title: Direct intrahepatic portocaval shunt for treatment of portal thrombosis and Budd-Chiari syndrome.
abstract: Budd-Chiari syndrome (BCS) refers to hepatic venous outflow obstruction that in severe cases can lead to acute liver failure prompting consideration of revascularization or transplantation. Here, a 22 year old female with angiographically proven BCS secondary to JAK2/V617F positive Polycythemia vera on therapeutic warfarin presented with acute liver failure (ALF). Imaging revealed a new, near complete thrombotic occlusion of the main portal vein with extension into the superior mesenteric vein. An emergent direct intrahepatic portocaval shunt (DIPS) was created and liver function promptly normalized. She has been maintained on rivaroxaban since that time. Serial assessment over 1 year demonstrated continued shunt patency and improved flow in the mesenteric vasculature on ultrasound as well as normal liver function. DIPS is a viable alternative in the treatment of ALF from BCS when standard recanalization is not feasible. Improved blood flow may also improve portal/mesenteric clot burden. While further investigation is needed, new targeted anticoagulants may be viable as a long term anticoagulation strategy.
authors.affiliation: Department of Internal Medicine, Phoenix, AZ, USA.
authors.forename: Mark R
authors.initials: MR
authors.lastname: Pedersen
authors.affiliation: Department of Pathology, , Phoenix, AZ, USA.
authors.forename: Peter
authors.initials: P
authors.lastname: Molloy
authors.affiliation: Department of Interventional Radiology, Phoenix, AZ, USA.
authors.forename: David
authors.initials: D
authors.lastname: Wood
authors.affiliation: Transplant Center and Advanced Liver Diseases, Banner University Medical Center, Phoenix, AZ, USA.
authors.forename: Anil
authors.initials: A
authors.lastname: Seetharam
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D001706:Biopsy; D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D057915:Drug Substitution; D005260:Female; D006801:Humans; D019934:International Normalized Ratio; D053614:Janus Kinase 2 / Q000235:genetics; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D009154:Mutation; D010690:Phlebography; D011087:Polycythemia Vera / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D011167:Portacaval Shunt, Surgical; D011169:Portal Vein / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D000069552:Rivaroxaban / Q000627:therapeutic use; D016896:Treatment Outcome; D014654:Vascular Patency; D020246:Venous Thrombosis / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D014859:Warfarin / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000925:Anticoagulants; D014859:Warfarin; D000069552:Rivaroxaban; C507924:JAK2 protein, human; D053614:Janus Kinase 2