A Case Report of Aortic Valve Replacement for a Patient with von Willebrand Disease / 日本心臓血管外科学会雑誌

We report a 30-year-old patient with von Willebrand disease who received AVR under cardio-pulmonary bypass. AR was diagnosed at the age of 13, and von Willebrand disease was revealed after cardiac catheterization because of a bleeding episode. His von Willebrand factor (vWF) activity was significantly low, 43% of normal. Infusion of vWF concentrates (Confact F^®) was administered before surgery. AVR was safely performed and no bleeding complications occurred during the perioperative period. Blood transfusion was unnecessary, vWF infusion was considered to be very useful.

A Rare Case of Leiomyosarcoma Originating from the Abdominal Aorta / 日本心臓血管外科学会雑誌

We report an extremely rare case of leiomyosarcoma originating from the abdominal aorta. The patient was a 57-year-old man who had palpable abdominal mass with pain. The symptoms were consistent, and urgent operation was done due to impending rupture of the abdominal aortic aneurysm. The intraoperative findings showed that the mass was a primary tumor of the abdominal aorta, and the histological diagnosis was leiomyosarcoma. It is reported that its prognosis is very poor, but he survived 7.5 years after diagnosis by reason of aggressive management including surgical treatment, chemotherapy and radiotherapy.