variceal hemorrhage (see Chapters 80 and 81 )

Increased portal pressure is a determining factor for both variceal rupture and the severity of the bleeding episode. The common hemodynamic end point after decompressing the portal vein is a portosystemic gradient of 12 mm Hg. Clinical success is indicated by cessation of demonstrable gastrointestinal bleeding, transfusion requirements, pharmacologic support, or balloon tamponade, and by return of hemodynamic stability with or without performance of adjunctive variceal embolization when indicated. The main downfall to TIPS has been the development of new hepatic encephalopathy or exacerbation of existing encephalopathy, occurring in 20% to 31% of cases (see Chapter 79 ). It is for this reason that TIPS has been recommended only after failure of medical management and not as front-line therapy for variceal bleeding. The concept of early TIPS within 72 hours from the initial bleeding episode (ideally ≤24 hours) was described for patients at high risk of first-line treatment failure (i.e., Child-Pugh class B with active bleeding at index endoscopy or in Child-Pugh class C score lower than 14 points). Early TIPS demonstrates an important advantage in terms of absolute risk reductions of mortality in high-risk patients with MELD ≥19 or Child-Pugh C cirrhosis. , Multiple studies and one meta-analysis have all shown significantly improved survival with stent graft TIPS compared with medical/endoscopic therapy. ,

Isolated gastric varices and portal gastropathy

The risk of bleeding for gastric varices has been reported at about 10% to 16% per year. Sarin classification is the most used to classify gastric varices and is based on the endoscopic and anatomic characteristics. The approach for cardiofundal varices Gastroesophageal varices type 1 in Sarin’s classification (GOV 1), considered an anatomic continuation of the esophageal varices, has been described with a relative consensus that they be treated like the esophageal varices with medical and endoscopic therapy as a first-line and TIPS as a second-line treatment. Isolated gastric varices often appear as a result of portomesenteric vein thrombosis and are associated with spontaneous portosystemic shunts. Some reports suggest that the use of cyanoacrylate may be the elective treatment for gastric varices followed by repeated sessions of glue injection along with nonselective beta-blockers as secondary prophylaxis. A randomized trial showed that rebleeding was significantly less frequent in the TIPS group compared with variceal obliteration with endoscopic cyanoacrylate injection (see Chapters 80 and 81 ).

Portal hypertensive gastropathy (PHG) might be clinically important because it is sometimes responsible for insidious blood loss (chronic iron deficiency anemia) and in exceptional cases even overt acute bleeding. In patients with medically refractory PHG and compensated cirrhosis, TIPS has been shown to improve the endoscopic appearance and decrease the transfusion requirement. In one of the largest studies (40 patients with PHG) endoscopic improvement was detected between 6 weeks and 3 months after TIPS placement.

Ectopic varices

Ectopic varices are defined as large pressurized portosystemic venous collaterals occurring anywhere in the abdomen except in the gastroesophageal region and account for up to 5% of all variceal bleeding. These collaterals occur where the portal venous system is in juxtaposition to the systemic venous system. After development of intrahepatic portal hypertension, these shunts act to divert flow from the increased intrahepatic vascular resistance. The varices can be related with prior abdominal surgeries in ostomies, or can appear in unusual locations such as ovaries and bladder. They have also been associated, in the absence of portal hypertension, with congenital anomalous portosystemic anastomoses, abnormal vessel structures, arteriovenous fistulae, or thromboses. TIPS decompression provides primary intervention or salvage modality in cases of bleeding; however, although ectopic varices decompress through different pathways, the rebleeding rate is higher. Therefore the combination with embolization has been reported to be superior to TIPS alone.

Ascites

Early investigators recognized that TIPS led to a reduction or resolution of ascites in many patients (see Chapter 79 ). Several studies have tried to determine positive predictors of post-TIPS ascites resolution. MELD score, aspartate transaminase levels, hepatic-portal venous gradient, creatinine levels, glomerular filtration rate, and platelet count have been found to predict a good response to TIPS. , In general, 54% to 79% of patients show significant ascites resolution after TIPS. A recent randomized trial found covered stents for TIPS to increase the proportion of patients with cirrhosis and recurrent ascites who survive transplantation-free for 1 year, compared with patients given repeated large-volume paracenteses and albumin. These findings support TIPS as the first-line intervention in such patients.

Hepatic hydrothorax

TIPS is reserved for patients with hepatic hydrothorax who are refractory to medical management (see Chapter 79 ). Liver transplantation, if indicated, constitutes the best long-term treatment option for patients with refractory hepatic hydrothorax (see Chapter 105 ). TIPS has been considered as definitive treatment or bridge to transplantation in patients with refractory hepatic hydrothorax. Patients requiring multiple thoracenteses or chest tubes can benefit from TIPS, with a beneficial response reported in 74% to 79% of patients undergoing the procedure. , Of those who respond, about two thirds have complete resolution of the hydrothorax, and the remainder experience partial resolution of the effusion but are symptomatically improved, with either decreased or resolved dyspnea. In our experience, the patients who showed no clinical response to TIPS were all critically ill with elements of multiorgan failure, and 30-day mortality was 83%. Thus TIPS is unlikely to be helpful as a last-ditch effort to improve pulmonary function in severely ill patients on mechanical ventilation.

