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.

Popliteal Artery Pseudoaneurysm Associated with Osteochondroma / 日本心臓血管外科学会雑誌

Osteochondromas, or exostoses, are the most frequent benign bone tumors. Although many osteochondromas are asymptomatic, they are sometimes responsible for vascular complications, most often associated with the popliteal artery. Here, we present a rare case of pseudo-aneurysm of the popliteal artery secondary to an osteochondroma of the femur in a 48-year-old woman. During surgery, a pseudo-aneurysm developed from a 1-mm hole in the anterior aspect of the popliteal artery, which was closely related to the protrusion of the femoral osteochondroma. The surface of the osteochondroma was quite sharp, rigid and firm. It was removed completely through the lumen of the aneurysm. A short segment of the popliteal artery, including the hole, was resected with an end-to-end anastomosis. The postoperative course was uneventful, and the patient recovered completely. The precise pathogenesis of pseudo-aneurysmal formation is still unknown. We speculate that local compression of the popliteal artery by a spiky osteochondroma can stretch the vessel and lead to rupture of the artery by continuous friction. Considering the potential risk of this vascular complication, shonld be closely monitered patients with osteochondroma of the femur.

Stanford Type A Acute Aortic Dissection with Left Extra-pleural Hematoma and Lung Hemorrhage / 日本心臓血管外科学会雑誌

A 68-year-old woman with a sudden onset of back pain was brought to our hospital by ambulance. Computed tomography (CT) showed Stanford type A (DeBakey type II) acute aortic dissection, left hemothorax, and hematoma extending along the pulmonary artery ; therefore, the patient underwent emergency operation. We performed a median sternotomy. Pericardial effusion was not observed ; however, a hematoma was found around the ascending aorta. Preoperative CT showed left hemothorax, but pleural effusion was not observed in the left pleural cavity. The left hemothorax, which was detected on preoperative CT, was diagnosed as an extrapleural hematoma. The dissection entry site was located at the proximal aortic arch ; therefore, ascending aorta-hemiarch replacement was performed. After weaning from cardiopulmonary bypass, the patient experienced sudden airway bleeding. The bleeding was attributed to the hematoma extending along the pulmonary artery. Here, we have reported a rare case of Stanford type A acute aortic dissection with the left extrapleural hematoma and lung hemorrhage.