2025 Volume 54 Issue 2 Pages 78-81
MYH9 disorders are autosomal dominant disorders characterized by thrombocytopenia with giant platelets and leukocyte inclusions. This disease is the most frequent of the congenital macrothrombocytopenia, estimated at about 1 in 100,000. We performed double valve replacement for aortic valve stenosis and mitral valve regurgitation with MYH9 disorder without hemorrhagic complications or infections. A 78-year-old woman was on maintenance hemodialysis, and had no particular subjective symptoms. Chest X-ray showed cardiomegaly. Echocardiography revealed aortic valve stenosis and mitral valve regurgitation. Surgery was performed with perioperative platelet transfusion for thrombocytopenia. To reduce operative time and blood loss, we chose valve replacement instead of valvuloplasty for the mitral valve, and performed double valve replacement of the aortic valve and mitral valve. The postoperative course was good without recognizing hemorrhagic complication and infectious disease in the perioperative period.
