Approach

The aim of therapy and interventional management in patients with Budd-Chiari syndrome (BCS) is to relieve hepatic congestion and prevent necrosis, fibrosis, and ultimately liver failure, cirrhosis, and/or portal hypertension.

The management of BCS requires a team approach with hepatology, haematology, radiology, and surgical expertise. The use of a stepwise treatment algorithm in which therapeutic procedures are performed in order of increasing invasiveness, based on the response to the previous treatment, is recommended.​[1][2][54]​​

Conventional management of patients with BCS is anticoagulation, medical treatment of the complications of portal hypertension, and treatment of any underlying (haematological) disease. However, this approach is successful in only 18% of patients. By adding recanalisation (thrombolysis, angioplasty, or stenting) of short-length hepatic vein stenoses, the success rate increases to 32%.[54] Thus, most patients need more aggressive treatments.

The interventional approach should be carried out in a specialised liver centre that is able to perform all interventional radiological techniques as well as surgical shunts and liver transplantation.

The therapeutic approach to primary BCS is diverse and should be adapted to disease severity and symptoms. Treatment of secondary BCS is by treatment of the cause: for example, tumour resection.

Fulminant primary BCS

Patients with fulminant BCS (hepatic encephalopathy within 8 weeks after the development of jaundice; tender and enlarged liver; renal failure and coagulopathy) require emergency liver transplantation. If this is not feasible, transjugular intrahepatic portosystemic shunts (TIPS) is an interim measure until liver transplantation becomes available.[2]​ Anticoagulant therapy is started after liver transplantation, and treatment of the underlying pro-coagulant condition is also required.

Non-fulminant primary BCS: medical therapy

Anticoagulant therapy is required to prevent progression of thrombosis. Anticoagulation is initiated in all patients, unless there are contraindications such as oesophageal varices, or there is evidence of ongoing liver necrosis (e.g., worsening symptoms, poorly controlled ascites, rising serum transaminases).[42] Asymptomatic patients also require anticoagulation, as underlying prothrombotic states are often present. Use low molecular weight heparin initially (e.g., enoxaparin, dalteparin), followed by a vitamin K antagonist (e.g., warfarin).[1]

Thrombolysis may be used to relieve hepatic venous outflow obstruction and restore normal blood flow.[42] It is most effective in acute BCS (within 72 hours) and in short segment thrombosis as adjuvant to angioplasty.[55] Local, systemic, or combined thrombolysis has been used.[56]

Ascites is treated with diuretics (e.g., furosemide, spironolactone). Combined diuretic therapy is recommended if response to single therapy is not satisfactory. If ascites is still not controlled by medical therapy, paracentesis may be required.

The predisposing thrombophilic condition is treated in accordance with standard practice. See the following topics: Essential thrombocythaemia (Management), Polycythaemia vera (Management), Myelofibrosis (Management), Chronic myeloid leukaemia (Management), Antiphospholipid syndrome (Management), Paroxysmal nocturnal haemoglobinuria (Management).

If control of ascites and other complications of BCS (e.g., encephalopathy, coagulopathy, gastrointestinal bleeding) cannot be managed by medical therapy alone, or if clinical features and biochemical testing suggests ongoing liver necrosis, invasive treatments are required.

Non-fulminant primary BCS: radiological interventional therapy

Interventional procedures include angioplasty of hepatic vein, inferior vena cava (IVC), or both, and TIPS. If there is fresh clot, thrombolytic therapy can be given alongside interventional treatment, by infusing a thrombolytic agent directly into the involved vein. Calcification of a stenosed segment precludes radiological treatment and is an indication for surgery.[57]

The approach to radiological interventional therapy differs according to geographical regions, due to the differing aetiology of BCS. In Western countries, extensive hepatic vein thrombosis due to myeloproliferative neoplasms predominates, while in Asia, hepatic vein obstruction is mostly due to a membranous web. Therefore, the most common approach may be TIPS in Western countries, and recanalisation (e.g., percutaneous transluminal angioplasty and stenting) in Asia.[17]

Hepatic angioplasty

  • Hepatic angioplasty (balloon dilation with or without stent insertion) of localised narrowed hepatic veins is reported to relieve symptoms in 70% of patients.[15][58] However, success rates vary widely across centres.

