ABSTRACT
Primary antiphospholipid syndrome is an autoimmune coagulation disorder associated with recurrent arterial and/or venous thrombotic events and the presence of antiphospholipid antibodies. Echocardiography studies have disclosed heart valve abnormalities in about third of patients with primary antiphospholipid syndrome. Valvular lesions associated with antiphospholipid antibodies occur as valve masses [nonbacterial vegetations] or thickening. Both can be associated with valve dysfunction. The predominant functional abnormality is regurgitation, stenosis is rare. The mitral valve is mainly affected, followed by the aortic valve. Antiphospholipid antibodies often inhibit phospholipids dependent coagulation in vitro and interfere with laboratory testing of hemostasis. Therefore, the management of anticoagulation during cardiopulmonary bypass can be quite challenging for those patients. Here, we present a case of mitral valve replacement due to severe mitral regurgitation in a young patient with primary antiphospholipid syndrome