Course of disease in a Budd- Chiari syndrome
In the Budd- Chiari syndrome, there is an increasing deterioration of the liver function due to the outflow disorder. This leads to the accumulation of abdominal fluid and the abdominal girth increases. Depending on when the Budd- Chiari Syndrome is treated and whether the treatment ensures a successful blood supply to the liver, the symptoms may improve.
If the Budd- Chiari Syndrome is not treated, the increased pressure in the liver leads to progressive liver damage and to a connective tissue remodelling of the liver (liver fibrosis). This can lead to liver failure. The duration of the treatment of a Budd- Chiari syndrome depends on the underlying cause, the time of its development and the success of the therapy.
It aims at restoring the blood supply to the liver. A chronic Budd- Chiari syndrome may require a lifelong therapy with blood thinners. The prognosis of the Budd- Chiari Syndrome, however, depends on the beginning and success of the therapy. If the Budd- Chiari Syndrome is not treated, in 90% of cases the survival time due to liver failure is limited to less than three years. If therapy is started early, up to 10% of patients die within the first five years.
Causes of a Budd- Chiari Syndrome
The Budd- Chiari syndrome is caused by a complete or incomplete occlusion of the laxative liver vessels. In most cases, the great hepatic vein is affected, which carries the blood from the liver to the heart through the inferior vena cava. The majority of the occlusion is caused by a thrombosis, i.e. a blood clot.
In this case, the person affected should subsequently be examined for (malignant) blood disorders associated with disturbed coagulation. This also includes myeloproliferative syndrome, which can be understood as a precursor to malignant blood cancer (leukaemia). It is one of the most frequent causes of the development of the Budd- Chiari Syndrome. Other blood diseases that increase the risk of Budd- Chiari Syndrome are factor V and thrombophilia.
Further questions
The Budd- Chiari Syndrome is mostly caused by the formation of a blood clot which closes the liver vein. This is not contagious. The risk factors of a Budd- Chiari syndrome (blood clotting disorders), however, can be inherited.
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