Antiphospholipid syndrome (APS) is a rare syndrome mainly characterized by several hypercoagulation disorders. Clinical findings of this syndrome include arterial or venous thrombosis, recurrent pulmonary embolism, valve diseases, intracardiac thrombus formations, pulmonary hypertension, coronary artery disease, and dilated cardiomyopathy. A definitive diagnosis can be made in a patient with history of arterial or venous thrombosis or recurrent abortions and positive for anticardiolipin antibodies, anti-beta 2-glycoprotein-1 antibodies -also known as antiphospholipid antibodies- and/or lupus anticoagulants. The incidence of thrombosis is highest during the following periods: 1) preoperative period due to the withdrawal of warfarin; 2) postoperative period due to potential hypercoagulability despite warfarin or heparin therapy; and 3) postoperative period before the start of adequate anticoagulation treatment. Irregular thickening of the valve leaflets due to deposition of immune complexes may lead to vegetation and valve dysfunction. The most commonly affected valve is the mitral valve, followed by the aortic and tricuspid valves. Anticoagulation dosage should be adjusted carefully during cardiopulmonary bypass due to the high risk of thrombosis in patients with APS. Multidisciplinary approaches are needed to reduce risk of bleeding and thrombosis during peri- and postoperative periods by adequate anticoagulation adjustment. Further prospective studies are required on anticoagulation adjustments in patients with APS during cardiovascular surgery.

Keywords : Antiphospholipid syndrome; cardiovascular surgery; intracardiac mass