Portal vein thrombosis

TIPS in portal vein thrombosis (PVT) is considered in select cases of obstructive PVT with worsening symptomatic portal hypertension but only in select institutions where expertise in performing this procedure optimally is available. Excellent rates of improvement and recanalization of PVT with TIPS have been reported; however, anticoagulation is also associated with similar results. , In addition, portal vein recanalization (PVR) and TIPS creation (PVR-TIPS) has been performed in patients with PVT to improve their candidacy for transplantation by providing a patent portal vein at the time of transplantation and treat portal hypertension symptoms, decreasing the mortality on the liver transplant waiting list , ( Fig. 85.1 ). The goal is to permit a physiologic portal vein reconstruction, which has demonstrated similar survival to that of patients without PVT. , Although this can often still be accomplished from a transjugular route, use of a percutaneous transhepatic or transsplenic access is sometimes needed to initially recanalize the portal vein ( Fig. 85.2 ). Thornburg et al. reported 60 successful cases of transsplenic PVR-TIPS with no cases of recurrent PVT following transplantation and 5-year overall survival rate of 82%. In the acute PVT setting, TIPS constitutes a mechanical tool to reestablish the venous outflow when the intrahepatic portal venous branches are thrombosed and provides an access route to perform thrombectomy and portomesenteric venous lysis. The technical success rate for establishing a shunt and maintaining patency of the portal system is generally 70% to 100%, , with the technical failures a result of the inability to traverse the portal occlusion. However, in patients in whom the occlusion has progressed to cavernous transformation, the technical success rate in some studies has been as low as 35%. , Recently, the use of transsplenic approach has become more common for PVR-TIPS even in the setting of cavernous transformation.

FIGURE 85.1, Patient with cavernous transformation of the portal vein requiring transjugular intrahepatic portosystemic shunt (TIPS) for portal hypertension. A, Initial venogram with the catheter in the right hepatic vein. B, A Colapinto needle (Cook Medical) used to target the portal vein collaterals. It is inserted through an introducer extending from the right internal jugular vein across the right atrium and hepatic vein. C, Venogram with the catheter at the SMV (Superiotr Mesenteric Vein) demonstrating cavernous transformation of the portal vein without visualization of a main residual vessel. D and E, The shunt tract was measured using a marker catheter. The stent was deployed from the collateral vein draining the portomesenteric vein confluence into the hepatic vein confluence. Final TIPSogram demonstrated widely patent stent and no further filling of portal venous collaterals.

FIGURE 85.2, Transsplenic recanalization of the portal vein: patient with portal vein thrombosis requiring transjugular intrahepatic portosystemic shunt (TIPS) for intractable ascites. A, Transsplenic access and catheterization of the splenic vein using ultrasonographic guidance. B, Contrast injection in the splenic vein through a catheter demonstrated occlusion of the main portal vein and opacification of the inferior mesenteric vein (IMV) with retrograde flow. C, A wire was manipulated across the remnant main portal vein and is used as a target to create the shunt. A Colapinto needle (Cook Medical) is advanced from the hepatic vein through the liver into the main portal vein remnant. D and E, A wire was inserted throughout the tract and the tract was dilated. F, A final TIPSogram demonstrated widely patent shunt without visualization of retrograde flow or varices.

If a large enough extrahepatic collateral vessel is present it can be used as a valid “landing zone” for the portal side of the TIPS. Malignant portal vein thrombosis due to hepatocellular carcinoma has been sometimes felt to be a contraindication for TIPS. However, several studies have shown that TIPS can still be done in this setting with very high technical success rates, low complications, and good relief of both variceal bleeding and intractable ascites. , In patients with a reasonable life expectancy TIPS can be considered for palliation of portal hypertensive symptoms despite malignant thrombosis.

Budd-Chiari syndrome

TIPS is an alternative treatment in Budd-Chiari syndrome (BCS) after failed medical therapy and other endovascular therapies such as angioplasty and stent insertion on the hepatic veins or inferior vena cava (IVC) (see Chapter 86 ). In patients without a remaining hepatic vein stump or complete occlusion, a direct portosystemic shunt (DIPS) is an option, with a puncture from the intrahepatic IVC into the liver parenchyma to target the portal vein via the caudate lobe. If a remnant of the hepatic vein (HV) is available, the needle passing across the liver parenchyma can start from the HV stump, if it can be engaged. If the HV stump cannot be engaged, passes can be made directly from the IVC itself. The needle pass should start from the retrohepatic segment of the IVC to avoid the chance of hemorrhage. Garcia-Pagan et al. reported long-term outcomes for patients with severe BCS treated with TIPS even in high-risk patients, suggesting that TIPS may improve survival. They also identified a small subgroup of BCS patients with poor prognosis despite TIPS who could possibly benefit from early OLT.

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