Management Strategies for Budd-Chiari Syndrome

Budd–Chiari syndrome is a rare hepatic vascular disorder marked by obstruction of hepatic venous outflow, leading to elevated sinusoidal pressure, hepatomegaly, ascites and progressive liver dysfunction. Management follows a stepwise algorithm that begins with identification and correction of underlying prothrombotic conditions and the initiation of anticoagulation therapy in all patients. Where medical treatment alone fails to restore adequate venous drainage, endovascular interventions such as percutaneous transluminal angioplasty, stenting and transjugular intrahepatic portosystemic shunt (TIPS) are employed to decompress the liver and re-establish flow. In refractory cases or those with fulminant hepatic failure, orthotopic liver transplantation remains the definitive rescue therapy. Contemporary strategies emphasise multidisciplinary assessment, incorporating advanced imaging techniques for precise characterisation of vascular lesions, risk stratification to guide timing and choice of intervention, and long-term surveillance for complications such as portal hypertension and hepatocellular carcinoma. Tailored approaches consider regional aetiological differences, notably the predominance of myeloproliferative disorders in certain populations, and seek to balance procedural risk against the potential for durable symptom relief and transplant-free survival.

Research from Nature Portfolio

Recent studies have demonstrated that transjugular intrahepatic portosystemic shunt offers a safe and effective option for patients with diffuse hepatic vein occlusion. Technical success rates approach 100 per cent, with significant reduction in portosystemic pressure gradients and rapid improvement in bilirubin levels and liver function tests. Long-term follow-up in the treated cohort shows markedly lower mortality compared with patients managed without shunt creation, underscoring the value of timely endovascular decompression in both acute and subacute presentations.

Management Strategies for Budd-Chiari Syndrome publication trend

The graph below shows the total number of articles in management strategies for budd-chiari syndrome across all publications each year (not limited to Nature Index journals).

Technical terms

Budd–Chiari syndrome: Obstruction of hepatic venous outflow at the level of the hepatic veins or inferior vena cava leading to liver congestion and portal hypertension.

Transjugular intrahepatic portosystemic shunt (TIPS): A radiological procedure creating a channel between the portal and systemic venous systems through the liver to decompress portal hypertension.

Percutaneous transluminal angioplasty: Endovascular dilation of a stenosed or occluded vein using a balloon catheter to restore vessel patency.

Stent placement: Deployment of a mesh tube within a blood vessel to maintain luminal integrity after angioplasty.

Orthotopic liver transplantation (OLT): Surgical replacement of a failing liver with a donor organ, reserved for patients with irreversible hepatic failure or refractory complications.

Hypercoagulable states: Conditions in which there is an increased tendency for blood clot formation, often underlying the thrombotic basis of Budd–Chiari syndrome.

References

1. Budd-Chiari Syndrome. Prague Medical Report (2017).
2. Budd-Chiari Syndrome Imaging Diagnosis: State of the Art and Future Perspectives. Diagnostics (2023).
3. Transjugular intrahepatic portosystemic shunt for Budd–Chiari syndrome with diffuse occlusion of hepatic veins. Scientific Reports (2016).
4. Budd‐Chiari Syndrome in China: A Systematic Analysis of Epidemiological Features Based on the Chinese Literature Survey. Gastroenterology Research and Practice (2015).
5. Excellent long-term outcomes of endovascular treatment in budd–chiari syndrome with hepatic veins involvement. Medicine (2018).
6. Treatment of Budd-Chiari syndrome with a focus on transjugular intrahepatic portosystemic shunt. World Journal of Hepatology (2013).