Good Clinical Outcomes in Budd–Chiari Syndrome with Hepatic Vein Occlusion

Abstract

Background

Budd–Chiari syndrome (BCS) with hepatic vein (HV) occlusion is manifested by severe liver damage in acute cases and esophageal variceal bleeding or refractory ascites in chronic cases, which is difficult to differentiate from cirrhotic portal hypertension.

Aims

To evaluate the clinical efficacy and safety of HV angioplasty and transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of BCS with HV occlusion.

Methods

Between May 1995 and December 2014, 60 patients with HV occlusive BCS underwent HV angioplasty or TIPS. BCS was subacute or chronic in 55 patients and acute in 5 patients. HV angioplasty was performed in 18 patients with HV occlusion, combined HV and IVC angioplasty in 9 patients with HV and IVC occlusion, TIPS in 12 patients with HV occlusion, and modified TIPS in 21 patients with extensive HV occlusion.

Results

The interventional procedure was successfully performed in all 60 patients. The portal pressure decreased from 41.23 ± 10.46 cmH₂O preoperatively to 26.68 ± 6.46 cmH₂O postoperatively, while the portal flow velocity increased from 14.31 ± 10.43 to 52.16 ± 13.68 cm/s in patients undergoing TIPS or modified TIPS. During hospitalization, two patients died from hepatic failure, and acute shunt occlusion occurred in two other patients during subsequent treatment with repeated intervention. During 82.25 ± 46.16 months of follow-up, three patients underwent re-intervention with a stenotic shunt, and other three with repeated dilation of the stenotic HV.

Conclusion

HV angioplasty and TIPS yield excellent long-term outcomes in patients with HV occlusive BCS.

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