[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.

Influence of residual primary entry following the tear-oriented strategy for acute type A aortic dissection.

OBJECTIVES: Although a tear-oriented strategy has contributed to improving short-term surgical outcomes of acute type A aortic dissection (ATAAD), long-term clinical influences of residual entry tear in the downstream aorta have not been fully investigated. The goal of this study was to assess the long-term surgical outcomes of ATAAD with or without a residual entry tear in the downstream aorta. METHODS: Medical records of 1107 patients with ATAAD who underwent emergency surgery between 1990 and 2018 were retrospectively reviewed. A tear-oriented paradigm was adopted for the baseline strategy. The 837 patients in whom the entry tears were resected comprised the resected group, and the 270 patients with a residual entry tear comprised the residual group. Of these patients, 252 in each group were analysed using propensity score matching, and long-term outcomes were compared with or without residual entry. RESULTS: Hospital deaths were lower in the resected group (3.2% vs 8.3%; P = 0.020). The survival rate was not significantly different between the groups: It was 83.8% and 68.5% in the resected group and 80.2% and 66.5% in the residual group at 5 and 10 years, respectively (P = 0.600). However, residual entry in the downstream aorta affected the distal aortic event-free survival rate (90.4% and 80.6% in the resected group and 82.3% and 67.4% in the residual group at 5 and 10 years, respectively; P = 0.003). Furthermore, multivariable risk analysis of 1107 patients confirmed that a residual entry in the downstream aorta was a risk factor for distal aortic events. CONCLUSIONS: The tear-oriented strategy remains the gold standard for high-risk patients; however, the extensive operation might be considered for stable patients to reduce long-term aortic events.

Artificial endocrine pancreas with a closed-loop system effectively suppresses the accelerated hyperglycemic status after reperfusion during aortic surgery.

OBJECTIVES: To control intraoperative hyperglycemia in patients who underwent aortic surgery using STG-55® artificial endocrine pancreas and clarify the effectiveness of this device. METHODS: Blood glucose control using the STG-55® was performed in 18 patients (15 men and 3 women; age, 66 ± 10 years) who required hypothermic circulatory arrest (STG-55® group). Seventeen patients (10 men and 7 women; age, 71 ± 8 years) whose blood glucose was controlled using the conventional method were included in the control group. Glucose concentration was controlled with the aim of maintaining it at 150 mg/dl. RESULTS: In both groups, the blood glucose concentrations did not significantly change during the interruption of systemic perfusion; however, a sharp increase was noted immediately after reperfusion. Although the hyperglycemic status persisted after reperfusion in the control group, it was effectively suppressed in the STG-55® group (STG® vs. control group at 50 min after reperfusion: 180 ± 35 vs. 212 ± 47 mg/dl, p = 0.026) and blood glucose concentration reached the target value of 150 mg/dl at 100 min after reperfusion (STG® vs. control group: 153 ± 29 vs. 215 ± 43 mg/dl, p = 0.0008). The total administered insulin dose was 175 ± 81 U and 5 ± 3 U in the STG® and control groups, respectively (p < 0.0001). CONCLUSIONS: To treat the accelerated hyperglycemic status in aortic surgery requiring circulatory arrest, strict glycemic control using an artificial endocrine pancreas might be beneficial.

Successful treatment of Aspergillus mural endocarditis in malignant lymphoma using a combined antifungal and surgical approach: a case report.

BACKGROUND: Aspergillus endocarditis (AE) is a rare and lethal cardiac infection with a high rate of mortality. AE most commonly presents in immunocompromised patients and is associated with various co-morbidities. Herein, we present a case of AE associated with lung, brain, and cervical abscesses after chemotherapy for malignant lymphoma that was successfully treated by a combination of antifungal and surgical therapy. CASE PRESENTATION: A 29-year-old man was admitted to our hospital with an unidentified fever. He was diagnosed with malignant lymphoma (extra-nodal NK/T cell lymphoma nasal type), and chemotherapy was administered. After chemotherapy, nodular lung shadows along with new brain, cervical, and myocardial abscesses appeared, despite anti-bacterial/fungal therapy. Gene analysis of the cervical abscess biopsy revealed the presence of Aspergillus fumigatus species, and the transesophageal echocardiogram showed a mobile mural vegetation in the left ventricle (22 × 8 mm). He underwent surgical resection of this mural vegetation. His postoperative course was uneventful. He remains healthy at 28 months after surgery with continued oral antifungal therapy. CONCLUSION: Although AE associated with immunosuppression is a fatal clinical presentation, combined treatment with surgical resection and antifungal therapy was effective.

[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.

Effects of Obesity on Outcomes of Acute Type A Aortic Dissection Repair in Japan.

Background: The prevalence of obesity among Japanese acute type A aortic dissection (ATAAD) patients and its effect on repair outcomes remain to be elucidated. Methods and Results: The prevalence of obesity (body mass index [BMI] ≥30.0 kg/m2) among 1,059 patients (mean [±SD] age 64.3±12.7 years) who underwent ATAAD repair between 1990 and 2018 was compared with that among the general Japanese population (National Health and Nutrition Survey data). The prevalence of obesity among male patients (17.1% [6/35], 20.0% [18/90], and 14.4% [20/139] for those aged 20-39, 40-49, and 50-59 years, respectively) was significantly higher than that among the age- and sex-matched general population. The 1,059 patients were divided into groups according to weight (normal [BMI <25.0 kg/m2; n=742], overweight [BMI 25.0-29.9 kg/m2; n=248], or obese [BMI ≥30.0 kg/m2; n=69]). Comparing the normal weight, overweight, and obese groups revealed significant differences among the 3 groups in median cardiopulmonary bypass time (143, 167, and 183 min, respectively), ventilation >48 h (44.5%, 60.1%, and 78.3%, respectively), and in-hospital mortality (7.0%, 7.3%, and 17.4%, respectively), but not in 30-day survival. Shock, visceral malperfusion, operation time >360 min, obesity, and coronary malperfusion were identified as predictors of in-hospital mortality. Conclusions: The prevalence of obesity is increased among Japanese male patients with ATAAD aged ≤59 years. Obesity may increase these patients' operative risk; overweight does not.

