A Case of Aortopulmonary Fistula with Post-Operative Aortic Pseudoaneurysm Diagnosed by Transesophageal Echocardiography.

Tracheobronchial or esophageal fistula after aortic surgery has been reported sporadically in the literature, however, reports of an aortopulmonary fistula associated with a post-operative aortic pseudoaneurysm are rare. We experienced a case of refractory heart failure due to an aortopulmonary fistula associated with a post-operative aortic pseudoaneurysm. A 60-year-old man who had undergone aortic surgery 2 years earlier was hospitalized for congestive heart failure. He was diagnosed with refractory heart failure after 10 days of diuretic therapy failed to improve his condition. He underwent a contrast-enhanced computed tomography (CT) scan and was suspected to have pulmonary artery perforation of an aortic pseudoaneurysm at the anastomotic site of the ascending aortic surgery. Transesophageal echocardiography showed shunt blood flow from the aortic aneurysm into the right pulmonary artery, leading to a definitive diagnosis of aortopulmonary fistula with post-operative aortic pseudoaneurysm. Computed tomography angiography is commonly used to diagnose an aortic fistula; however, diagnosis is often difficult because of the subtle imaging findings. We highlight the usefulness of transesophageal echocardiography in providing a definitive diagnosis and detailed morphologic information on this pathophysiology.

A Case of Acute Right Coronary Artery Ostial Obstruction Six Days After Surgical Aortic Valve Replacement Requiring Emergent Coronary Artery Bypass Grafting.

Acute coronary artery obstruction after surgical aortic valve replacement (SAVR) is a rare but potentially life-threatening event that must be prevented. Here, we report a rare case of an 84-year-old woman who underwent SAVR with a 19-mm aortic bioprosthetic valve for severe aortic stenosis and who suddenly developed ST-elevation myocardial infarction six days after surgery as a result of right coronary artery (RCA) ostial obstruction. She experienced cardiogenic shock, and mechanical support devices were introduced; however, she underwent emergency coronary artery bypass grafting (CABG) to the RCA (#3) and survived. We were aware of the risk of RCA ostial obstruction intraoperatively but were unable to prevent it because blood flow was preserved in the early postoperative period. The present case is worth reporting because the patient developed fatal STEMI at a time when she would normally be considered for discharge. A major learning point from this case is that a coronary artery ostium that is patent immediately after SAVR may not be sufficient for patients considered at high risk of coronary artery occlusion.

Successful detection of multiple communicating holes in multiple spinal extradural arachnoid cysts by using time-spatial labeling inversion pulse magnetic resonance imaging: illustrative case.

BACKGROUND: Spinal extradural arachnoid cysts (SEACs) communicate with the subarachnoid space through small communicating dural holes. The precise preoperative detection of all communicating holes, followed by minimally invasive dural closure, is the ideal treatment to prevent postoperative spinal deformities, especially in cases of multiple SEACs. However, standard imaging methods often fail to detect communicating hole locations. Although a few cases of successful single-hole detection via cinematic magnetic resonance imaging (MRI) have been reported, this modality's ability to detect multiple holes has not been demonstrated. OBSERVATIONS: The authors describe the case of a 14-year-old male with myelopathy due to multiple SEACs at T5-8 and T8-12. Myelography revealed a complete block at the T8 level; no cephalic cyst or communicating holes were identified. Time-spatial labeling inversion pulse (T-SLIP) MRI revealed cerebrospinal fluid flow into the cyst at T10 and T7. A limited laminectomy or hemilaminectomy was performed at T7 and T10, and two dural holes were closed without a total cystectomy. The patient's gait disturbance and rectal disorder disappeared. The cysts were confirmed to have completely disappeared on conventional MRI at 1 year postoperatively. LESSONS: T-SLIP MRI, a cinematic MRI, is useful for detecting multiple communicating holes in SEACs.

A Case of Femoral Artery Aneurysm Caused by Methotrexate Associated Lymphoproliferative Disease.

