[Embolization Using a Debranched Left Common Carotid Artery Stump for Gutter Endoleak After Thoracic Endovascular Aortic Repair with the Double-debranching and Chimney Technique].

An 88-year-old male underwent thoracic endovascular aortic repair (TEVAR) with the double-debranching and chimney technique for arch aortic aneurysm. When the aforementioned procedure was performed, the left common carotid artery was closed and transected, and the left subclavian artery was embolized and bypassed, respectively. However, postoperatively, the gutter endoleak persisted, and the aneurysm enlarged;therefore, requiring additional surgery. A skin incision was made on the left side of the neck, and the closed and dissected left common carotid artery stump was detected. A sheath was placed at the stump and an angiographic catheter and guidewire were used to retrograde cannulate the gutter beside the chimney graft, and coil embolization was performed. No endoleak was observed at postoperatively and 6-month follow up computed tomography( CT). We believe that embolization from a deblanched left common carotid artery stump is useful for endoleaks after TEVAR employing the chimney and debranching technique.

A New Case With Cortical Malformation Caused by Biallelic Variants in LAMC3.

Objective: In this study, we report the case of a 24-year-old man with intellectual disability and childhood-onset seizures. This patient had newly identified biallelic variants in the laminin subunit gamma 3 (LAMC3) gene with unreported cortical malformation. Methods: Exome sequencing. Results: Genetic analyses revealed new biallelic variants in the LAMC3 gene. An MRI examination of the brain revealed cortical malformations predominantly in the temporal lobes and mildly in the occipital, frontal, and parietal lobes. In addition, our patient also exhibited mild midline malformation in the ventral pons, which is unique to LAMC3 variants. Discussion: Patients with LAMC3 variants have been reported to exhibit cortical malformation predominantly in the occipital lobes, but this patient exhibited cortical malformation predominantly in the temporal lobes and mildly in the occipital, frontal, and parietal lobes. In addition, this patient also exhibited mild midline malformation in the ventral pons. These unique findings cast new light on the role of LAMC3 in brain development.

Clinical impact of 11C-Pittsburgh compound-B positron emission tomography in addition to magnetic resonance imaging and single-photon emission computed tomography on diagnosis of mild cognitive impairment to Alzheimer's disease.

ABSTRACT: This study aimed to evaluated the clinical impact of adding [11C] Pittsburgh compound-B (11C-PiB) PET for clinical diagnosis of mild cognitive impairment (MCI) to Alzheimer's disease (AD) dementia.Twenty six (mean age 78.5 ±â€Š5.18 years, 21 females) AD (n = 7), amnestic MCI (n = 12), non-amnestic MCI (n = 3), vascular dementia, progressive supranuclear palsy (PSP) with frontotemporal dementia (FTD), FTD (n = 1 each), and normal (n = 1) patients underwent 11C-PiB-PET, MRI, and SPECT scanning. 11C-PiB-PET was compared with MRI and SPECT for clinical impact.11C-PiB-PET showed positivity in 6, 9, and 0 of the AD, amnestic MCI, and non-amnestic MCI patients, respectively, and 0 of those with another disease. Parahippocampal atrophy at VSASD was observed in 5 AD patients, 6 amnestic and PiB-positive MCI patients, 1 amnestic and PiB-negative MCI patient, and 1 vascular dementia patient. Parietal lobe hypoperfusion in SPECT findings was observed in 6, 4, and 2 of those, respectively, as well as 1 each of non-amnestic MCI, vascular dementia, and normal cases. Sensitivity/specificity/accuracy for selecting PiB-positive patients among the 15 MCI patients for 11C-PiB-PET were 100% (9/9)/100% (6/6)/100% (15/15), for VSRAD were 66.7% (6/9)/83.3% (5/6)/73.3% (11/15), and for SPECT were 44.4% (4/9)/50.0% (3/6)/46.7% (7/15), while those were 88.9% (8/9)/33.3% (2/6)/66.7% (10/15)/for combined VSRAD and SPECT. 11C-PiB-PET accuracy was significantly higher than that of SPECT.11PiB-PET alone may be useful for selecting patients who will progress from MCI to AD in the future, although follow-up study is necessary to clarify the outcome of MCI patients.

[Re-operation Using Bovine Pericardium for Infective Endocarditis after Aortic Valve Neocuspidization;Report of a Case].

