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Fibromuscular dysplasia (FMD) is an arterial disease characterized by fibrous arterial wall thickening and irregular proliferation and degeneration of smooth muscle cells in muscular arteries. Abdominal aortic aneurysms (AAA) are rare, with only a few reported cases. A characteristic feature of AAA is an aneurysm protruding forward near the terminal aorta with stenosis. Here, we report a rare case of a 66-year-old woman who underwent abdominal aortic replacement for a fusiform AAA, diagnosed with aortic FMD (without stenotic lesions) on pathological examination., (@ 2024 The Editorial Committee of Annals of Vascular Diseases.)

Background: Various cardiovascular diseases cause acquired von Willebrand syndrome (AVWS), which is characterized by a decrease in high-molecular-weight (large) von Willebrand factor (VWF) multimers. Mitral regurgitation (MR) has been reported as a cause of AVWS. However, much remains unclear about AVWS associated with MR., Objectives: To evaluate VWF multimers in MR patients and examine their impact on clinical characteristics., Methods: Moderate or severe MR patients ( n  = 84) were enrolled. VWF parameters such as the VWF large multimer index (VWF-LMI), a quantitative value that represents the amount of VWF large multimers, and clinical data were prospectively analyzed., Results: At baseline, the mean hemoglobin level was 12.9 ± 1.9 g/dL and 58 patients (69.0%) showed loss of VWF large multimers defined as VWF-LMI < 80%. VWF-LMI in patients with degenerative MR was lower than in those with functional MR. VWF-LMI appeared to be restored the day after mitral valve intervention, and the improvement was maintained 1 month after the intervention. Seven patients (8.3%) had a history of bleeding, 6 (7.1%) of whom had gastrointestinal bleeding. Gastrointestinal endoscopy was performed in 23 patients (27.4%) to investigate overt gastrointestinal bleeding, anemia, etc. Angiodysplasia was detected in 2 of the 23 patients (8.7%)., Conclusion: Moderate or severe MR is frequently associated with loss of VWF large multimers, and degenerative MR may cause more severe loss compared with functional MR. Mitral valve intervention corrects the loss of VWF large multimers. Gastrointestinal bleeding may be relatively less frequent and hemoglobin level remains stable in MR patients., (© 2024 The Authors.)

Background: Severe aortic stenosis (AS) causes acquired von Willebrand syndrome by the excessive shear stress-dependent cleavage of high molecular weight multimers of von Willebrand factor (VWF). While the current standard diagnostic method is so-called VWF multimer analysis that is western blotting under nonreducing conditions, it remains unclear whether a ratio of VWF Ristocetin co-factor activity (VWF:RCo) to VWF antigen levels (VWF:Ag) of <0.7, which can be measured with an automated coagulation analyzer in clinical laboratories and is used for the diagnosis of hereditary von Willebrand disease., Objectives: To evaluated whether the VWF:RCo/VWF:Ag is useful for the diagnosis of AS-induced acquired von Willebrand syndrome., Methods: VWF:RCo and VWF:Ag were evaluated with the VWF large multimer index as a reference, which represents the percentage of a patient's VWF high molecular weight multimer ratio to that of standard plasma in the VWF multimer analysis., Results: We analyzed 382 patients with AS having transaortic valve maximal pressure gradients of >30 mmHg, 27 patients with peripheral artery disease, and 46 control patients free of cardiovascular disease with osteoarthritis, diabetes, and so on. We assumed a large multimer index of <80% as loss of VWF large multimers since 59.0% of patients with severe AS had the indices of <80%, while no control patients or patients with peripheral artery disease, except for 2 patients, exhibited the indices of <80%. The VWF:RCo/VWF:Ag ratios, measured using an automated blood coagulation analyzer, were correlated with the indices (r s  = 0.470, P  < .001). When the ratio of <0.7 was used as a cut-off point, the sensitivity and specificity to VWF large multimer indices of <80% were 0.437 and 0.826, respectively., Conclusion: VWF:RCo/VWF:Ag ratios of <0.7 may indicate loss of VWF large multimers with high specificity, but low sensitivity. VWF:RCo/VWF:Ag ratios in patients with AS having a ratio of <0.7 may be useful for monitoring the loss of VWF large multimers during their clinical courses., (© 2023 The Author(s).)

