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Orthotopic Liver Transplant for Budd-Chiari Syndrome: An Analysis of 14 Cases
Ahmet Bülent Doğrul1, Yücel Yankol, Nesimi Mecit
1From the Şişli Memorial Hospital, Solid Organ Transplantation Unit, Okmeydanı, Istanbul, Turkey.
Insights
Orthotopic liver transplant offers a life-saving option for Budd-Chiari syndrome patients. Living-donor liver transplants are a viable alternative, especially when organ donation is limited, with good survival rates.
Area of Science:
- Hepatology
- Transplant Surgery
- Vascular Surgery
Background:
- Budd-Chiari syndrome involves hepatic venous outflow obstruction, a critical condition requiring advanced treatment.
- Orthotopic liver transplantation (OLT) is a recognized intervention for both acute and chronic Budd-Chiari syndrome.
- This study reviews OLT outcomes in 14 patients with Budd-Chiari syndrome.
Discussion:
- The study included 14 patients (1 female) with a mean age of 33, predominantly with severe liver disease (9 Child-Pugh C).
- Common etiologies included Factor V Leiden mutation, polycythemia vera, and thrombotic disorders.
- A total of 15 OLTs were performed, with 10 utilizing living donors, highlighting its importance.
Key Insights:
- Patient survival rates at 1, 3, and 5 years post-transplant were 87%, 71%, and 71%, respectively.
- Living-donor liver transplantation (LDLT) demonstrated feasibility and success in this cohort.
- Identifying the underlying cause of Budd-Chiari syndrome is crucial for long-term management.
Outlook:
- OLT is a critical treatment for Budd-Chiari syndrome, particularly in fulminant cases and cirrhosis.
- LDLT presents a viable solution in regions with organ donor shortages.
- Long-term anticoagulation and antiplatelet therapy are essential components of post-transplant care.
Objectives:
Budd-Chiari syndrome is a low-prevalence, life-threatening disorder characterized by hepatic venous outflow obstruction at the hepatic venules, the large hepatic veins, the inferior vena cava, or the right atrium. Orthotopic liver transplant should be considered for patients with fulminant and chronic forms of the syndrome.
Materials And Methods:
Fourteen patients received 15 orthotropic liver transplants at our center from September 2006 to March 2013. This study retrospectively reviewed the prospectively collected data from these 14 patients.
Results:
The mean age of the patients was 33 years; only 1 patient was female. The severity of liver disease was Child-Pugh score A in 1 patient, B in 4 patients, and C in 9 patients. Mean calculated Model for End-Stage Liver Disease score was 18 (range, 6-30). The cause of Budd-Chiari syndrome was factor 5 Leiden mutation in 3 patients, polycythemia vera in 2 patients, factor 2 and 3 deficiency in 1 patient, fulminant essential thrombocytosis in 1 patient, and protein C deficiency in 2 patients. We performed 15 transplants in 14 patients. Five grafts were obtained from deceased donors, and 10 grafts were from living-related donors. Mean graft-to-recipient weight ratio was 1,12 for patients receiving a living-donor liver transplant. Median follow-up was 29 months. Patient survival rates were 87%, 71%, and 71% at 1, 3, and 5 years.
Conclusions:
Liver transplant is an option for treating Budd-Chiari syndrome in cases of fulminant presentation and cirrhosis. Living-donor liver transplant is a viable choice in countries where procuring organ donations is still a problem. To manage the long-term medical therapy and follow-up for these patients, a careful evaluation is necessary to determine the cause of Budd-Chiari syndrome. Anticoagulant and antiaggregant therapy remains the mainstay of treatment for this syndrome.
