[Coagulation Management During Open Heart Surgery Based on a Heparin Dose Sensitivity Test for Antiphospholipid Antibody Syndrome].

A 63-year-old woman with severe aortic regurgitation was admitted to our hospital due to congestive heart failure. She also had antiphospholipid syndrome (APS), necessitating strict coagulation management. Given her history of cerebellar infarction, deep vein thrombosis, and recurrent miscarriages, her thrombosis risk was higher, with all three types of antiphospholipid antibodies testing positive. Before the surgery, we created a heparin-activated clotting time (ACT) titration curve using the patient's blood, and the calculated ACT corresponding to the target heparin concentration of 3 U/ml was 650 seconds. We planned to administer heparin according to this target during cardiopulmonary bypass. The patient underwent an aortic valve replacement (AVR) using a bioprosthesis and was discharged without complications.

[Primary Cardiac Undifferentiated Pleomorphic Sarcoma in the Left Atrium:Report of a Case].

A rare case of primary cardiac undifferentiated pleomorphic sarcoma (UPS) is reported. A 77-yearold female was admitted to the authors' hospital with complaints of palpitation and dyspnea on effort. Echocardiography revealed a mobile tumor arising from the atrial septum of the left atrium. The tumor obstructed the mitral valve and the patient experienced acute heart failure. Emergent open-heart surgery was performed to resect the tumor. Although she was discharged from hospital on postoperative day 14 in a satisfactory condition, local recurrence in the left atrium was observed 16 months after surgery. Repeated tumor resection was performed, and histological examination confirmed UPS. The patient did not agree to undergo chemotherapy or radiation therapy and died of local recurrence 27 months after the first surgery.

[Incidentally Detected Right Atrial Blood Cyst].

A 48-year-old man underwent computed tomography for the examination of lower back pain, which incidentally detected a cardiac tumor in the right atrium. On echocardiography, the tumor was identified as a 30 mm round mass with a thin wall and iso- and hyper-echogenic contents that originated from the atrial septum. The tumor was successfully removed under cardiopulmonary bypass, and the patient was discharged in good health. The cyst was filled with old blood, and focal calcification was observed. Pathological examination revealed that the cystic wall was composed of thin-layered fibrous tissue lined with endothelial cells. Regarding a treatment, it is reported that early surgical removal is preferable to avoid embolic complications, however it is controversial. Furthermore, it needs to discuss about the difference between fetal/neonatal and adult cases.

[Stanford Type A Acute Aortic Dissection Complicated by Takotsubo Cardiomyopathy:Report of a Case].

No case report about takotsubo cardiomyopathy with Stanford type A acute dissection is present in the literature. Here we report a case of takotsubo cardiomyopathy that was diagnosed following Stanford type A acute aortic dissection. A 65-year-old man was admitted with dyspnea. He had experienced acute chest pain 10 days prior. Computed tomography (CT) confirmed Stanford type A aortic dissection and primary entry tear in the proximal aortic arch with a thrombosed false lumen in the ascending aorta. Echocardiography revealed takotsubo-like wall motion, with an ejection fraction (EF) of 20%. Electrocardiography (ECG) showed ST-segment elevation in V2-V3. Subsequently, coronary artery disease was excluded by coronary CT. After 1 month, ECG findings and EF appeared normal. Thirty-five days after admission, aortic arch replacement was performed with the frozen elephant trunk technique. The patient's postoperative course was uneventful. Takotsubo cardiomyopathy should be considered as a possible complication of acute aortic dissection with ST-segment elevation.

[Surgical Pulmonary Embolectomy for Acute Massive Pulmonary Embolism Using a Surgical Endoscope;Report of a Case].

Despite advances in medical and surgical therapeutic techniques, acute massive pulmonary embolism has a high mortality rate. Complete clot extraction without arterial wall injury is essential to save critically ill patients. Herein, we present a case of a 72-year-old woman who was treated by surgical pulmonary embolectomy using a surgical fiberscope. The patient was admitted to our hospital with a complaint of dyspnea. Computed tomography demonstrated a massive pulmonary embolism, and echocardiography revealed a floating thrombus in the right atrium and severe right heart failure. As she suffered from circulatory collapse, percutaneous cardiopulmonary support was immediately introduced and emergency surgical embolectomy was performed. Surgery was performed under circulatory arrest, and complete clot extraction was achieved using a surgical endoscope. The patient recovered well and was discharged from the hospital on day 48, with good health.

