Hepatic vein thrombosis, or Budd-Chiari syndrome (BCS), is a rarely diagnosed liver disease. It can lead to its failure, cirrhosis and even necrosis. What are the causes and symptoms of hepatic vein thrombosis? What is the treatment of BCS syndrome?

Hepatic vein thrombosis , otherwiseBudd-Chiari syndrome (BCS) , is a disease whose essence is the blockage of blood outflow from the hepatic veins until the inferior vena cava joins the right atrium. Depending on the degree of obstruction in the outflow of venous blood from the liver and obstruction of the hepatic veins, it is distinguished by a fulminant, acute and chronic form of the disease.

Hepatic vein thrombosis - causes

There are primary and secondary hepatic vein thrombosis. Primary BCS can be defined as a process within the vessel (e.g. blood clot, inflammation) that causes blood flow disorders. It may be the result of an inherited condition (e.g. protein C deficiency, mutation of the prothrombin gene) or an acquired disease (e.g. antiphospholipid syndrome, nocturnal paroxysmal hemoglobinuria, or Behçet's disease).

Secondary thrombosis of the hepatic veins is a consequence of pressure on the vessel by adjacent structures, e.g. cancerous tumors (liver cancer, kidney cancer), abscesses and cysts.

There are also cases where thrombosis is associated with the use of oral contraceptives and immunosuppressants.

Read also: It affects women more often. How to prevent thrombosis?

Hepatic vein thrombosis - symptoms

The first symptoms of hepatic vein thrombosis are abdominal pain and fever (if an infection has developed at the same time). In the advanced stage of the disease, symptoms appear as a consequence of liver failure and portal hypertension:

  • ascites,
  • enlargement of the liver (hepatomegaly) and spleen (so-called splenomegaly),
  • peripheral edema,
  • bleeding from esophageal or gastric varicose veins,
  • encephalopathy (disturbance of the functioning of the central nervous system due to the action of toxins appearing in the system due to liver damage).

In patients with advanced and long-term inferior vena cava thrombosis, significant dilatation of subcutaneous vessels may be observedvenous abdominal cavity. Depending on the form of the disease, these symptoms may develop slowly and be poorly felt (chronic form) or very quickly and intensely (fulminant form).

Hepatic vein thrombosis - diagnosis

If hepatic vein thrombosis is suspected, an ultrasound of the abdominal cavity is performed, which allows to assess the flow in the hepatic veins and the portal system. The final diagnosis is made on the basis of computed tomography and Doppler ultrasound, thanks to which the direction and speed of blood flow within the hepatic veins can be assessed.

Read also: What is Doppler testing?

Hepatic vein thrombosis - treatment

There are four forms of treatment for hepatic vein thrombosis. Therapy usually begins with the administration of anticoagulants. Only when this method turns out to be ineffective, another one is used until the patient is healed.

  1. Pharmacological anticoagulant treatment (low molecular weight heparin and other anticoagulants are administered)
  2. Hepatic veins angioplasty and prosthesis.
  3. Transjugular Portal Systemic Intrahepatic Fistula (TIPS). This procedure can be performed on patients who have an obstructed portal vein.
  4. Liver transplantation can be performed in patients who have failed anticoagulation, angioplasty, and TIPS treatment, and in patients who have been diagnosed with fulminant liver failure.

Category:

Cardiology