BACKGROUND   : Anastomotic leakage at the distal anastomotic site after surgery for acute type A aortic dissection (AAD) occasionally leads to malperfusion or false lumen dilatation in the remnant dissected aorta. Open surgery remains the optimal therapy; however, subsequent surgery with re-sternotomy is excessively invasive in elderly and frail patients. CASE PRESENTATION: We report the case of an 81-year-old woman who was treated with chimney thoracic endovascular aortic repair (TEVAR) for anastomotic leakage after ascending aortic replacement for AAD. A pressure difference between the upper and lower limbs appeared after the primary surgery, and a computed tomography scan showed a stenotic true lumen in the descending aorta due to a "new tear" at the distal anastomotic site. Chimney TEVAR was performed to direct blood flow into the aortic true lumen in order to restore compromised blood flow to lower body because open surgery is excessively invasive for elderly and frail patients. No endoleak and a better expanded true lumen were detected after the subsequent surgical intervention. CONCLUSIONS: Chimney TEVAR combined with an arch debranching procedure for sealing the new entry at the distal anastomotic site after ascending aortic replacement for AAD may be a viable option for high-risk patients., (© 2023. The Author(s).)

Acquired von Willebrand syndrome (AVWS), characterized by reduced von Willebrand factor (VWF) large multimers, has recently been implicated as the principal mechanism underlying bleeding in patients implanted with left ventricular assist devices (LVADs). Hematological severity of AVWS varies among patients, even if an identical device is implanted. We investigated whether this diversity in hematological severity is due to individual variability in VWF fragility, according to responses to incremental shear stress. Whole-blood samples were sheared at 20,000-40,000 s -1 shear rate, an index of shear stress, using a custom-made shear stressor that could generate shear stress compatible with that produced by an LVAD. The degree of VWF large multimers degradation was evaluated using the VWF large multimer index. A significant inverse correlation was observed between the VWF large multimer index and LVAD-compatible magnitudes of shear stress: the VWF large multimer indices were 68.5 ± 18.3, 48.0 ± 13.9, 33.9 ± 12.1, 23.7 ± 7.9, and 18.7% ± 8.7% at 20,000, 25,000, 30,000, 35,000, and 40,000 s -1 of shear rates, respectively ( P < 0.0001). Furthermore, experimental VWF large multimer index values were compatible with those derived from patients with implanted LVADs (median; 28.9%). Finally, reduction in the VWF large multimer index corresponding to shear stress showed individual variation. We demonstrated that the combined use of a novel high shear stress loading device and quantitative evaluation of VWF large multimers may predict risk of bleeding before LVAD implantation., Competing Interests: Disclosure: There are no conflicts of interest to report., (Copyright © ASAIO 2021.)

Herein, we report a case of thoraco-abdominal aortic repair in a 55-year-old man with a multiple treatment history for aortic aneurysm and aortic dissection. A computed tomography scan revealed that the Adamkiewicz artery was connected to an occluded intercostal artery, suggesting that the left inferior epigastric artery was the key artery supplying the Adamkiewicz artery; the key artery was identified through direct monitoring of cerebrospinal fluid temperature and selective hypothermic perfusion. No spinal cord injury was detected during the postoperative period., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

This study investigated hemodynamic changes in the thoracic aorta and aortic arch branches before and after aortic valve replacement (AVR) by 4D-flow MRI in patients with aortic valve stenosis (AS). Thoracic 4D-flow MRI was performed in 10 AS patients before and after AVR (mean 27 ± 1.9 days). Fifteen aortic planes and 3 aortic arch branches planes were set to evaluate the mean volume flow rate in each plane during a cardiac cycle and the angle between the main flow direction in a specified plane and the axial direction of the aorta. We also focused on the distribution and magnitude of helicity density to evaluate the flow complexity. A significant increase in the volume flow rate after AVR was found in the ascending aorta (before 59.2 ± 8.7 mL/s vs after 77.3 ± 6.2 mL/s, P < 0.05) and the aortic arch branches (before 26.5 ± 2.8 mL/s vs after 35.8 ± 3.3 mL/s, P < 0.001). The flow angle significantly decreased in the ascending aorta (before 39.2 ± 2.7 degree vs after 25.2 ± 1.7°, P < 0.0001) and the arch aorta (before 19.3 ± 2.0 degree vs after 13.4 ± 0.9°, P < 0.001). The volume flow rate in the ascending aorta and the arch branches increased within 1 month after AVR, showing an increased blood supply to the upper body, including to the brain. The postoperative change was accompanied with an increased blood flow in the ascending aorta and a decreased flow complexity proximal to the arch branches., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Continuous flow-left ventricular assist devices (CF-LVADs) have become a therapeutic option in the management of advanced heart failure. Several studies show that patients with CF-LVAD are at an increased risk of gastrointestinal bleeding (GIB). However, few reports have presented the characteristics of GIB in Japanese populations. We investigated the incidence, etiology, and outcome of GIB in patients with CF-LVAD. Records of adult patients who received CF-LVADs between October 2008 and January 2017 were reviewed. GIB was defined as detection of bleeding sites by any type of diagnostic imaging. 54 patients received CF-LVAD, of which eight (14%) presented with overt GIB (12 events). GIB patients are significantly older (p = 0.04) and their pre-operative inferior vena cava diameter was larger (p = 0.02). Multivariate analysis revealed that the use of Jarvik 2000 (p = 0.003) was a risk factor for GIB. In total, 85.8% of patients were free from GIB at 1 year. The most common site was the small intestine (67%). The most common cause was angiodysplasia (50%). Six patients required blood transfusion (nine events) and four underwent endoscopic clippings (five events); however, no patients needed surgeries. The incidence of GIB in our cohort was similar to the global registry data. Double balloon endoscopy is useful for diagnosis and treatment of small intestinal lesions. Future efforts to further understand the incidence of GIB in Japanese populations by multicenter data are needed.

