Chapter 17: Budd-Chiari Syndrome: Transjugular Intrahepatic Portosystemic Shunt and Hepatic Vein Recanalization

Budd-Chiari syndrome (BCS) refers to hepatic venous outflow obstruction, occurring at any level from hepatic veins to the suprahepatic segment of the inferior vena cava (IVC). The obstruction may be caused by hepatic lesions such as a tumor, cyst, or abscess and is termed “secondary BCS,” but this accounts for only a small number of cases. In most cases, it occurs from thrombosis of the hepatic veins or the IVC and is termed “primary BCS.” Several prothrombotic risk factors are known to cause primary BCS ( Table 17.1), one or more of which are detected in almost 90% of cases.¹

Primary BCS begins with acute thrombosis of the hepatic vein outflow over a variable length of the hepatic vein, IVC, or both. This later evolves toward fibrous sequelae, forming stenosis, webs, or membranes.² The resultant venous obstruction causes an increase in hepatic sinusoidal pressure that leads to a cascade of events in the liver, beginning with hepatocellular congestion, necrosis, and finally cirrhosis. Over a period of time, venous collaterals develop in an attempt to spontaneously decongest the liver. Extrahepatic portal vein thrombosis is seen in 15% of patients with BCS, likely secondary to flow stasis within the portal vein and preexisting prothrombotic diatheses.³

Budd-Chiari syndrome is classified according to the level of hepatic venous outflow obstruction ( Table 17.2). Endovascular treatment of BCS is based on the level of obstruction, chronicity of disease, and morphology of the obstructive lesion.^4,5

Treatment of Budd-Chiari Syndrome

Anticoagulation and, if possible, treatment of underlying disorders (e.g., myeloproliferative disease, paroxysmal nocturnal hemoglobinuria) form the cornerstone of therapy in BCS and should be initiated as early as possible in the disease.⁴ Anticoagulation alone succeeds in controlling liver disease in 10% of patients.⁵

Next, whenever possible, recanalization of the hepatic venous outflow by angioplasty and stenting should be attempted because it restores physiological blood flow and decongests the liver with minimal risk. This is offered in both symptomatic and asymptomatic patients and is feasible if the stenosis or occlusion of the hepatic veins or IVC is of short segment.⁶

A transjugular intrahepatic portosystemic shunt (TIPS) is recommended in symptomatic patients with BCS when (1) the hepatic vein occlusive segment is long, (2) there is failure to recanalize the hepatic veins, or (3) there is no clinical benefit from hepatic vein recanalization.^7–10 Surgery is considered only when radiologic procedures fail to resolve the symptoms or if the liver dysfunction is very severe, warranting an urgent liver transplantation.

Hepatic Venous Recanalization

Strategies to Recanalize Hepatic Venous Outflow

The first step involves angiographic assessment of the venous outflow. If the IVC lumen is compromised at or above the level of insertion of hepatic veins, it should be recanalized first. Caval recanalization would suffice if the hepatic veins are patent or if there is a prominent inferior right hepatic vein that contributes to the hepatic venous outflow. Hepatic vein recanalization is required in absence of any single, dominant hepatic outflow channel. For this, restoring flow to the largest of the three hepatic veins is usually sufficient for relief of symptoms.

Table 17.1 Common Causes of Thrombotic Diathesis Associated with Budd-Chiari Syndrome, with Recommended Investigations1

MTHFR: methylene tetrahydrofolate reductase.

Table 17.2 Classification of Budd-Chiari Syndrome According to Site of Obstruction4,5

IVC: inferior vena cava.

Technique of Inferior Vena Cava Recanalization

Recanalization is usually attempted from a femoral transvenous approach using a catheter and hydrophilic guidewire to cross the lesion. An IVC web or stenosis is generally traversable and can be easily dilated and stented with a large-diameter self-expandable or balloon-expandable stent ( Fig. 17.1).

An IVC membrane, however, cannot be crossed easily with a guidewire and often requires fenestration. This can be achieved with the stiff back end of a guidewire or with a Rösch-Uchida or Ring needle, at times accompanied by a coaxial long 22-gauge needle advanced carefully toward the patent suprahepatic IVC. A suitable entry point can be identified with a pigtail catheter placed in the suprahepatic IVC from a jugular approach ( Fig. 17.2). After successful fenestration, the membrane is sequentially dilated with angioplasty balloons of increasing diameter before deployment of a large-diameter stent.

Technique of Hepatic Vein Recanalization

The hepatic vein can be recanalized from a transvenous approach or a transhepatic approach, either alone or in combination.

1. Transvenous approach ( Fig. 17.3): The hepatic vein ostium is accessed from a jugular or femoral access, after which the hepatic vein lesion is traversed with a guidewire or fenestrated with a TIPS puncture needle. The lesion is then dilated and stented, usually with a balloon-expandable stent.

2. Transhepatic approach ( Fig. 17.4): This is generally used when the transvenous approach fails. A peripheral hepatic vein branch is accessed with an Accustick or Neff set under ultrasound guidance, and a 6- to 8-Fr sheath is inserted. An appropriately angled angiographic catheter and guidewire are then used to cross the hepatic vein lesion. If the lesion cannot be traversed with a guidewire, a Chiba needle can be used for fenestration. After it is crossed, the lesion is dilated and stented from the transhepatic route itself, after which the tract is embolized with coils or glue. It is common for patients with BCS to have large-volume ascites; therefore, a peritoneal drain may be required when this approach is being used.

Fig. 17.1 A 54-year-old man with Budd-Chiari syndrome from an occult myeloproliferative disorder. (a) Cavogram shows a weblike narrowing (arrow) in the suprahepatic inferior vena cava with a 30–mm Hg pressure gradient across it. (b) The lesion could be crossed with a hydrophilic wire, dilated sequentially with 10-and 16-mm angioplasty balloons, and stented with a 20-mm diameter nitinol stent.

Fig. 17.2 Inferior vena cava (IVC) membrane in a 43-year-old man with systemic lupus erythematosus. (a) Cavogram shows a 2-cm-long IVC occlusion. (b) A Rösch-Uchida liver access set (RUPS) cannula was introduced, with its tip directed superiorly and anteriorly toward the patent suprahepatic segment. (c) Fenestration done with a 22-gauge coaxial Chiba needle, using a pigtail catheter placed in the suprahepatic IVC as a target. (d) Cavogram after balloon dilatation and deployment of a Palmaz XL balloon-expandable stent.

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