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Liver transplantation with caval thrombectomy and cavoatrial shunt in acute Budd‐Chiari syndrome

A 38-year-old woman was admitted to her local hospital with acute-onset upper abdominal pain and vomiting. Examination revealed mild jaundice, ascites, smooth, tender hepatomegaly, and bilateral lower limb edema. Liver function tests were abnormal, with increased serum bilirubin of 37 mol/L and aspartate transaminase of 515 IU/L. Abdominal ultrasound revealed a diffusely enlarged hypoechoic liver with thrombus in the inferior vena cava (IVC) extending to the hepatic veins, moderate ascites, and a patent portal vein. A procoagulant screen was normal, with the exception of lupus anticoagulant, which was increased at 1.46 (normal range 0.8-1.2). No anticoagulation therapy had been used. Abdominal computed tomographic scan confirmed the presence of luminal caval thrombus extending from the level of the bifurcation superiorly to the right atrium (RA) (Fig. 1). The patient was transferred to our liver intensive care unit for further management of acute Budd-Chiari syndrome. Her clinical condition deteriorated over several days, and she was listed for urgent liver transplantation. Intraoperative findings included moderate ascites, severe portal hypertension, and an enlarged, hard, congested liver. The IVC was thrombosed along its length from the iliac veins to the RA. Access for left axillary vein to portal vein venovenous bypass was prepared early. The femoral veins were not cannulated because of the presence of iliac thrombus. The supradiaphragmatic IVC was severely stenosed, with extensive hard caval thrombus. A midline sternotomy was performed, the RA cross-clamped just below the coronary sinus, and the stenotic IVC excised. The infrahepatic vena cava was thrombectomized with a Foley and a large Fogarty catheter, followed by retrograde infusion of 3 mg of recombinant tissue plasminogen activator. The liver was removed after clamping the infrahepatic vena cava. Because the donor heart had been retrieved leaving a very short suprahepatic cava, donor infrarenal IVC was used as an interpositional graft, with anastomosis to the RA (Fig. 2). The graft was implanted by first suturing the suprahepatic vena cava to the caval interpositional graft. The infrahepatic vena cava was re-bled, and a further thrombectomy performed. Portal, arterial, and