  • Hepatic venographic approaches include a femoral or transjugular route, direct percutaneous transhepatic approach, or a combined approach. A combined approach is used when ultrasound shows patency of a significant segment of a major hepatic vein, but this is inaccessible from the IVC.[59][60]

  • Angioplasty can be used to treat complications of surgical shunts and to manage recurrent venous obstruction until liver transplantation is available.[61][62]

IVC angioplasty

  • Balloon dilation is usually sufficient to open up the obstruction (web or stenosis). However, when the obstruction is associated with thrombus below, mechanical or medical thrombolysis is needed prior to dilation.

  • IVC stent insertion is usually needed when balloon dilation is insufficient alone, or when there is recoil, or recurrent stenosis.[63]

  • Combined hepatic vein angioplasty is required when hepatic vein outflow is compromised.[64][65]

TIPS

  • Failure of angioplasty (because the remaining patent veins are too small or have insufficient flow) and the presence of diffuse hepatic vein thrombosis are indications for shunting procedures (TIPS or surgical shunting).

  • TIPS is considered the most common interventional procedure for BCS in Western countries.[66] For the shunt to function, the portacaval pressure gradient should be <10 mmHg.

  • TIPS procedure can be used in both elective and emergency situations.[67][68] Elective TIPS may be performed in patients with refractory ascites, recurrent variceal bleeding, hepatic outflow obstruction resulting from compression of the intrahepatic IVC by a hypertrophied caudate lobe, or poor hepatic reserve precluding surgical shunting. TIPS is done on an emergency basis in fulminant BCS to serve as a bridge to liver transplantation.[1][2]

  • TIPS has been used in the treatment of patients with concurrent hepatic outflow obstruction and portal vein thrombosis, although the procedure can be challenging or impossible with chronic portal vein thrombosis.[69]

  • The introduction of covered stents has been reported to improve the short-term outcome results of TIPS in BCS.[70] This is now in use in liver intervention centres.

Non-fulminant primary BCS: surgical management

Surgical procedures in BCS include shunt surgery and liver transplantation.

Surgical shunting

  • Indicated in patients not improving by radiological angioplasty and if the portacaval venous pressure gradient is ≥10mmHg.

  • Indicated if there is good hepatic reserve and insignificant fibrosis. Better surgical outcomes are seen in patients with Child-Pugh class A and where the underlying cause has a favourable long-term outcome, such as essential thrombocythaemia.[42]

  • Side-to-side portacaval shunts, side-to-side mesocaval shunts, and cavoatrial shunts are used for patients with both caval and hepatic venous obstruction.[71]

  • Mesoatrial shunt is indicated if the IVC is obstructed, thrombosed, or compressed by a hypertrophied caudate lobe, resulting in low portacaval pressure gradient (however, a TIPS is usually a better approach in this setting).

  • Inferior vena caval membranous obstruction is managed by transatrial membranectomy, or, less commonly, by dorsocranial resection of the liver with hepatico-atrial anastomosis.[71][72]

Liver transplantation

  • Indications for transplantation include advanced cirrhosis, and failure of non-surgical treatment approaches or portosystemic shunting.[38][73][74]

  • Seventy-five percent of patients with BCS have a detectable genetic hypercoagulable state. Liver transplantation can cure almost all hereditary thrombophilias; however, thrombosis can still occur and anticoagulation is necessary.[4]

  • Living-donor liver transplantation using modified cavoplasty is a highly effective treatment for BCS and, in conjunction with long-term anticoagulant therapy and interventional radiological treatment, provides good long-term survival.[75]

Secondary BCS

Surgical removal of the compressing cyst, abscess, or invading tumour is required.

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