Objectives: Acquired von Willebrand syndrome, characterized by the reduction in von Willebrand factor (vWF) large multimers, has recently been considered as one of the causes of gastrointestinal bleeding (GIB). It remains unclear whether its haematological severity is linked with susceptibility to bleeding because the definition of the haematological severity of acquired von Willebrand syndrome has not been precisely determined. This study sought to establish a quantitative methodology to assess the haematological severity of acquired von Willebrand syndrome and to define the threshold for occurrence of GIB in patients implanted with left ventricular assist devices (LVADs)., Methods: In total, 41 patients treated with continuous-flow LVAD implanted between 2011 and 2017 at Tohoku University Hospital were investigated. vWF large multimers were quantitatively evaluated using the 'vWF large multimer index' defined as the ratio of a large multimer proportion in total vWF derived from a patient to that from a normal control. Using this index, the amount of vWF large multimers was expressed as a percentage of its normal control value obtained with a simultaneous analysis of each time measurement., Results: Twelve (29%) patients developed GIB events during follow-up periods (median 591 days) after an LVAD implantation. The vWF large multimer index in patients with GIB was significantly lower than that in those without GIB (25.0 ± 10.3% vs 37.5 ± 17.8%, P = 0.008). Most importantly, all patients experiencing GIB exhibited a vWF large multimer index below 40%., Conclusions: Patients with GIB exhibited a more severe loss of vWF large multimers. The vWF large multimer index may dictate the risk of GIB after an LVAD implantation., Clinical Trial Registration Number: UMIN000018135.

Background: Men score higher on mental rotation tasks compared to women and suffer from depression and anxiety at half the rate of women. The objective of this study was to confirm the gender-specific effects of depression and anxiety on mental rotation performance., Methods: We collected data in non-experimental conditions from 325 university students at three universities. Participants completed rating scales of depressive and anxiety symptoms, and then simultaneously performed a mental rotation task using tablet devices., Results: We observed no significant difference between men and women in the depressive and anxiety symptoms and task response time. Men had a significantly higher correct answer rate compared with women. The scores of depression and anxiety of all participants were positively correlated. Task response time correlated positively with intensity of depressive symptoms and anxiety in women, but not in men. Women with high depressive symptoms had significantly longer response times than did women with low depressive symptoms, while men had no differences due to depressive symptoms., Limitations: We did not directly examine brain functions; therefore, the underlying neurobiological results are only based on previous knowledge and action data., Conclusions: The pathology of depression and anxiety was reflected in the correct answer rate and response time in relation to the gender difference of brain function used in mental rotation., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Heparin resistance (HR) is often encountered during cardiovascular operations that require cardiopulmonary bypass. Clinical risk factors and the mechanism underlying heparin resistance are yet to be determined. The aim of this study was to elucidate the clinically valid preoperative predictors related to HR., Methods: The study evaluated 489 patients undergoing cardiovascular operations. Of these, 25 patients presented with HR and received antithrombin III for the initiation of cardiopulmonary bypass with an effective activated coagulation time. The remaining 464 patients, who did not receive antithrombin III, served as controls (NHR). Preoperative patient demographic and laboratory data were analyzed to identify risk factors for HR., Results: The preoperative laboratory data showed platelet count, fibrinogen, D-dimer, creatinine, and C-reactive protein were significantly higher in the HR group than in the NHR group. As expected, the antithrombin III level was significantly lower overall in the HR group (86.0% vs 95.5%, p = 0.009); however, 80% of the patients in the HR group showed normal antithrombin III levels preoperatively. Multivariable logistic regression analysis identified chronic aortic dissection, chronic obstructive pulmonary disease, smoking, and elevated fibrinogen levels as independent predictors for HR., Conclusions: HR was shown to be associated with preoperative high fibrinogen levels, a smoking habit, and a preoperative diagnosis of chronic, but not acute, aortic dissection, with chronic obstructive pulmonary disease as comorbidity. Administration of antithrombin III resolved HR in all of the affected patients, even when their preoperative antithrombin III level was within the normal limit., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Patients supported by mechanical circulatory support have to wait for longer periods for heart transplantation in Japan. Infective events are a major complication and influence survival. Here, we present the case of a patient with an implantable left ventricular assist device for 6 months who had the complication of ruptured infective common iliac aneurysm. Graft placement with an omental flap was successfully performed via the alternative surgical approach to avoid percutaneous driveline injury. In samples of aortic specimens, 16S ribosomal DNA gene analysis identified Helicobacter cinaedi. Complete removal of the infected tissue and correct pathogen identification may have been relevant to the good clinical course.

