Interventional Treatment of Budd-Chiari Syndrome

Abstract

Medical treatment is regarded as the primary course of action in patients with Budd-Chiari syndrome (BCS). Its efficacy, however, is limited, and most patients require interventional treatment during follow-up. Short-segment stenosis or the occlusion (the so-called web) of hepatic veins or the inferior vena cava are frequent in Asian countries. An angioplasty with or without stent implantation is the treatment of choice to restore hepatic and splanchnic blood flow. The long-segment thrombotic occlusion of hepatic veins, common in Western countries, is more severe and may require a portocaval shunting procedure to relieve hepatic and splanchnic congestion. Since it was first proposed in a publication in 1993, the transjugular intrahepatic portosystemic shunt (TIPS) has gained more and more attention, and in fact it has been so successful that previously utilized surgical shunts are only used for few patients for whom it does not work. Both interventional treatment options can be performed successfully in about 95% of patients even after the complete obliteration of the hepatic veins. The long-term patency of the TIPS, a considerable problem in its early years, has been improved with PTFE-covered stents. The complication rates of these interventions are low and the survival rate is excellent with five- and ten-year survival rates of 90% and 80%, respectively. Present treatment guidelines recommend a step-up approach indicating interventional treatment after the failure of medical treatment. However, this widely accepted algorithm has several points of contention, and early interventional treatment is proposed instead.

Keywords: Budd–Chiari syndrome; TIPS; angioplasty; interventional treatment; survival; transjugular intrahepatic portosystemic shunt.

Figure 1

(A) Complete BCS. The portal vein is used as outflow tract. Enlarged caudate lobe leads to stenosis of the inferior caval vein; (B) Angiography of the inferior caval vein showing complete occlusion by the enlarged liver (VCI: inferior caval vein).

Figure 2

(A) Hand-injection of contrast into the parenchyma of the liver showing retrograde filling of a portal branch. (B) Sonographic guidance of the puncture. The location of the needle (arrow) and the portal vein is demonstrated.

Figure 3

(A) Portography (DSA) after successful puncture of a very narrow right intrahepatic branch of the portal vein. (B) Simultaneous portography and cavography demonstrating good shunt function and perfect modelling of the two stents at both ends. This patient had only mild stenosis of the inferior caval vein by the enlarged caudate lobe.

Figure 4

Duplex sonography showing stenosis in the proximal stent with a turbulent flow and a maximum flow velocity of 200 cm/s (2 m/s). According to the Bernouilli equation, the maximum flow velocity corresponds to a pressure gradient across the stenosis of 16 mmHg.

Figure 5

A 16-year-old female patient with fulminant Budd–Chiari syndrome due to essential thrombocytosis received a TIPS in 1998. Between 1998 and 2013, seven TIPS revisions were performed with implantation of additional stents with or without thrombolytic treatment. In 2013, the patient was admitted to the hospital for another revision after severe gastric variceal bleeding (see bucrylate clot). The catheterization of the occluded stent shunt was not possible and a transcaval puncture was performed for parallel stent implantation. The portography and simultaneous cavography (DSA) show good stent position and function. The shunt has been fully patent since then. Birth of a healthy girl in 2019.

Figure 6

Example of a very high portal pressure in a patient with fulminant BCS. The height of the water column (between the yellow arrows) is 82 cm, corresponding to 63 mmHg. The value was confirmed by electronic measurement.

Figure 7

Intrahepatic portal flow (blue) in a patient with BCS after TIPS implantation. Hepatic artery in red.

Figure 8

Development of Child–Pugh score, as well as Clichy and Rotterdam prognostic BCS indexes in 59 patients. TIPS improves the Child–Pugh score as well as the Clichy and the Rotterdam indexes significantly during the index hospital stay and thereafter. The effect was greatest in patients treated for acute or fulminant BCS (Adapted and modified with permission from Dr. T. Kappenschneider [31]).

Figure 9

Survival of patients after TIPS implantation. Two patients were transplanted; one of them died.

Figure 10

Proposal of a treatment algorithm and timing of interventions. Angioplasty should be performed without delay if a web-like BCS has been diagnosed. After successful angioplasty (demonstrated by lack of a significant gradient across the stenosis), the hepatic venous pressure gradient should be determined. An elevated gradient (>12 mmHg) should indicate the need for a transjugular liver biopsy, and, in case of advanced fibrosis, a TIPS should be implanted. The timing of the TIPS in patients with long-segment BCS may depend on the clinical severity of the disease. In symptomatic patients (liver failure, ascites, varices), early TIPSs may be indicated without waiting on their response to medical treatment.