An 82-year-old man with left leg edema was referred to our department after an ultrasound examination by his previous physician, which revealed deep vein thrombosis (DVT) in the left superficial femoral vein and a left common femoral artery aneurysm (CFAA). The DVT was caused by the CFAA. The patient was adjudged to be at high risk of peripheral embolization due to the irregular shape of the varicose vein and a large amount of mural thrombus. Surgery was performed to replace the artificial blood vessel. The patient displayed firm adhesion to the surrounding area, marked lymph node swelling, and a large amount of mural thrombus in the mass. The superficial femoral artery (SFA) demonstrated severe intimal thickening and partial dissection. The postoperative course was good, and the patient was undergoing rehabilitation to be discharged home; however, B-cell lymphoma was suspected based on the pathology results of the mass wall submitted intraoperatively. The patient had a history of rheumatoid arthritis and was treated with methotrexate (MTX). During the course of his illness, a subcutaneous mass was found on his right forearm, and a skin biopsy revealed MTX-associated lymphoproliferative disease (MTX-LPD), which had resolved with MTX withdrawal. The histopathological results of the skin biopsy matched those of the CFAA mural thrombus, and Epstein-Barr virus-positive cells were also observed, leading to the diagnosis of MTX-LPD, which was considered to be the cause of CFAA. No MTX-LPD was identified in the vessel walls or intramural thrombus. We herein report a case of CFAA with an extremely rare etiology and clinical presentation.

Successful Percutaneous Thoracic Duct Embolization for Chylothorax After Total Arch Replacement.

Chylothorax after cardiac surgery is a rare complication associated with severe morbidity and mortality. This report documents successful treatment with percutaneous thoracic duct embolization for chylothorax after total arch replacement. A 69-year-old man underwent replacement of the aortic arch to treat a ruptured aortic aneurysm. After surgery, the left thoracic drain discharged 2,000 to 3,000 mL serosanguineous fluid per day, even though the patient took nothing orally and was administered subcutaneous octreotide therapy. On postoperative day 9, percutaneous thoracic duct embolization was performed, and the drain could be removed. The chylothorax did not recur, and the patient was discharged on postoperative day 17.

A Surgical Case of Infected Popliteal Artery Aneurysm From Rare Bacteria, Eubacterium sp.

A 79-year-old man presented to our hospital with complaints of a sudden worsening of swelling in the right popliteal fossa and fever persisting for a week. Upon close examination, an infected popliteal artery aneurysm (PAA) was identified. Given the risk of rupture, the patient was advised to undergo surgery. The surgical procedure involved resecting the infectious PAA using a lateral approach. Additionally, a bypass was performed from the superficial femoral artery to the below-knee artery, utilizing the great saphenous vein located at the posterior aspect of the knee. Surgical findings revealed a popliteal artery pseudoaneurysm. Preoperative blood cultures identified Eubacterium spp., and cultures of the inoperative aneurysm specimens confirmed the presence of the same bacteria. After surgery, inflammation quickly subsided, and the patient was discharged on postoperative day (POD) 41 after receiving transvenous antibiotic therapy. Although PAA accounts for approximately 80% of all peripheral arterial aneurysms, mycotic aneurysms are relatively rare. Eubacterium spp. is part of the human intestinal or oral flora, and very few reports of bacteremia have been published. The present case of bacteremia caused by Eubacterium sp. is very rare; to the best of our knowledge, no literature has been published on this topic.

Delayed Giant Pseudoaneurysm With Left-to-Right Shunt Following Postinfarct Ventricular Septal Perforation Repair.

Left ventricular pseudoaneurysm with a left-to-right shunt is extremely rare, requiring surgery if symptomatic; however, surgery has a high risk. Here, the case of a 77-year-old man with heart failure symptoms is reported, in which he develops a giant left ventricular pseudoaneurysm 16 months after ventricular septal perforation repair as a result of acute myocardial infarction, with mild shunt blood flow from the pseudoaneurysm to the right ventricle. Intraoperative findings showed a free wall rupture along the area where the patch was secured during the initial surgery. The patient was discharged on postoperative day 13, and postoperative examination revealed no abnormalities.