Objective: We evaluated the effect of cerebrospinal fluid oxygenation for the prevention of spinal cord ischemic injury after infrarenal aortic occlusion in a rabbit model., Methods: Twenty white Japanese rabbits were categorized into the following 4 groups (5 in each): group S (sham), balloon catheter insertion on to the aorta; group C (control), spinal cord ischemic injury by infrarenal abdominal aortic balloon occlusion for 15 minutes; group N (nonoxygenated), spinal cord ischemic injury with cerebrospinal fluid replacement by nonoxygenated artificial cerebrospinal fluid; and group O (oxygenated), spinal cord ischemic injury with cerebrospinal fluid replacement by nanobubble-oxygenated artificial cerebrospinal fluid. The changes in cerebrospinal fluid partial pressure of oxygen during the peri-ischemic period, modified Tarlov score, and histopathology of the spinal cord 48 hours after aortic maneuvers were evaluated., Results: Cerebrospinal fluid partial pressure of oxygen significantly increased in group O compared with group N after cerebrospinal fluid replacement (254.5 ± 54.8 mm Hg vs 136.1 ± 43.5 mm Hg, P = .02). After 15 minutes of spinal cord ischemic injury, cerebrospinal fluid partial pressure of oxygen in group C decreased to 65.8 ± 18.6 mm Hg compared with baseline (148.8 ± 20.6 mm Hg, P < .01), whereas cerebrospinal fluid partial pressure of oxygen in group O was maintained at remarkably high levels after spinal cord ischemic injury (291.9 ± 51.8 mm Hg), which was associated with improved neurologic function, with 20% of spinal cord ischemic injury having a Tarlov score less than 5 compared with 100% of spinal cord ischemic injury in group C. Preservation of anterior horn neurons in groups N and O was confirmed by histopathologic analysis with significant reduction of degenerated neurons compared with group C., Conclusions: Cerebrospinal fluid oxygenation with artificial cerebrospinal fluid can exert a protective effect against spinal cord ischemic injury in rabbits., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

A 61-year-old man was implanted with a Jarvik 2000, a continuous axial flow type left ventricular assist device (LVAD), for end-stage heart failure due to dilated cardiomyopathy. One month later, his postoperative course was complicated with intractable oozing-type gastrointestinal bleeding from multiple small shallow ulcers and erosions in the colon. In addition, repeated bleeding episodes were encountered at around thoracentesis site for pleural effusion. Hematological examination showed that platelet counts and coagulation factors were kept within normal ranges. We, thereafter, revealed remarkable loss of the large multimers of von Willebrand factors (VWFs), which might be closely associated with his intractable bleeding tendency.

Tolvaptan is a new selective vasopression V2-receptor antagonist. We report our experience with a use of tolvaptan for preoperative fluid management in a patient with severe constrictive pericarditis. A 66-year-old man presented with heart failure symptoms derived from constrictive pericarditis. Chest X-ray showed right pleural effusion and chest computed tomography demonstrated severe pericardial calcification. Despite that he received optimal conventional medical treatments, his hemodynamic condition further exacerbated during hospitalization. We administered tolvaptan in an attempt to optimize preoperative fluid management. Tolvaptan was found to be remarkably effective in that regard. The body weight decreased and the heart failure symptoms improved. Pericardiectomy was performed successfully, and he recovered uneventfully.