A 70-year-old man, who had undergone aortic valve neocuspidization using his own pericardium 8 months before, complained of back pain, and was diagnosed with pyrogenic spondylitis. As the result of blood culture, Enterococcus faecalis was found to be the causative bacterium, and antibiotic therapy was started. Six days after admission, hemodynamics collapsed suddenly, and percutaneous cardio-pulmonary support was established. Echocardiography showed severe aortic valve regurgitation, and he was diagnosed with active infective endocarditis. We performed re-do aortic valve neocuspidization using bovine pericardium. There was a tear on the non-coronary cusp and the cusps were thickened because of infection. Aortic annular tissue was not destroyed and we could fix the neo-valve directly to the annulus. After these procedures, severe reduction of antero-septal wall motion was noted, which suggested dissection of the main trunk of the left coronary artery. Coronary artery bypass grafting to the left anterior descending and the circumflex branches was added. The patient came off percutaneous cardio-pulmonary support 5 days after surgery. Although trivial aortic regurgitation remained, he was discharged after 2 months of rehabilitation.

[Reports of 12 Cases of Stanford Type A Acute Aortic Dissection Emergent Surgery for Jehovah's Witnesses patients].

The Jehovah's Witnesses (JW) is well known for declining blood transfusions. Especially, cardiovascular surgery on JW poses unique challenges. We herein report 12 JW emergent cases of Stanford type A acute aortic dissection which underwent graft replacement between 2003 and 2019. Graft replacement of ascending aorta was performed in all cases. Operative time and anesthetic time were 344±100 and 396±109 minutes respectively. The mean intraoperative hemoglobin nadir was 4.9±1.2 g/dl. The postoperative hemoglobin nadir was 6.3 ±2.4 g/dl. There were 2 deaths within 24 hours after surgery. We did not transfuse any packed red blood cells, fresh frozen plasma or platelets for JW patients of Stanford type A acute aortic dissection surgery.

Transcatheter aortic valve implantation after aortic valve neocuspidization using autologous pericardium: a case report.

BACKGROUND: Aortic valve neocuspidization (AVNeo), a novel surgical procedure used in the treatment of aortic valve diseases, including aortic stenosis (AS), involves the replacement of three aortic valve cusps by glutaraldehyde-treated autologous pericardium. Although reoperation risk is low, no case report on the deterioration of the AVNeo has yet been published. CASE SUMMARY: An 80-year-old woman who underwent AVNeo for severe degenerative tricuspid AS 6 years previously complained of shortness of breath. Echocardiographic assessment revealed the reconstructed aortic valve leaflet was elongated, thickened, and marginally calcified resulting in recurrent severe AS. Transcatheter aortic valve implantation using balloon-expandable transcatheter heart valve was successfully performed. DISCUSSION: To our knowledge, this is the first case report regarding the structural deterioration of the AVNeo resulting in restenosis 6 years after the first surgery. Transcatheter aortic valve implantation is possibly a suitable approach for post-procedural recurrence after AVNeo to avoid redo open-heart surgery which would be of prohibitive risk especially in an elderly population.

[Aortoesophageal Fistula with Infected Thoracic Aortic Aneurysm Rupture;Report of a Case].

A 67-year-old man was referred to our hospital because of fever and discomfort of the throat. Gastrointestinal endoscopy revealed hematoma at the middle thoracic esophagus. Computed tomography revealed posterior mediastinal hematoma extending the descending aorta. Bacillus was detected in the blood culture. Aortoesophageal fistula with an infected thoracic aortic aneurysm rupture was diagnosed. First, thoracic endovascular aortic repair (TEVAR) was performed. Resection of the thoracic esophagus and omentopexy was conducted 15 days after TEVAR. Esophageal reconstruction using a gastric tube was performed 43 days after esophagectomy. He has been doing well since then.

Nutritional status and management of risks due to physical therapy in patients hospitalized for long periods.

[Purpose] This study aimed to understand the nutritional status of patients hospitalized for long periods and the risk of physical therapy (PT) for such patients. [Subjects and Methods] Participants were selected from patients who were hospitalized at a designated medical long-term care sanatorium. The participants were divided into 5 groups (A-E) depending on their mode of energy intake and ambulatory ability during PT. The serum albumin level, energy intake, total daily energy expenditure, and total daily energy expenditure per session of PT (EEPT) were evaluated for each group. [Results] Protein-energy malnutrition was observed in 69.6% of the participants. No significant association was identified between the serum albumin level and body mass index. Energy intake was significantly higher in Groups D and E, whose energy intake was via ingestion, than in Groups A and B, whose intake was via tube feeding. EEPT was highest in patients of Group E who had gait independence different from the ability of those in groups A-D. [Conclusion] The actual energy intake is lower with tube feeding than with ingestion. Risk management and energy intake should be revisited in elderly patients who have been hospitalized for long periods and subsequently obtain gait independence.

Patient with Niemann-Pick disease type C: over 20 years' follow-up.

