Inferior Vena Cava-Atrial Anastomosis in Liver Transplant Recipient with Inferior Vena Cava Occlusion: A Case Report and Literature Review

Abstract

A 25-year-old woman with decompensated liver cirrhosis and complete inferior vena cava (IVC) occlusion was referred to our department for liver transplantation. The etiology of cirrhosis was Budd-Chiari syndrome (BCS) related to systemic lupus erythematosus, autoimmune hepatitis, and primary biliary cholangitis (AIH-PBC) overlap syndrome. Transplantation was feasible due to an extensive collateral circulation of pre-vertebral veins that drained blood from the lower extremities and both kidneys to the azygos-hemiazygos veins. This venous anomaly enabled the excision of the obstructed retrohepatic IVC, followed by an alternative anastomosis of the suprahepatic IVC to the right atrium without reconstruction of the infrahepatic IVC. Despite good venous patency and normalization of liver graft function, the patient developed cecum perforation, cardiovascular and respiratory insufficiency, which led to the patient’s death two months after transplantation. This case report supports an individual approach and highlights the feasibility of liver transplantation despite an extensive IVC thrombosis. To our knowledge, it is the first description of the application of a deceased donor liver transplantation in patients with AIH-PBC overlap syndrome and lupus-related BCS. A concise review of published literature on IVC-atrial anastomosis in adult liver transplant recipients is provided, and the technique is discussed based on our recent experience.