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.,
Chest: the cardiopulmonary and critical care journal
Department of Radiology

Bunge, J. J. H., Wiersema, U. S., Moelker, A., van Bommel, J., & Tjwa, E. (2014). Refractory hypoxemia in a 23-year-old patient with budd-chiari syndrome. Chest: the cardiopulmonary and critical care journal, 146(5), e149–e152. doi:10.1378/chest.13-2879