Association Between Serum GDF15 Concentration and Total Mortality in Community-Dwelling Japanese Older Populations: The Involvement of Renal Dysfunction.

BACKGROUND: Serum growth differentiation factor 15 (GDF15) is associated with age-related adverse outcomes. However, renal function has not been thoroughly evaluated in studies addressing the association between GDF15 and mortality. We aimed to clarify whether GDF15 is associated with total mortality after carefully controlling renal function markers. METHODS: We divided 1 801 community-dwelling Japanese older adults into quartiles according to their serum GDF15 concentrations. The correlation of GDF15 with renal function and inflammation markers was assessed by calculating Spearman correlation coefficients. Cumulative survival rates of the quartiles were estimated. In a Cox regression analysis adjusted for confounders, the association between GDF15 and mortality was evaluated. The discriminative capacity of GDF15 for the prediction of mortality was assessed with receiver-operating characteristic analysis. RESULTS: GDF15 was correlated with cystatin C (r = 0.394), ß2-microglobulin (r = 0.382), C-reactive protein (r = 0.124), and interleukin-6 (r = 0.166). The highest GDF15 quartile showed poor survival compared to the others. Older adults with higher GDF15 were associated with an increased mortality risk, independent of demographics and clinically relevant variables (hazard ratio [95% confidence interval]: 1.98 [1.09-3.59]). This significant association disappeared when additionally adjusted for cystatin C (1.65 [0.89-3.05]) or ß2-microglobulin (1.69 [0.91-3.12]). The ability to predict mortality was approximately comparable between GDF15 (area under the curve: 0.667), cystatin C (0.691), and ß2-microglobulin (0.715). CONCLUSIONS: Serum GDF15 is associated with total mortality in older Japanese after adjustment for major confounders. The increased mortality risk in older adults with higher GDF15 may be partly attributed to decreased renal function.

Intraoperative Type B Aortic Dissection during Total Arch Replacement.

BACKGROUND: Intraoperative aortic dissection is an extremely serious complication that should be prevented whenever possible. When it does occur, it requires urgent clinical management. CASE PRESENTATION: We report the case of a 78-year-old man with Marfan syndrome who developed an intraoperative complicated type B aortic dissection with a distal anastomosis entry site during total arch replacement for a chronic dissection. CONCLUSION: Performing immediate thoracic endovascular aortic repair, we were able to improve malperfusion to the lower extremities occurred during total arch replacement.

A Single Arm Clinical Study on the Effects of Continuous Erythropoietin Receptor Activator Treatment in Non-Dialysis Patients with Chronic Heart Failure and Renal Anemia.

Erythropoiesis-stimulating agents improve the NYHA functional class and decrease the hospital readmission rates for heart failure; however, little is known about the influence of continuous erythropoietin receptor activator (CERA) on the heart. Therefore, a prospective study was conducted to investigate the effects of CERA on cardiac and renal function and oxidative stress in chronic heart failure with renal anemia. Sixty patients with chronic heart failure and renal anemia were enrolled and received CERA for 12 months. The primary endpoints were hemoglobin (Hb) and hematocrit, and the secondary endpoints were: (1) atrial natriuretic peptide (ANP) and B-type natriuretic peptide (BNP); (2) NYHA class; (3) echocardiography; (4) blood urea nitrogen, creatinine, cystatin C, and urinary albumin; (5) high-sensitivity C-reactive protein; (6) oxidized low-density lipoprotein (Ox-LDL); and (7) renin, angiotensin-II, and aldosterone. There was a significant difference in the Hb levels measured before and after CERA administration. The BNP, ANP, NYHA, left ventricular mass index, renal function, and Ox-LDL decreased significantly after CERA administration. This study shows that CERA improves anemia and reduces renal impairment, as well as cardiac and oxidative stress. The result of this study is useful for a study in which switching from CERA to a new renal anemia drug, hypoxia-inducible factor prolyl-hydroxylase inhibitor, is investigated.

Novel Therapeutic Potentials of Taxifolin for Obesity-Induced Hepatic Steatosis, Fibrogenesis, and Tumorigenesis.

