後天性von Willebrand症候群が疑われた連合弁膜症

Combined Valvular Disease with Suspected Acquired von Willebrand Syndrome

重症大動脈弁狭窄症（AS）では，強いずり応力によりvon Willebrand因子（vWF）の切断が生じて後天性von Willebrand症候群（vWS）をきたす．われわれは弁膜症手術の周術期に，vWSと思われる著明な出血傾向を認めた症例を経験したので報告する．

A 73-year-old female was referred to our department for surgical treatment of combined rheumatic valvular disease. She had undergone percutaneous transluminal mitral commissurotomy 29 years before. She underwent double valve replacement with bioprosthetic valves and tricuspid annuloplasty combined with left atrial appendectomy for chronic atrial fibrillation. Because of remarkable bleeding following surgery, rapid blood transfusion and re-thoracotomy were immediately performed. However, the bleeding persisted even after re-thoracotomy, and acquired von Willebrand syndrome due to valvular disease was suspected. Von Willebrand factor/factor Ⅷ preparation was administered, and bleeding promptly became under control. Recently, acquired von Willebrand syndrome due to valvular disease has attracted attention. We need to keep it in mind during perioperative management for patients with valvular disease.