Estrategias de Perfusión en enfermedades Raras: Síndrome antifosfolípido.

Introduction: The antiphospholipid syndrome is considered as rare diseases, it is an autoimmune disease characterized by the formation of thrombus. Among the criteria for establishing the diagnosis are: venous and arterial thrombosis, recurrent pregnancy loss and the presence of antiphospholipid antibodies. Objectives: The treatment of patients with antiphospholipid syndrome in cardiac surgery with extracorporeal circulation represents a challenge in terms of anticoagulation strategy since this syndrome, although associated with thrombocytopenia and deficiencies of coagulation factors, is characterized by the formation of thrombus against triggering situations such as the extracorporeal circuit among others; anticoagulation and monitoring becomes complex since the coagulation cascade is altered at multiple points. Methods: We present a case of a 30-year-old patient with antiphospholipid syndrome, endocarditis of the aortic valve, previous cerebrovascular accident. Plan: Aortic valve replacement. Heparinization was administered prior, postoperatively and in extracorporeal circulation accompanied by constant monitoring in the transoperative period with activated clotting time and synergistically with thromboelastogram. Results: A higher concentration of heparin than usual was necessary in extracorporeal circulation, achieving a reversal of heparin with unusual minimum doses of protamine sulfate. Conclusion: Preparation and consultation guided the strategies adopted in the conduct of perfusion to achieve optimal management of anticoagulation during cardiovascular surgery in this challenging pathology in which the surgical team, especially the perfusionist, may be faced.