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Results of portal systemic shunts in Budd-Chiari syndrome
Insights
Portal systemic shunts effectively treat Budd-Chiari syndrome (BCS) with low risks. This procedure offers excellent long-term outcomes for BCS patients, preventing complications like encephalopathy.
Area of Science:
- Vascular Surgery
- Hepatology
- Gastroenterology
Background:
- Budd-Chiari syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction.
- Management of BCS often involves complex surgical interventions to restore portal flow and decompress the liver.
Purpose of the Study:
- To evaluate the efficacy and safety of portal systemic shunts in patients with Budd-Chiari syndrome.
- To assess different shunt configurations based on the specific venous obstruction patterns.
Main Methods:
- Nine patients with BCS underwent surgical treatment with portal systemic shunts.
- Interposition mesocaval shunts using autologous jugular vein were performed in seven patients.
- Specialized shunts (portoatrial, cavoatrial) were used for complex cases involving superior mesenteric vein thrombosis or inferior vena cava obstruction.
Main Results:
- No operative deaths or major postoperative complications were observed.
- All surviving patients showed significant improvement, with no instances of hepatic encephalopathy.
- Long-term follow-up (8 months to 6 years) demonstrated sustained patency and clinical well-being.
Conclusions:
- Portal systemic shunts are a safe and effective treatment for symptomatic Budd-Chiari syndrome.
- The mesocaval shunt is suitable for IVC stenosis, while cavoatrial shunts are reserved for complete IVC obstruction.
- Early intervention with portal systemic shunts offers an excellent long-term prognosis, barring concurrent malignancies.
Abstract:
Nine patients with Budd-Chiari syndrome (BCS) were treated by a portal systemic shunt. One had thrombosis of the superior mesenteric vein (SMV) and another had complete obstruction of the retrohepatic inferior vena cava (IVC). All other patients had a marked stenosis of the retrohepatic IVC with caval pressure ranging from 12 to 24 mmHg (mean: 17 mmHg). Seven patients had an interposition mesocaval shunt using an autologous jugular vein. The patient with a thrombosed SMV had a portoatrial shunt. The patient with an obstructed IVC had a cavoatrial shunt after an erroneous portacaval shunt had failed to relieve ascites. There were no operative deaths and no major postoperative complications. One patient died 19 months after operation of acute leukemia complicating polycythemia rubra vera. All other patients were alive and well 8 months to 6 years after operation. None of them had encephalopathy. These results suggest several comments: Portal systemic shunts are a good treatment for BCS and have a low operative risk. The mesocaval shunt is an efficient procedure, even when there is stenosis of the IVC with high caval pressure; shunts to the right atrium should be performed only in the case of complete obstruction or inaccessibility of the IVC. The long-term prognosis is excellent, except in patients with potential malignancies. Therefore, portal systemic shunts should be indicated early in patients with symptomatic BCS.