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.

Left Atrial Aneurysm Complicated with Mitral Regurgitation and Severe Heart Failure / 日本心臓血管外科学会雑誌

Left atrial aneurysm (LAA) is extremely rare. We report a surgical case of LAA complicated with mitral regurgitation (MR) and severe heart failure. A 71-year-old man presented dyspnea and leg edema, followed by congestive heart failure. Transthoracic echocardiogram (TTE) showed moderate MR, deteriorated left ventricular function, and echo free space connecting to the posterior wall of the left atrium. Three-dimensional reconstruction of computed tomography (3D-CT) clearly showed the whole shape of the LAA and its location relating to surrounding structures. LAA was 5×6 cm, expanding to apex side, and originated from the posterior wall of left atrium between circumflex branch of the left coronary artery and coronary sinus. LAA wall extended to the mitral posterior annulus, causing annular deformity and MR. Mitral valve plasty and aneurysmorrhaphy were performed. Biventricular pacing leads were implanted for cardiac resynchronization therapy, because of severe heart failure. Postoperative 3D-CT showed reduction of the LAA with no deformity of coronary vessels. No MR was detected by postoperative TTE. The patient has recovered without any complication after our treatments.

Surgical Treatment of Proximal Aortic Dissection in Patients with Stanford Type A Acute Aortic Dissection / 日本心臓血管外科学会雑誌

<b>Objective</b> : Although dissection extending to the aortic root is a common finding, it is potentially fatal in patients with acute type A aortic dissection. The purpose of this study was to evaluate surgical results of acute type A aortic dissection with proximal involvement. The proximal extension of dissection, types of aortic root procedure and its feasibility were investigated. <b>Methods</b> : Between 1997 and 2011, 80 patients with acute type A aortic dissection underwent emergent operation. <b>Results</b> : Dissection reaching around the coronary artery orifice was observed in 28 patients. In 11 patients, both left and right coronary arteries were involved with aortic dissection. Aortic root replacement was performed in 4 patients. In 7 patients, the dissected aortic root was reinforced by GRF glue and proximal aorta was replaced with a graft. Among these patients, postoperative aortic root redissection with severe aortic regurgitation was observed in 5 patients during postoperative long-term periods. All of them required surgical re-intervention of the aortic root. In 17 patients, dissection was extended to the right coronary artery. Aortic root reconstruction was performed in 2 patients due to pre-existing annulo-aortic ectasia. The remaining 15 patients underwent proximal reinforcement with GRF glue. No patient showed dissection extending to the left coronary artery alone. Operative mortality was 11% and other types of complications concerning the aortic root was not observed. <b>Conclusion</b> : An acceptable outcome was demonstrated with our surgical strategy of proximal aortic dissection. For patients, in particular, with proximal involvement to both the left and right coronary arteries, redissection of the aortic root should be noticed as a late complication with considerable frequency. Special care should be taken for precise recognition of the proximal extension of dissection and appropriate surgical procedure including simultaneous aortic root replacement.

Surgical Experience of Superior Mesenteric Venous Aneurysm / 日本心臓血管外科学会雑誌

Superior mesenteric venous aneurysm (SMVA) is rare and no standard treatment protocol has yet been established. We report our experience in performing surgical treatment for SMVA. A 64-year-old man was found to have a SMVA by computed tomography which had been performed during follow-up for gastrectomy. The SMVA was observed to gradually increase in diameter, and surgical treatment was therefore indicated. We successfully resected the aneurysm and then closed the defect with a bovine pericardial patch. Considering the potential risk of rupture, venous aneurysms that present with a saccular shape and an expanding tendency should be immediately surgically treated.

Port-Access Minimally Invasive Cardiac Surgery for Patent Foramen Ovale Complicated with Paradoxical Cerebral Embolism / 日本心臓血管外科学会雑誌

The patient was a 63-year-old man, who had developed cerebral infarction during treatment for sleep apnea syndrome. He also presented typical features of deep venous thrombosis of the right lower extremity. Transesophageal echocardiography clearly showed the blood flow passing through the patent foramen ovale (PFO) followed by Valsalva maneuver. Paradoxical cerebral embolism caused by a PFO was diagnosed. Several procedures were considered to prevent recurrence of cerebral infarction, he underwent PFO closure by minimally invasive procedure, so-called port-access cardiac surgery. He started walking on the day of surgery, and postoperative echocardiography showed no residual shunt flow. Currently, no catheter-based PFO closure device is allowed in Japan, the PFO closure by the port-access technique should be considered as a feasible alternative.

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.