Background: The aim of this study was to examine and compare the areas of brain blood flow in patients with chronic low back pain (CLBP) without structural abnormality and acute low back pain (ALBP) with lumber disc herniation (LDH). Functional neuroimaging studies provide evidence of abnormalities in the regional cerebral blood flow during low back pain. Recent studies have shown that CLBP is associated with plastic, pathophysiological changes in the brain. However, there has been no report yet statistically or by neuro-images on the compared brain single photon-emission computed tomography (SPECT) findings between CLBP and ALBP patients., Methods: The subjects comprised 14 patients, 7 CLBP and 7 ALBP patients. The CLBP group included the patients who had no or minor structural abnormality in the lumbar spine on magnetic resonance imaging (MRI) and met the criteria for a classification of "pain disorder" (chronic) according to the Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision. The ALBP group included the patients who had symptoms within 3 months of onset and LDH revealed by MRI. All patients were assessed using brain SPECT. We then performed a two-tailed view analysis using the easy Z score imaging system, determined the mean Z scores, and performed vBSEE software (Fujifilm RI Pharma, Tokyo, Japan) for both CLBP and ALBP patients., Results: The CLBP group showed significantly reduced blood flow in the bilateral prefrontal cortex of the frontal lobe and increased blood flow in the bilateral posterior lobe of the cerebellum., Conclusions: SPECT images and statistical analyses revealed the brain blood flow alterations in the patients with ALBP and CLBP. These results may suggest that the dysfunction of the prefrontal cortex could lead to the appearance of unconscious pain behavior controlled by the cerebellum in the patients with CLBP.

A 78-year-old man who fell from a step ladder was transported to our hospital by ambulance under the diagnosis of multiple rib fractures and right hemothorax. Since he was in shock on arrival, endotracheal intubation and tube thoracotomy were immediately performed. Though 2 liters of blood was evacuated, persistent hemorrhage was observed, requiring continuous rapid infusion and blood transfusion. Emergency thoracic arteriography revealed active bleeding from a branch of the right internal thoracic artery. Transcatheter arterial embolization (TAE) was performed using vascular embolization coils and porous gelatin particles. These procedures successfully controlled active hemorrhage from the chest. Intrathoracic hematoma was evacuated through the 2nd large chest tube. Chest tubes were removed on the 7th day. He was discharged on the 17th day without any complications.

UVA1 induces the formation of 8-hydroxy-2'-deoxyguanosines (8-OH-dGs) and cyclobutane pyrimidine dimers (CPDs) in the cellular genome. However, the relative contribution of each type of damage to the in vivo genotoxicity of UVA1 has not been clarified. We irradiated living mouse skin with 364-nm UVA1 laser light and analyzed the DNA damage formation and mutation induction in the epidermis and dermis. Although dose-dependent increases were observed for both 8-OH-dG and CPD, the mutation induction in the skin was found to result specifically from the CPD formation, based on the induced mutation spectra in the skin genome: the dominance of C --> T transition at a dipyrimidine site. Moreover, these UV-specific mutations occurred preferentially at the 5'-TCG-3' sequence, suggesting that CpG methylation and photosensitization-mediated triplet energy transfer to thymine contribute to the CPD-mediated UVA1 genotoxicity. Thus, it is the CPD formation, not the oxidative stress, that effectively brings about the genotoxicity in normal skin after UVA1 exposure. We also found differences in the responses to the UVA1 genotoxicity between the epidermis and the dermis: the mutation induction after UVA1 irradiation was suppressed in the dermis at all levels of irradiance examined, whereas it leveled off from a certain high irradiance in the epidermis.

An experiment was conducted to analyze the space-movement processes which unilateral spatial neglect (USN) patients manifested. Three kinds of movement features were tested which were reported in previous studies; (a) general coarse response tendency, (b) hypokinesia in the disordered space-side, and (c) more frequent response toward the normal space-side. Five left USN patients with right brain damage were compared with five non-USN right brain damaged patients, and five normal controls. As an experimental movement task, a non-visual movement reproduction with regard to kinesthetic sensory modality was adopted. Subjects were blindfolded and asked to reproduce the criterion-movement (length of 10 cm) toward right hand side in space. Three different experimental spaces were used in relation to the subject's body positions; 'left space (range from left shoulder to median line)', 'right space (from median line to right shoulder)', and 'right-outside space (outside of right shoulder)'. The criterion-movement was presented to the subjects, and then they reproduced the movement in either one of these three spaces. Generally, the left USN patients reproduced movements with more errors and less accuracy when the execution side was in the 'left space'. The results confirmed the observation that left USN patients showed more frequent response toward the right hand side direction in the 'left space'. The left USN patients with lesions in frontal and/or basal ganglia regions showed larger errors in this task compared to the left USN patient whose lesion is localized in the parietal region in the right hemisphere.