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

Abstract

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 μmol/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. Sagittal contrast-enhanced abdominal computed tomographic scan demonstrating extensive caval thrombus extending to right atrium. IVC, inferior vena cava; RA, right atrium. 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 biliary anastomoses were completed in standard fashion. Postoperative recovery was complicated by renal failure and sepsis. The patient is currently well 1 year after transplantation and has patent cava and hepatic veins (Fig. 3). An intraoperative photograph showing vascular anastomosis: an interpositional donor infrarenal caval graft (thin black arrow) was anastomosed to the right atrium (fat black arrow) after excision of the supradiaphgramatic recipient inferior vena cava (IVC). Thrombectomy of the recipient intrahepatic IVC (thin white arrow) was performed on portoaxillary bypass (fat white arrow). Reconstructed magnetic resonance angiogram showing patent cava and hepatic veins. Budd-Chiari syndrome is a heterogenous group of disorders characterized by obstruction of hepatic venous outflow.1 Clinical presentation depends on the extent of hepatic venous outflow occlusion with fulminant, acute, subacute, and chronic forms. The optimal mode of management is determined by the site and extent of venous occlusion.2, 3 In fulminant Budd-Chiari syndrome, liver transplantation is the treatment of choice.4 Direct side-to-side portocaval shunt has been successfully used when performed early in the course of Budd-Chiari syndrome where occlusion is confined to the hepatic veins.5 In the presence of IVC thrombosis, the use of mesoatrial shunts have been associated with a high incidence of graft thrombosis and death in some series.5-8 Portal decompression combined with bypassing the obstructed IVC with side-to-side portocaval and cavoatrial shunts with synthetic graft has also resulted in long-term survival.5 In the present case, the acute mode of presentation with progressive clinical deterioration was treated with liver transplantation. Attempts at radiological intervention in the form of caval thrombolysis, or angioplasty and stenting were deemed inappropriate because of the presence of fulminant liver failure. Extensive caval thrombolysis and thrombectomy were performed at the time of transplant with excision of the recipient infra- and suprahepatic IVC, and placement of an interposition donor infrarenal IVC graft between the donor cava and the recipient RA. In previously reported cases with IVC stricture or thrombosis extending to the RA that were undergoing orthotopic liver transplantation, direct atriocaval anastomosis has been described.9, 10