Abstract
INTRODUCTION: Coagulation disorders can lead to massive perioperative bleeding regardless of the type of surgery. Their preoperative identification is essential (from a complete history of bleeding tendency) and steps should be taken to mitigate such complications at the time of surgery. Alpha(2)-plasmin inhibitor (α2-PI) deficiency is a rare congenital coagulation disorder resulting in activation of fibrinolysis and requiring specific treatment with antifibrinolytic agents. Lung transplantation has not been previously reported in a patient with α2-PI deficiency. CASE PRESENTATION: A 46-year-old female affected by chronic obstructive pulmonary disease with congenital α2-PI deficiency was referred to our hospital for cadaveric lung transplantation. Due to a previous history of intramedullary hemorrhage, we conducted lung transplantation with prophylactic administration of fresh frozen plasma (FFP) and tranexamic acid during surgery. We used the point of care test (POC) rotational thromboelastometry (ROTEM) to diagnose intraoperative coagulopathy. The postoperative course was uneventful, and she was discharged from the hospital 42 days after lung transplantation. Six months have passed since transplant, and she is still attending outpatient clinics in good health and with no record of bleeding episodes. CONCLUSIONS: Lung transplantation for a patient with α2-PI deficiency was safely performed with the use of planned FFP transfusion and tranexamic acid. A POC ROTEM testing approach to perioperative management was useful during lung transplantation.