Budd‐Chiari syndrome

Abstract

Budd-Chiari syndrome (BCS) is a rare and potentially life-threatening disorder characterized by obstruction of the hepatic outflow tract at any level between the junction of the inferior vena cava with the right atrium and the small hepatic veins. In the West, BCS is a rare hepatic manifestation of one or more underlying prothrombotic risk factors. The most common underlying prothrombotic risk factor is a myeloproliferative disorder, although it is now recognized that almost half of patients have multiple underlying prothrombotic risk factors. Clinical manifestations can be diverse, making BCS a possible differential diagnosis of many acute and chronic liver diseases. The index of suspicion should be very low if there is a known underlying prothrombotic risk factor and new onset of liver disease. Doppler ultrasound is sufficient for confirming the diagnosis, although tomographic imaging (computed tomography (CT) or magnetic resonance imaging (MRI)) is often necessary for further treatment and discussion with a multidisciplinary team. Anticoagulation is the cornerstone of the treatment. Despite the use of anticoagulation, the majority of patients need additional (more invasive) treatment strategies. Algorithms consisting of local angioplasty, TIPS and liver transplantation have been proposed, with treatment choice dictated by a lack of response to a less-invasive treatment regimen. The application of these treatment strategies allows for a five-year survival rate of 90%. In the long term the disease course of BCS can sometimes be complicated by recurrence, progression of the underlying myeloproliferative disorder, or development of post-transplant lymphoma in transplant patients.

Dabigatran as an oral anticoagulant in patients with Budd–Chiari syndrome post‐percutaneous endovascular intervention

“…Eight (8.1%) patients developed restenosis post‐endovascular intervention over a median interval of 5 months . Of these, four (11%) were on dabigatran, and four (6%) were on VKAs ( P = 0.912).…”

“…Budd–Chiari syndrome (BCS) or hepatic venous outflow obstruction is characterized by obstruction anywhere from the hepatic veins (HVs) to inferior vena cava (IVC) outflow . Management of BCS includes four successive steps: (i) anticoagulation, (ii) percutaneous interventions including angioplasty and stenting, (iii) transjugular intrahepatic portosystemic shunt (TIPS), and (iv) liver transplant . One key aspect involved in management includes anticoagulants.…”

A young male with liver cirrhosis and portal hypertension found to have chronic idiopathic Budd Chiari syndrome

“…Doppler ultrasonography is usually sufficient to confirm the diagnosis, although tomographic imaging (CT) or magnetic resonance imaging (MRI) is often necessary for further [8]. Myeloproliferative neoplasms should be actively screened for even when a clear causative factor has been identified [4].…”

Management of portal hypertension and ascites in polycystic liver disease

“…Hepatic venous outflow obstruction is characterized by reduction in the outflow of venous blood from the liver into the caval vein (Figure ). HVOO is a rare condition, which can either be caused by (I) hepatic vein thrombosis in Budd‐Chiari Syndrome (BCS), (II) external compression by tumour, cyst or abscess, or (III) after liver transplantation …”

“…HVOO is a rare condition, which can either be caused by (I) hepatic vein thrombosis in Budd-Chiari Syndrome (BCS), (II) external compression by tumour, cyst or abscess, or (III) after liver transplantation. 7,8 Portal vein obstruction is characterized by obstruction of inflow from the portal venous system into the liver. This may be because of external compression, but also by portal vein thrombosis induced by disruption of portal vein inflow and stagnant blood flow because of mechanical effects from compression.…”