Outcomes of Early-Onset Acute Type A Aortic Dissection　- Influence of Etiologic Factors.

BACKGROUND: Outcomes of early-onset acute type A aortic dissection (ATAAD) associated with Marfan syndrome (MFS) are known, but not with other etiologies. Methods and Results: ATAAD patients from 2 centers (n=1,001) were divided into 2 groups: age ≤45 years (n=93) and age >45 years (n=908). Although in-hospital death and 10-year survival were similar (12% vs. 7% and 62.6% vs. 67.3%), the 10-year aortic event-free survival differed (50.0% vs. 80.2%; P<0.01). ATAAD patients from 3 centers (n=132), all aged ≤45 years, were divided into 5 groups: lone hypertension (HTN, n=71), MFS (n=23), non-syndromic familial thoracic aortic aneurysm and dissection (NS-FTAAD, n=16), bicuspid aortic valve (BAV, n=11), and no known etiologic factor (n=11). The incidence of severe aortic insufficiency varied between groups (HTN: 11%, MFS: 39%, NS-FTAAD: 38%, BAV: 55%, no known factor: 46%; P<0.01), whereas in-hospital death did not (14%, 22%, 0%, 0%, and 9%; P=0.061). The 10-year survival was 52.2%, 64.7%, 83.6%, 100%, and 90.9%, respectively, and 10-year aortic event-free survival was 55.6%, 36.3%, 77.5%, 90.0%, and 30.0%. Median descending aorta growth (mm/year) was 1.1 (0.1-3.4), 2.3 (0.3-5.3), 1.9 (1.3-2.7), 0.9 (-0.1-2.0), and 1.0 (-0.2-2.9) (P=0.15), respectively. CONCLUSIONS: Late aortic events are common in young ATAAD patients. Known etiologic factors, though not BAV, negatively influence late outcomes in these patients.

[Right Atrium Thrombus Due to Cardiac Amyloidosis;Report of a Case].

A 51-year-old man complaining of exertional dyspnea and syncope was admitted to our hospital. Computed tomography(CT) and transesophageal echocardiography demonstrated a mobile tumor-like lesion in the right atrium. Surgical resection was performed under cardioplegic arrest, which revealed an organized thrombus. Pathological examination revealed amyloid deposition in the myocardium, and bone marrow biopsy revealed CD138 (+) stem cells. Therefore, the patient was diagnosed with amyloid light-chain (AL) amyloidosis associated with multiple myeloma. Although he required percutaneous cardiopulmonary support for postoperative right heart failure for 3 days, he recovered well and was discharged from the hospital on day 44, in a good condition. He was treated with bortezomib for multiple myeloma after surgery. Thus, despite the maintenance of sinus rhythm, intra-cardiac thrombus could be formed with amyloidosis.

[Hip Fracture Repair with Concomitant Transcatheter Aortic Valve Implantation;Report of a Case].

The incidences of hip fracture and aortic valve stenosis are increasing in the aging population. Operative repair for hip fracture contributes to excellent clinical results. Transcatheter aortic valve implantation, which does not require cardiopulmonary bypass, represents a new era for the treatment of aortic valve stenosis. We herein describe a patient with both hip fracture and severe aortic valve stenosis. A 93-year-old woman underwent transcatheter aortic valve implantation for the valve stenosis immediately followed by open repair surgery for the fracture. She fully recovered without heart failure during the postoperative rehabilitation period. The performance of concomitant surgeries for hip fracture and aortic valve stenosis might increase in the future.

[Idiopathic Pulmonary Artery Aneurysm].

Pulmonary artery aneurysm (PAA) is usually associated with congenital heart disease, pulmonary artery hypertension, and connective tissue abnormalities, but idiopathic PAA is a rare clinical entity. We experienced a surgical case of idiopathic PAA measuring 60 mm in diameter. A 72-year-old man had been admitted to a nearby hospital 5 years before because of an abnormal shadow on chest X-ray, and was diagnosed with an idiopathic PAA measuring 37 mm in diameter. The PAA gradually expanded during follow-up and surgery was scheduled. Aneurysmal resection and reconstruction with a 24 mm expanded polytetrafluoroethylene graft were performed. The postoperative course was uneventful and the patient was discharged on the 11th postoperative day.

[Two-stage Surgery for Uterine Intravenous Leiomyomatosis with Right Ventricular Extension;Report of a Case].