[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.

Malperfusion in type A aortic dissection: results of emergency central aortic repair.

BACKGROUND: Although outcomes of acute type A aortic dissection (ATAAD) have improved, malperfusion remains associated with high morbidity and mortality rates, and its optimal therapeutic treatment is unknown. Emergency central repair has been performed as our first-line approach for malperfusion. Here, we analyzed outcomes of ATAAD with malperfusion and reassessed emergency central repair. METHODS: In total, 1026 ATAAD patients underwent emergency surgery within 48 h of symptom onset, of whom 318 (30.9%) patients complicated with any preoperative malperfusion were included. Pathophysiology of malperfusion and surgical outcomes were analyzed. RESULTS: The in-hospital mortality rate was 12.9% for patients with malperfusion and 4.8% for patients without malperfusion (p < 0.0001). Coronary malperfusion was complicated in 7.5% of patients (% dead per group, 19.5%), mesenteric malperfusion in 3.6% (24.3%), renal malperfusion in 8.8% (14.4%), lower leg malperfusion in 12.6% (13.7%), brain malperfusion in 9.7% (12.0%), and spinal malperfusion in 1.1% (18.2%). Mortality rates varied substantially according to the number of affected organ systems (none, 4.8%; one system, 10.4%; two systems, 14.5%; three systems, 30.0%, and four systems; 30.3%; p < 0.0001). In malperfused patients, logistic regression analysis revealed that obesity (body mass index > 30 kg/m2), preoperative shock (systolic blood pressure < 80 mmHg), and visceral ischemia were independent predictors for hospital death. CONCLUSIONS: Malperfusion of more organ systems and mesenteric malperfusion resulted in unfavorable prognosis, and effects of central repair were limited in such severe/complex malperfusion. To further improve outcomes of ATAAD with malperfusion, emergency reperfusion of affected organs followed by central repair might be considered.

Surgical outcomes of acute type A aortic dissection in dialysis patients.

BACKGROUND: Acute type A aortic dissection (ATAAD) is relatively uncommon in dialysis patients, and characteristics and repair outcomes are not fully understood. PATIENTS AND METHODS: Patients with ATAAD (n = 960) were divided into a dialysis group (n = 19) and non-dialysis group (n = 941), depending on whether they required dialysis for preoperative end-stage renal disease (ESRD). Hospital charts and imaging data were reviewed, and characteristics and outcomes were compared between the groups. Segmental aortic wall or intima/media flap calcification in the thoracic and abdominal aorta was assessed in the dialysis patients. RESULTS: The leading primary causes of ESRD were polycystic kidney disease (n = 5) and chronic glomerulonephritis (n = 5). There were no significant differences (dialysis group vs. non-dialysis group) in age (60.5 vs. 64.5 years), preoperative hemodynamics, or organ ischemia. Dialysis patients were more likely to have an entry tear in the aortic arch (42% vs. 15%, p = 0.003). These patients showed moderate-to-severe calcification (multiple focal or single focal calcification > 10 mm) in the ascending aorta (17%), aortic arch (61%), descending aorta (67%), and abdominal aorta (83%). Arch replacement was common in this group (37% vs. 18%, p = 0.030). Although in-hospital mortality was increased in this group (21% vs. 7%, p = 0.059), morbidities did not differ significantly. Six-year survival was 60.3 ± 13.4% and 78.8 ± 1.6%, respectively (p = 0.01). CONCLUSIONS: Dialysis patients tend to have aortic calcification and a primary tear in the aortic arch. Outcomes are acceptable.

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.

[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.

Open Surgical Decompression Is Useful for the Prevention and Treatment of Abdominal Compartment Syndrome after the Repair of Ruptured Abdominal Aortic and Iliac Artery Aneurysm.

Objective: This study was performed to determine whether open surgical decompression (OSD) decreased the mortality associated with abdominal compartment syndrome (ACS) following open repair (OR) of ruptured abdominal aortic aneurysm and iliac aneurysm (rAAA), and to investigate the risk factors associated with OSD. Material and Methods: Total 113 consecutive patients with rAAA underwent OR in our institution. Ninety patients underwent primary abdominal closure; however, three of them developed ACS and required OSD. Prophylactic OSD was performed at the initial OR in 23 patients. Results: The in-hospital mortality rate was higher in those who underwent OSD than in those who did not undergo OSD [27.0% (7/26) vs. 6.9% (6/87), respectively; p=0.01]. However, no ACS-related death occurred in the OSD group. Multivariate analyses revealed that a preoperative/intraoperative base excess (BE)<-11 [p=0.045; odds ratio (OR), 3.33; 95% confidence interval (CI), 1.021-10.850], performance of left thoracotomy (p=0.038; OR, 5.17; 95%CI, 1.098-24.357), and intraoperative blood transfusion >1,800 mL (p=0.012; OR, 4.30; 95%CI, 1.386-13.322) were associated with OSD. Conclusion: The prevalence and mortality rates of ACS were low at our institution. OSD is considered to be useful for the prevention and treatment of ACS after repair of rAAA. OSD should be considered in patients with the above-mentioned factors.