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.

Three Cases of Graft Replacement of Distal Arch Aneurysm after Open-Stent Graft Technique due to Stent Migration and Endoleak / 日本心臓血管外科学会雑誌

Exposure of the surgical field and bleeding control are main problems of distal anastomosis during an operation for distal arch aneurysms. The open-stent technique and thoracic endovascular aortic repair (TEVAR) are useful techniques for the resolution of these problems. Recently, TEVAR has progressively expanded in the treatment of various complex thoracic aortic diseases. However, complications such as endoleaks and graft migrations have still remained an issue. Although some patients who have late distal endoleaks can be almost treated successfully with additional TEVAR, some of them cannot. We report 3 cases of graft replacement of descending aorta after open-stent technique due to stent migrations and endoleaks. All of them were previously performed by total arch replacement with open-stent technique for distal aortic arch aneurysms. The follow-up CT after the first operation revealed graft migrations and endoleaks. The open surgical repairs through left lateral thoracotomy were performed, followed by graft replacements. The stent grafts were easily clamped after the incision of the aneurysm. In 2 cases, grafts were directly anastomosed to the descending aorta after the removal of the stent. In 1 case, graft was extended with new graft and then anastomosed to the descending aorta. These procedures were technically successful ; there were no trouble to exfoliate aorta, to perform anastomosis and hemostasis, and neither patient developed major complications. These results indicate that open surgical repair of descending aorta could be one of the safety options for the treatment of endoleaks and stent migrations of thoracic aortic stent graft in the era of increasing endovascular therapy.

Emergency Redo Aortic Root Replacement for Composite Graft Dehiscence due to Aortitis Syndrome in a Child / 日本心臓血管外科学会雑誌

We report a case of 10-year-old woman with aortitis syndrome who had a graft dehiscence at the site of proximal anastomosis 8 months after aortic root replacement. Because she suffered severe chest compression and ST depression was demonstrated on 12 lead ECG, she was admitted on a suspicion of vasospasmic angina. However, transesophageal echocardiogram and CT showed an echo-free space around the previous operated aortic composite graft, so we concluded that a proximal graft dehiscence and bleeding around it was the cause of her severely deteriorated circulatory condition, and emergency redo aortic replacement was planned. After deep hypothermic circulatory arrest was accomplished, selective cerebral perfusion was performed following re-sternotomy. Previous composite graft was detached at the site of proximal anastomosis, and the aortic annulus was friable and edematous. Redo aortic replacement successful. Laboratory findings revealed uncontrollable aortitis syndrome as the etiologic factor of graft dehiscence. Postoperatively, she was complicated with cerebral infarction due to a stuck valve. She was discharged at 56 postoperative day.