Abstract Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) provides temporary mechanical circulatory support for circulatory failure. It carries a high complication and mortality rate, with thrombosis being one of the most common. Antiphospholipid syndrome is a systemic autoimmune disorder characterized by arterial, venous, or microvascular thrombosis. Its most rare and life-threatening variant is known as catastrophic antiphospholipid syndrome (CAPS), in which patients rapidly develop widespread thrombosis leading to multiorgan failure and death. Fortunately, CAPS has an incidence of less than 1% in patients with a known history of antiphospholipid syndrome, and there have been only 805 cases of CAPS reported in the medical literature. This report reviews the case of a 42-year-old female supported with central VA-ECMO after undergoing high-risk aortic and mitral valve replacement. Although successfully decannulated, she rapidly developed superior vena cava syndrome (SVC) secondary to CAPS. Patient is a 42-year-old female with a history of antiphospholipid syndrome on warfarin and bridged with heparin, mitral valve replacement secondary to endocarditis, and chronic kidney disease. There was no complete APS profile available to risk-stratify prior to surgery. She underwent a high-risk mitral and aortic valve replacement that necessitated VA-ECMO, inhaled nitric oxide, epinephrine, milrinone, norepinephrine, and vasopressin. Her Swan-Ganz hemodynamics revealed CVP 24, PAP 39/20, and CI 2.5. Blood gas showed pH 7.374, PCO2 42.8, PO2 48.5, and HCO3 25. Inotropic agents were subsequently down-titrated due to excessive support increasing native cardiac output and causing north-south syndrome. Despite ten liters removed by CRRT and the discontinuation of inotropes, her CVP remained elevated. Heparin was held due to elevated aPTT and severe thrombocytopenia. After decannulation, aspirin was added to balance thrombotic risk against bleeding. Suddenly, her dialysis catheter and other central venous lines clotted with facial swelling. All lines were replaced despite the presence of microvascular clots, and the new left femoral Swan-Ganz revealed CVP 15 and PAP 40/20. Imaging confirmed a complete lack of flow through the left brachiocephalic vein. Bivalirudin, plasma exchange, methylprednisolone, and IVIG were initiated. Rituximab was administered for refractory CAPS. Bivalirudin was later changed to heparin with Anti-Xa monitoring. The patient continued to decline, and the family opted for compassionate withdrawal. This case details the importance of appropriate anticoagulation strategies in the setting of CAPS. The patient was not adequately anticoagulated, as the patient’s aPTT was falsely elevated secondary to CAPS antibodies. Titration should be based on anti-Xa. Failure to diagnose can lead to a catastrophic event. This abstract is funded by: None
Moriles et al. (2026) studied this question.