Intravenous leiomyomatosis is a rare neoplastic condition characterized by the benign intravascular proliferation of smooth muscle cells originating from either the uterine venous wall or a uterine leiomyoma. In the present report, we describe the case of a 36-year-old woman, who was referred to our institution due to abdominal pain. Computed tomography indicated the presence of a giant intravenous leiomyoma originating from the uterus and extending to the right ventricle with complex pathways. The patient was successfully treated by tumor resection under circulatory arrest in two-stage operations in conjunction with gonadotrophin-releasing hormone agonists therapy. She continued to recover and did not exhibit any recurrence at the 8-month follow-up.

[Intravascular Hemolysis Caused by Stenosis of an Elephant Trunk;Report of a Case].

Symptomatic intravascular hemolysis after prosthetic aortic graft replacement is rare. It is primarily attributed to mechanical injury of red blood cells caused by stenosis of the vascular graft. A 50-year-old man presented with hemolytic anemia, 5 years after total arch replacement with an elephant trunk for type A aortic dissection. The hemolysis was caused by graft stenosis of the elephant trunk. Endovascular treatment for the stenotic elephant trunk was successfully performed. The postoperative course was uneventful, and the hemolysis was resolved immediately after operation.

[Aortic Valve Replacement Via a Partial Sternotomy in an Ankylosing Skeletal Hyperostosis Patient after Prophylactic Tracheostomy;Report of a Case].

A 77-year-old man presented with exertional dyspnea. The patient had a history of ankylosing skeletal hyperostosis and sleep apnea syndrome. Echocardiographic examination revealed severe aortic valve regurgitation. The patient underwent a prophylactic tracheostomy 2 weeks before cardiac surgery to decrease his risk of perioperative respiratory failure. He successfully underwent aortic valve replacement through a lower partial sternotomy. His postoperative course was uneventful, and the tracheostoma was closed 6 months after the cardiac surgery.

[Coronary Artery Perforation Secondary to Primary Percutaneous Coronary Intervention;Report of a Case].

Stent perforation of a coronary artery during percutaneous coronary intervention (PCI) is a rare but life-threatening complication. A 70-year-old man was admitted to our hospital for management of acute coronary syndrome. PCI was attempted for the left circumflex artery(LCX);however, catheterization was complicated by perforation of the LCX by the stent. He developed cardiac tamponade and was resuscitated using percutaneous cardiopulmonary support. Emergency surgery( ligation of the LCX and aorto-coronary bypass grafting for the distal LCX) was performed. His postoperative course was uneventful, and the patient was discharged on the 20th postoperative day. Collaboration between cardiologists and surgeons is essential to salvage such a critical patient.

[Prophylactic Effectiveness of Vacuum-assisted Closure for High-risk Patients Undergoing Cardiovascular Surgery through Median Sternotomy].

Vacuum-assisted closure(VAC) therapy is mainly used for tissue defects. VAC therapy can remove exudate that could impair the healing process. We applied VAC therapy in patients considered at high risk of surgical site infection who underwent cardiovascular surgery via standard median sternotomy. Risk factors included advanced heart failure, obesity, diabetes mellitus, steroid administration, immunosuppressant administration, and chronic renal failure, etc. VAC therapy was used in 134 patients. Only 3 of these patients (2.2%) developed surgical site infection caused by Staphylococcus epidermidis;2 patients fully recovered after prolonged VAC therapy for 2 weeks, and the other required an additional sternal fixation after the sternal wires were removed for wound infection control. No patient developed infective mediastinitis. Prophylactic VAC therapy can reduce postoperative wound infection in high risk patients undergoing open heart surgery via full sternotomy.

Heart valve surgery in hemodialysis-dependent patients: nutrition status impact on surgical outcome.