The molecular pathogenesis of nonalcoholic steatohepatitis (NASH) includes a complex interaction of metabolic stress and inflammatory stimuli. Considering the therapeutic goals of NASH, it is important to determine whether the treatment can prevent the progression from NASH to hepatocellular carcinoma. Taxifolin, also known as dihydroquercetin, is a natural bioactive flavonoid with antioxidant and anti-inflammatory properties commonly found in various foods and health supplement products. In this study, we demonstrated that Taxifolin treatment markedly prevented the development of hepatic steatosis, chronic inflammation, and liver fibrosis in a murine model of NASH. Its mechanisms include a direct action on hepatocytes to inhibit lipid accumulation. Taxifolin also increased brown adipose tissue activity and suppressed body weight gain through at least two distinct pathways: direct action on brown adipocytes and indirect action via fibroblast growth factor 21 production in the liver. Notably, the Taxifolin treatment after NASH development could effectively prevent the development of liver tumors. Collectively, this study provides evidence that Taxifolin shows pleiotropic effects for the treatment of the NASH continuum. Our data also provide insight into the novel mechanisms of action of Taxifolin, which has been widely used as a health supplement with high safety.

Preoperative Sarcopenia Assessment Using Pectoralis Muscle Mass Indicated Poor Mid-term Cardiac Surgery Prognosis.

BACKGROUND: Many studies have defined sarcopenia based on psoas muscle mass using abdominal computed tomography (CT). We hypothesized that sarcopenia can be assessed by measuring pectoralis muscle mass on chest CT and aimed to examine its relationship with the postoperative prognosis of cardiac surgery. METHODS: This retrospective study included 189 patients who underwent cardiac surgery via median sternotomy between July 2020 and June 2022. We excluded patients <70 years old, urgent/emergent cases, no chest CT within 90 days before surgery, and cases in which evaluation of the pectoralis muscle was impossible with CT. The pectoralis muscle area (PMA) was measured using a preoperative chest CT. The sarcopenia cut-off value was defined as the lowest sex-specific tertile in PMA at the level of the 4th thoracic vertebrae. RESULTS: Eighty patients were included. The lower tertile were classified as the sarcopenia group (SG) (n = 26) and the rest as the non-sarcopenia group (NSG) (n = 54). In the SG, 1-year survival was significantly worse than that in NSG (NSG: 92.7% vs. SG: 54.9%, p < 0.0001). In the multivariate model, sarcopenia was an independent risk factor for mid-term all-cause death (hazard ratio, 4.89; 95% confidence interval: 1.14-21.0, p = 0.033). CONCLUSION: Preoperative sarcopenia defined using PMA was associated with poor mid-term survival after elective cardiac surgery via median sternotomy. The pectoralis muscle mass observed through a chest CT could be used for preoperative risk scoring in older patients undergoing cardiac surgery.

A Case of Thoracic Aortic Endovascular Repair of a Ruptured Mycotic Aortic Aneurysm Due to Pasteurella Multocida.

An 81-year-old man was admitted for general fatigue of one month's duration. Two sets of blood cultures revealed bacteremia, due to Pasteurella multocida, while computed tomography (CT) revealed a 47-mm descending aortic saccular aneurysm. After transfer to our hospital, the saccular aneurysm rapidly grew to 54 mm. An emergency thoracic endovascular aortic repair was performed, due to the aneurysm immediately rupturing after the CT scan. The patient was discharged on postoperative day 28.

Coronary Artery Stenosis Caused by Primary Malignant Pericardial Mesothelioma in a 76-Year-Old Man.

This report describes a 76-year-old man with diabetes mellitus who developed coronary artery stenosis from infiltration of a primary malignant pericardial mesothelioma. Three months before referral to the treating hospital, elevated liver function values and cardiac enzymes led to echocardiography, which revealed a motion abnormality in the anterior wall of the heart. The patient was diagnosed with congestive heart failure and admitted to the hospital, where chest computed tomography showed a tumor above the left atrial appendage that compressed the origin of the left anterior descending artery. He was referred to the treating hospital for surgery. Minimally invasive direct coronary artery bypass grafting was performed, but the mass was not resected because of its infiltrating nature and the potential for medical complications. Histologic examination of a biopsy specimen confirmed a primary malignant pericardial mesothelioma. The bypass procedure resolved the coronary artery stenosis caused by the tumor. Although the optimal treatment for primary malignant pericardial mesothelioma is controversial, minimally invasive methods, such as minimally invasive direct coronary artery bypass grafting, may be used successfully.

