title: Refractory hypoxemia in a 23-year-old patient with Budd-Chiari syndrome.
abstract: Antiphospholipid syndrome is an autoimmune disorder characterized by a hypercoagulable state, leading to arterial and venous thrombosis. We present a 23-year-old patient, suspected of having Budd-Chiari syndrome due to antiphospholipid syndrome, who developed severe and progressive hypoxemia, requiring prolonged mechanical ventilation. After a detailed but unsuccessful workup, a contrast CT scan revealed an occluded superior vena cava and azygos vein-superior vena cava junction and massive right-to-left shunting through a network of systemic to pulmonary venous collaterals. Restoring normal blood flow from the azygos vein into the right atrium by stenting the azygos-superior vena cava junction resolved the hypoxemia immediately. Within the same procedure, the hepatic outflow obstruction was successfully treated by stenting a severe stenosis of the suprahepatic inferior vena cava caused by calcified thrombus.
authors.affiliation: From the Department of Intensive Care, Erasmus Medical Centre University Hospital, Rotterdam, The Netherlands.
authors.forename: Jeroen J H
authors.initials: JJH
authors.lastname: Bunge
authors.affiliation: Department of Gastroenterology and Hepatology, Erasmus Medical Centre University Hospital, Rotterdam, The Netherlands.
authors.forename: Ubbo S
authors.initials: US
authors.lastname: Wiersema
authors.affiliation: Department of Radiology, Erasmus Medical Centre University Hospital, Rotterdam, The Netherlands.
authors.forename: Adriaan
authors.initials: A
authors.lastname: Moelker
authors.affiliation: From the Department of Intensive Care, Erasmus Medical Centre University Hospital, Rotterdam, The Netherlands.
authors.forename: Jasper
authors.initials: J
authors.lastname: van Bommel
authors.affiliation: Department of Gastroenterology and Hepatology, Erasmus Medical Centre University Hospital, Rotterdam, The Netherlands. Electronic address: etjwa@erasmusmc.nl.
authors.forename: Eric T T L
authors.initials: ETTL
authors.lastname: Tjwa
mesh_terms: D000792:Angiography; D019917:Blood Vessel Prosthesis Implantation / Q000379:methods*; D006502:Budd-Chiari Syndrome / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D000860:Hypoxia / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D015607:Stents; D014057:Tomography, X-Ray Computed; D014683:Vena Cava, Superior / Q000000981:diagnostic imaging / Q000601:surgery*; D055815:Young Adult