Hepatic Vein Thrombosis

GENERAL PRINCIPLES

Hepatic vein thrombosis (HVT), also known as Budd-Chiari syndrome, causes hepatic venous outflow obstruction. It has multiple etiologies and a variety of clinical consequences.

• Thrombosis is the main factor leading to obstruction of the hepatic venous system, frequently in association with myeloproliferative disorders (i.e., polycythemia vera), antiphospholipid antibody syndrome, paroxysmal nocturnal hemoglobinuria, factor V Leiden, protein C and S deficiency, Jak-2 mutation, and contraceptive use.

• Membranous obstruction of the inferior vena cava (IVC) and stenosis of the IVC anastomosis after liver transplantation are conditions that present clinically similar to HVT.

• Some cases are idiopathic.

DIAGNOSIS

Clinical Presentation

Patients may present with acute, subacute, or chronic illness characterized by ascites, hepatomegaly, and right upper quadrant abdominal pain. Other symptoms may include jaundice, encephalopathy, GI bleeding, and lower extremity edema.

Diagnostic Testing

• Serum-to-ascites albumin gradient (SAAG) is gt;1.1 g/dL. Serum albumin, bilirubin, AST, ALT, and PT/INR are usually abnormal.

• Laboratory evaluation to identify a hypercoagulable state should be performed (see Chapter 20, Disorders of Hemostasis and Thrombosis).

• The diagnosis can be established with Doppler ultrasound. Other diagnostic tools include magnetic resonance venography, hepatic venography, or cavography.

• Consider cross-sectional imaging to exclude space-occupying lesion leading to external compression and subsequently hepatic venous outflow obstruction.

TREATMENT

• Correct underlying cause when possible.

• To prevent propagation of the clot, initiate anticoagulation immediately, if there are no contraindications.

• Thrombolysis can be considered for well-defined clots and angioplasty if the venous obstruction is amenable to intervention for highly selected patients.

• Manage portal hypertension complications per guidelines.

• Consider transjugular intrahepatic portosystemic shunt (TIPS) if there is minimal improvement despite anticoagulation and maximal medical therapy.²²

Surgical Management

Liver transplantation is an option in patients who develop cirrhosis or ALF.