COL17A1 germline variant p.Ser1029Ala and mucosal malignant melanoma: An autopsy study.

Collagen type XVII α1 (COL17A1) encodes a hemidesmosomal protein at the epidermal-dermal junction and its variants are implicated in blistering skin diseases. Recent experiments in rodents revealed that Col17a1 has critical roles in stem cells of epidermal origin and in melanoma carcinogenesis. In the present study, it was investigated whether germline variants in COL17A1 are associated with skin cancer and other cancer types using indexed consecutive autopsy cases from the Japanese Geriatric Single Nucleotide Polymorphism database (n=2,343; mean age, 80 years). The database included 12 patients with skin cancer. A total of 53 COL17A1 missense variants on an exome chip were analyzed. One variant, p.Ser1029Ala (rs118166857), which had a minor allele frequency of 1.0%, exhibited a nominal positive sign of association with skin cancer [Fisher's exact P=0.002, odds ratio (OR)=16.93, 95% CI: 4.44-64.64]. This variant was detected in 2/2 patients with mucosal malignant melanoma (mMM) and 1/3 patients with extramammary Paget's disease, and in none of the patients with non-melanoma cancer, e.g., squamous cell and basal cell carcinoma. Other cancer types were searched in the database and the p.Ser1029Ala variant was indicated to be nominally associated with breast cancer (P=0.006, OR=4.17, 95% CI: 1.72-10.11). In the two mMM cases, targeted exome sequencing of 55 cancer-predisposing genes (including tumor protein 53, BRCA1/2 and mismatch repair genes) detected no apparent pathogenic variants, but revealed variants of unknown significance in axin 2, DNA directed polymerase ζ catalytic subunit and contactin 6. Since COL17A1 provides a niche for melanocyte stem cells, it was hypothesized that the p.Ser1029Ala variant in the COL17A1 ectodomain may affect the microenvironment, e.g., the cell competition. This is a working hypothesis generated from human autopsy cases and warrants further epidemiological and molecular biological validation.

An increased prothrombin time-international normalized ratio in patients with acute type A aortic dissection: contributing factors and their influence on outcomes.

PURPOSE: We investigated factors contributing to coagulopathy in patients with acute type A aortic dissection (ATAAD) and coagulopathy's influence on patient outcomes. METHODS: We grouped 420 patients who underwent ATAAD repair-none under anticoagulation therapy or with liver disease-by the prothrombin time-international normalized ratio (PT-INR) at admission: < 1.2 (no coagulopathy, n = 371), 1.2-1.49 (mild coagulopathy, n = 33), or ≥ 1.5 (severe coagulopathy, n = 16). We then compared the clinical presentation, dissection morphology, and outcomes among the groups. We assessed the PT-INR in relation to the preoperative hemodynamics and searched for factors predictive of a PT-INR ≥ 1.2. RESULTS: The transfusion volume and operation time were increased among patients with coagulopathy (P < 0.05). The in-hospital mortality (15.2-37.5% vs. 5.1%, P < 0.001) and 5-year survival (61.1-74.4% vs. 87.6%) were relatively poor for these patients. The median PT-INR was 1.03 (0.97-1.1) for patients with stable hemodynamics (n = 318), 1.11 (1.02-1.21) for those in shock (blood pressure < 80 mmHg) not given cardiopulmonary resuscitation (CPR) (n = 81), and 1.1 (1.0-1.54) for those in shock given CPR (n = 21) (P < 0.001). A multivariable analysis identified shock (P < 0.001), a partially thrombosed false lumen (P = 0.006), and mesenteric malperfusion (P = 0.016) as predictive variables. CONCLUSIONS: Shock, a partially thrombosed false lumen, and mesenteric malperfusion appear to be predictive of dissection-related coagulopathy, which influences outcomes negatively.