Valve surgery in hemodialysis-dependent patients is associated with postoperative complications and a high mortality rate, and such patients frequently suffer cachexia. This study aimed to determine pre- and intraoperative risk factors associated with in-hospital mortality and long-term survival in hemodialysis-dependent patients undergoing heart valve surgery from the viewpoint of nutrition status. Eighty-seven hemodialysis-dependent patients who underwent valve surgery between January 1998 and October 2015 were retrospectively reviewed. Thirty-seven potential perioperative risk factors were evaluated. The in-hospital mortality rate was 12.6 % (11 patients). Univariate analysis identified New York Heart Association Functional Classification III or IV, emaciation (body mass index <17.6 kg/m(2)), total cholesterol <120 mg/dl, serum albumin <3.0 mg/dl, emergent/urgent surgery, and intraoperative blood transfusion >3000 ml as predictors of in-hospital death. Multivariate logistic regression analysis confirmed low serum albumin <3.0 mg/dl (hazard ratio 7.22; p = 0.032) and emergent/urgent operation (hazard ratio 43.57; p = 0.035) as independent predictors of in-hospital death. The 1- and 3-year actuarial survival rates were 64.9 ± 5.4 and 51.8 ± 5.8 %, respectively. Long-term survival estimated by log-rank test was negatively impacted by anemia (hemoglobin <10 mg/dl), low serum albumin, emergent/urgent operation, and infective endocarditis. Multivariate analysis using Cox proportional hazards modeling indicated low serum albumin (hazard ratio 2.12; p = 0.047) and emergent/urgent operation (hazard ratio 8.97; p = 0.0002) as independent predictors of remote death. Hypoalbuminemia and emergent/urgent operation are strong predictors of in-hospital and remote death. Malnutrition before surgery should be considered for operative risk estimation, and adequate preoperative nutrition management may improve surgical outcomes for hemodialysis-dependent patients.

[A Giant Right Coronary Artery Aneurysm Exceeding 8-cm in Size].

A coronary artery aneurysm(CAA) that exceeds 20 mm in diameter is rare. In this case report, we describe the operative correction of a giant right CAA measuring greater than 8 cm in diameter. A 51-year-old male with a history of Kawasaki disease at 7 years of age was admitted to our hospital for evaluation of an abnormal shadow on a chest radiograph. Multi-detector row computed tomography (MDCT) demonstrated a giant, 82-mm right coronary aneurysm in the right atrioventricular groove with a layered thrombus. The patient underwent aneurysmectomy and coronary artery bypass grafting using the right internal thoracic artery under cardiopulmonary bypass. The postoperative course was uneventful, and postoperative MDCT revealed complete resection of the aneurysm and good patency of the bypass graft. The patient was discharged on the 8th postoperative day in good health.

[Successful mitral valve replacement in a patient with functional mitral regurgitation induced by cardiac sarcoidosis;report of a case].

We report a case of cardiac sarcoidosis associated with mitral valve regurgitation. A 62-year-old woman with cardiac sarcoidosis was admitted for the treatment of an intractable mitral regurgitation. She had been treated for cardiac sarcoidosis with prednisolone, and she had undergone pacemaker implantation because of advanced complete A-V block 5 years before. However, her hemodynamics deteriorated, and echocardiography revealed severe functional mitral regurgitation, thinning of the ventricular septum, and left ventricular dysfunction. The patient underwent mitral valve replacement with a mechanical prosthetic valve, and her postoperative course was uneventful. She is currently well without exacerbation of heart failure at 2 years after operation. Functional mitral regurgitation is a relatively common complication in patients with cardiac sarcoidosis. Mitral valve replacement should be considered in patients with medically intractable mitral valve dysfunction due to cardiac sarcoidosis.

[Papillary Muscle Rupture after Repair of Ischemic Left Ventricular Free Wall Rupture; Report of a Case].

A 67-year-old man experienced acute inferior myocardial infarction. Echocardiography and computed tomography showed massive pericardial effusion. He underwent emergency operation for ischemic ventricular free wall rupture. During the operation, an oozing type rupture was found on the inferior wall and the bleeding was completely controlled by applying fibrin glue sheets. On the 5th day after the operation, ventricular tachycardia appeared with hemodynamic deterioration. Echocardiography showed a ruptured posteromedial papillary muscle with massive mitral regurgitation. Intra-aortic balloon pumping was introduced and emergency repair operation was performed. The mitral valve was replaced with a bioprosthetic valve. The postoperative course was uneventful.

[Pannus Formation Six-years after Aortic and Mitral Valve Replacement with Tissue Valves;Report of a Case].

A 77-year-old man presented with exertional dyspnea. He had undergone aortic and mitral valve replacement with tissue valves 6-years earlier. The patient's hemoglobin level was 9.8 g/dl and serum aspartate aminotransferase (70 mU/ml) and lactate dehydrogenase (1,112 mU/ml) were elevated. Echocardiography revealed stenosis of the prosthetic valve in the aortic position with peak flow velocity of 3.8 m/second and massive mitral regurgitation. The patient underwent repeat valve replacement. Pannus formation around both implanted valves was observed. The aortic valve orifice was narrowed by the pannus, and one cusp of the prosthesis in the mitral position was fixed and caused the regurgitation, but they were free from cusp laceration or calcification. The patient's postoperative course was uneventful, and he continues to do well 14 months after surgery.