Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review

Abstract

Budd-Chiari syndrome (BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt (TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.

Keywords: Budd-Chiari syndrome; Interventional radiology; Liver; Orthotopic liver transplantation; Portal hypertension; Transjugular intrahepatic portosystemic shunt.

Figure 1

Twenty-nine years old girl with Budd-Chiari syndrome and leiden factor V positive for heterozygote. A: Gadolinium-enhanced T1-weighted magnetic resonance image (MRI) obtained during arterial phase. Hyperintense structures represent portal venules, which are visible because of postsinusoidal portal hypertension; B: T2-weighted MRI shows hyperintensity ascites and splenomegaly; C: Using a Rösch-Uchida transjugular liver access set, a small collateral hepatic vein branch was accessed, the portal vein was punctured (D), and wire access into the superior mesenteric vein was achieved. A 10-mm diameter, 6-cm long Viatorr stent (W. L. Gore and Associates, Flagstaff, AZ, United States) was deployed, extending from the right portal vein to the inferior vena cava (E, F).

Figure 2

Access technique. A: Initial inferior vein cava (IVC) venography depicting the origin of the obstructed right hepatic vein (arrowhead); B: Colapinto stylet positioning prior direct puncture of the IVC at the level of the origin of the thrombosed right hepatic vein (arrowhead), just below the diaphragm. Note the tip of the sheath within the right atrium (arrow); C: The Colapinto needle is turned anteriorly, parallel to the spine and access obtained at the main right portal branch; D: Final result after the deployment of 2 stent grafts.

Figure 3

Transjugular intrahepatic portosystemic shunt revision. Shunt created 9 years before, using a dedicated stent graft (VIATORR^® TIPS Endoprosthesis; GORE^®, United States), in a patient with Budd-Chiari syndrome due to primary thrombocythemia. A: Computed tomography image demonstrating a complete occlusion of the intra-parenchymal segment of the stent graft; B: Digital subtraction angiography (DSA) following lesion crossing, confirming the occlusion; C: Final DSA demonstrating flow restoration following deployment of a 10 mm × 80 mm stent graft (Fluency™; BD, United States).