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Arterial and portal circulation and parenchymal changes in Budd-Chiari syndrome: a study in 17 explanted livers
Dominique Cazals-Hatem1, Valérie Vilgrain, Pascal Genin
1Service d'Anatomie-Pathologique, Laboratoire d'Hématologie et d'Immunologie, Hôpital Beaujon, Clichy, France. dominique.cazals-hatem@bjn.ap-hop-paris.fr
Insights
Budd-Chiari syndrome (BCS) involves impaired liver blood flow, leading to nodular regenerative hyperplasia or infarcts. Compensatory arterial hyperemia in chronic BCS promotes FNH-like nodules, impacting patient outcomes.
Area of Science:
- Hepatology
- Vascular Biology
- Transplant Surgery
Background:
- Budd-Chiari syndrome (BCS) pathogenesis involves hepatic vein occlusion and altered blood flow.
- Understanding parenchymal changes in BCS is crucial for managing liver transplantation outcomes.
Purpose of the Study:
- To correlate pretransplant clinical course and vascular imaging findings with histopathologic features in livers explanted for severe Budd-Chiari syndrome.
Main Methods:
- Retrospective analysis of 17 patients undergoing liver transplantation for severe classic BCS.
- Review of pretransplant clinical data, vascular imaging (portal and arterial perfusion), and explanted liver histology.
Main Results:
- All patients showed obstructive portal venopathy and nodular regenerative hyperplasia (NRH); 9 had FNH-like nodules, 2 had cirrhosis.
- Decreased portal perfusion was universal (94%), while increased arterial perfusion occurred in 9 patients.
- Increased arterial inflow correlated with FNH-like nodules and protracted pretransplant course; acute portal thrombi correlated with infarcts and shorter courses.
Conclusions:
- Severe BCS is characterized by impaired hepatic perfusion inflow, independent of cirrhosis progression, influencing outcomes.
- Early portal hypoperfusion leads to NRH or infarcts, while chronic BCS exhibits compensatory arterial hyperemia, fostering FNH-like nodules.
Abstract:
Hepatic parenchymal changes associated with Budd-Chiari syndrome (BCS) have been tentatively explained by combined arterial and portal perfusion disturbances in addition to the complete occlusion of hepatic veins. The aim of this study was to correlate pretransplant course and vascular imaging with pathologic findings in livers explanted for BCS. Seventeen consecutive white patients who underwent transplantation for severe classic BCS were retrospectively analyzed. Pretransplant course was 1 year or less in 8 patients and more than 1 year in 9 patients. Thrombophilia was found in 16 patients (94%). Imaging showed decreased portal perfusion in 16 patients (94%) and increased arterial perfusion in 9 patients. Histology showed obstructive portal venopathy and nodular regenerative hyperplasia (NRH) aspects in all cases, large regenerative nodules resembling focal nodular hyperplasia (FNH) in 9 cases, and cirrhosis in 2 cases. Patients with increased arterial inflow had large regenerative nodules and a protracted pretransplant course. Patients with acute thrombi in portal veins had parenchymal infarcts (2 cases) and a short pretransplant course. In conclusion, patients with severe BCS have a constant impaired perfusion inflow unrelated to progression of cirrhosis but related to the outcome. An early decrease in portal perfusion is observed in the short term and is responsible for NRH or infarcts if complicated with large thrombi. An increase in arterial perfusion compensates impaired portal flow in chronic BCS. Arterial hyperemia contributes to the development of large regenerative nodules that are FNH-like. This pathologic situation offers an interesting vascular model to further understand the parenchymal response to changes in hepatic blood flow.