Budd–Chiari syndrome

Budd–Chiari syndrome is obstruction of hepatic venous outflow, usually from hepatic veins or the inferior vena cava. It causes blood to pool in the liver and can lead to liver injury, portal hypertension, and liver failure.

Hepatic veins: Veins that drain blood from the liver into the inferior vena cava. Their obstruction is the most common anatomical basis of the syndrome.
Thrombophilia: An increased tendency to form blood clots. Underlying clotting disorders account for many cases of hepatic vein thrombosis.
Doppler ultrasonography: An imaging method that uses reflected sound waves to assess blood flow and vessel structure. It is commonly used first to detect abnormal or absent flow in hepatic veins.
Sinusoidal obstruction syndrome: Liver injury caused by narrowing or blockage of small hepatic venules and sinusoids. It can resemble Budd–Chiari syndrome but obstructs smaller vessels within the liver.
Inferior vena cava: The large vein that returns blood from the lower body to the heart. Obstruction where hepatic veins enter it can also block liver outflow.
Myeloproliferative neoplasm: A group of bone marrow cancers that produce excess blood cells or platelets. These disorders are a major, sometimes initially hidden, risk factor for hepatic vein clots.
Computed tomography: An imaging technique that combines X-ray measurements to create cross-sectional images. Contrast-enhanced scans can map venous obstruction and changes in liver perfusion.
Congestive hepatopathy: Liver damage caused by chronically elevated pressure in the hepatic veins, often from right-sided heart disease. It produces hepatic congestion through backward pressure rather than primary hepatic-vein obstruction.
Portal hypertension: Abnormally high blood pressure in the portal venous system. Impaired hepatic drainage raises resistance and can transmit pressure into portal circulation.
JAK2 V617F: A mutation in the JAK2 gene that drives abnormal blood-cell production in several myeloproliferative neoplasms. Testing for this mutation can reveal a clotting predisposition behind the syndrome.

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