ABSTRACT
We describe a case of cardiac surgery for a patient with CD36 deficiency. A 56-year old man had progressive dyspnea on effort for 1 year. On admission, echocardiography revealed severe aortic valve regurgitation. He required medical treatment for heart failure, and subsequently elective aortic valve replacement was planned. Pre-operative cardiac scintigraphy (<sup>123</sup>I-BMIPP) showed total defect of myocardial uptake. CD 36 deficiency was diagnosed based on the characteristic findings. CD 36 deficiency could cause transfusion related complication by donor blood transfusion. We prepared 1,200 ml autologous blood preoperatively in a two week period. The operation was performed successfully without donor blood transfusion. He was discharged uneventfully.