ABSTRACT
A 72-year-old female was diagnosed with systemic lupus erythematosus and antiphospholipid syndrome (APS) in 2014 and was followed up. Severe mitral regurgitation coexisted with APS, but the case was nonsymptomatic, and surgery involved high risk. Therefore, the physicians continued their observation. In 2020, the patient experienced rheumatic severe mitral stenosis and shortness of breath on exertion. Paroxysmal atrial fibrillation and coronary stenosis were also detected. Therefore, we planned mitral valve replacement, tricuspid annuloplasty, coronary artery bypass, pulmonary vein isolation and left atrial appendage closure. During extracorporeal circulation (ECC), we performed coagulation management based on blood heparin concentration using HMS PLUS. Because the APS patient showed prolonged activated clotting time (ACT), and coagulation therapy based on ACT is unreliable. She was discharged from our hospital on postoperative day 23. No complications, including bleeding and thrombosis, were observed 2 years after the operation. We experienced a case of APS who underwent cardiac surgery and performed coagulation management by measuring heparin concentration during ECC. We targeted a 3.5 U/ml heparin concentration, and her clinical course was uneventful.