A case of right ventricular infarction due to acute type A aortic dissection with anomalous aortic origin of the right coronary artery from the left sinus of Valsalva.

Anomalous aortic origin of a coronary artery from the opposite sinus is a rare congenital condition that can cause sudden death in young people. When it is associated with acute aortic dissection, acute myocardial infarction can occur due to enlargement of the sinus of Valsalva. We report the case of a 71-year-old man with anomalous origin of the right coronary artery from the left sinus of Valsalva, who developed right ventricular infarction due to the compression of the right coronary artery between the aorta and pulmonary artery trunk.

Polymorphic SERPINA3-R124C reduces pathogenesis of its wild type by shortening the lifetime of oligomeric Aß.

Amyloid beta (Aß) 42 peptide accumulated in Alzheimer disease (AD) patients' brain, often colocalized with serine protease inhibitor family A member 3 (SERPINA3). Being a chaperon, SERPINA3 accelerated Aß42 fibrillization. While analyzing chaperon activity of human SERPINA3 polymorphisms, we found SERPINA3-R124C played a role in protecting cells from Aß42 cytotoxicity. SH-SY5Y cells exposed to Aß42 preincubated with wild-type SERPINA3 (SERPINA3-WT) resulted in extended toxicity leading cell death whereas Aß42 with SERPINA3-R124C resulted in less cytotoxicity. Transmission electron microscope and thioflavin T assay revealed that SERPINA3-R124C shortened lifetime of small soluble oligomer and maintained ß-sheet rich protofibril-like aggregates for longer time compared to that of with SERPINA3-WT. Western blot assay confirmed that SERPINA3-R124C converted Aß42 mostly into high molecular aggregates. Here, we demonstrate first time that polymorphic SERPINA3 acts as a benign chaperon by modulating the transition states of Aß42, which may contribute to the reduction of AD risk.

[Mitral Valve Repair via Right Thoracotomy Thirty-seven Years after Right Pneumonectomy:Report of a Case].

A 57-year-old man was referred to our hospital due to dyspnea on exertion with severe mitral regurgitation. Because he had underdone right pneumonectomy 37 years earlier due to congenital defect of the right pulmonary artery, his mediastinum was severely shifted to the right, and his pulmonary function was poor. Mitral valve repair was successfully performed with right thoracotomy approach, which made excellent exposure of the mitral valve. The patient was extubated three hours after the surgery. He was discharged on the 30th postoperative day without postoperative respiratory complications. With an appropriate plan of the surgery and postoperative optimal management, cardiac surgery can be performed on patients with a single lung.

Polymorphic SERPINA3 prolongs oligomeric state of amyloid beta.

Molecular chaperon SERPINA3 colocalizes with accumulated amyloid peptide in Alzheimer's disease (AD) patient's brain. From the QTL analysis, we narrowed down Serpina3 with two SNPs in senescence-accelerated mouse prone (SAMP) 8 strain. Our study showed SAMP8 type Serpina3 prolonged retention of oligomeric Aß 42 for longer duration (72 hr) while observing under transmission electron microscope (TEM). From Western blot results, we confirmed presence of Aß 42 oligomeric forms (trimers, tetramers) were maintained for longer duration only in the presences of SAMP8 type Serpina3. Using SH-SY5Y neuroblastoma cell line, we observed until 36 hr preincubated Aß 42 with SAMP8 type Serpina3 caused neuronal cell death compared to 12 hr preincubated Aß 42 with SAMR1 or JF1 type Serpina3 proteins. Similar results were found by extending this study to analyze the effect of polymorphism of SERPINA3 gene of the Japanese SNP database for geriatric research (JG-SNP). We observed that polymorphic SERPINA3 I308T (rs142398813) prolonged toxic oligomeric Aß 42 forms till 48 hr in comparison to the presence wild type SERPINA3 protein, resulting neuronal cell death. From this study, we first clarified pathogenic regulatory role of polymorphic SERPINA3 in neurodegeneration.