We report a 37-year-old woman with Niemann-Pick disease type C (NPC) 1. At the age of 8 years, she presented slow running followed by both fingers dystonia at the age of 10 years. At the age of 16 years, she developed declined scholastic achievement. On her first visit at the age of 17 years, she showed dystonia, ataxic gait and vertical supranuclear gaze palsy. We suspected it was NPC. She presented atrophies in the frontal lobes, brainstem and cerebellum in a brain MRI. She presented hepatomegalies and splenomegalies in an abdominal CT. At the age of 26 years, she undertook perpetually tracheal fistula because of recurrent aspiration pneumonia. Diagnosis of NPC1 was made by filipin staining and existence of foamy cells in the bone marrow and NPC1 gene analysis. We obtained informed consent of genetic analysis. Miglustat therapy was started at the age of 32 years. Improvements in swallowing capacity and in muscle tonus were seen.

Over 10†years MRI observation of a patient with neuronal intranuclear inclusion disease.

We present a sporadic neuronal intranuclear inclusion disease (NIID) patient with neuropathy followed by cognitive dysfunction along with brain MRIs findings of leucoencephalopathy. Her cognitive impairment gradually progressed along with abnormal intensity lesions in diffusion-weighted images. This pathological and clinical deterioration resemble pathological process in prion diseases.

[Thoracic Endovascular Aortic Repair for a Descending Thoracic Aneurysm Associated with Pseudocoarctation of the Aorta;Report of a Case].

A 77-year-old man with a descending thoracic aneurysm associated with pseudocoarctation of the aorta underwent thoracic endovascular aortic repair (TEVAR). Computed tomography demonstrated kinking of the descending thoracic aorta. The aneurysm was located distal to a kinking and constricting aortic isthmus. TEVAR was performed because of the patient's comorbidity such as valvular heart diseases and postradiation status for the thoracic Hodgkin disease. TEVAR was successful but small aortic dissection was occurred distal to the stentgraft. Computed tomography at 1 year after TEVAR demonstrated reduction of the aneurysmal diameter and no change in the small dissection. TEVAR is becoming the treatment of first choice for this disease.

Outcomes of limited proximal aortic replacement for type A aortic dissection in octogenarians.

OBJECTIVE: The number of older patients with acute aortic dissection type A (AAD [A]) is increasing as the population ages. We evaluated clinical outcomes for octogenarians with AAD (A) treated surgically at our hospital. Whenever possible, we limited the replacement site of the ascending aorta to the supracoronary and hemiarch. METHODS: Of 436 patients with AAD (A) seen in our hospital emergency room between April 2001 and August 2015, 90 were octogenarians. Surgery was performed using a simple cardiopulmonary bypass established through the right femoral artery and venous cannulation, and distal anastomosis was performed under deep hypothermic circulatory arrest at 20°C. RESULTS: Of the 90 octogenarians with AAD (A), 11 required cardiopulmonary resuscitation, 9 of whom died. Four patients with stable hemodynamics refused surgery. Thus, 77 were treated surgically. Of these 77 patients, isolated replacement of the ascending aorta or hemiarch was performed in 73 (94.8%), and total arch replacement in 4 (5.2%). Five patients (6.5%) died within 30 days, and 5 (6.5%) died in the hospital more than 30 days after surgery. Seven (9.1%) had a new stroke, 5 (6.5%) had pneumonia, and 4 (5.2%) had mediastinitis. Forty-four (57.1%) patients were discharged to their homes and 23 (30%) to rehabilitation hospitals. Three patients later required endovascular stent graft repair, which was successful in each case. The overall postoperative survival was 82%, 70%, and 62% at 1, 3, and 5 years, respectively. CONCLUSIONS: Our results suggest that our limited replacement protocol for emergency AAD (A) surgery has early and midterm survival benefits for octogenarians.

Massively parallel sequencing-based survey of eukaryotic community structures in Hiroshima Bay and Ishigaki Island.

In this study, we compared the eukaryote biodiversity between Hiroshima Bay and Ishigaki Island in Japanese coastal waters by using the massively parallel sequencing (MPS)-based technique to collect preliminary data. The relative abundance of Alveolata was highest in both localities, and the second highest groups were Stramenopiles, Opisthokonta, or Hacrobia, which varied depending on the samples considered. For microalgal phyla, the relative abundance of operational taxonomic units (OTUs) and the number of MPS were highest for Dinophyceae in both localities, followed by Bacillariophyceae in Hiroshima Bay, and by Bacillariophyceae or Chlorophyceae in Ishigaki Island. The number of detected OTUs in Hiroshima Bay and Ishigaki Island was 645 and 791, respectively, and 15.3% and 12.5% of the OTUs were common between the two localities. In the non-metric multidimensional scaling analysis, the samples from the two localities were plotted in different positions. In the dendrogram developed using similarity indices, the samples were clustered into different nodes based on localities with high multiscale bootstrap values, reflecting geographic differences in biodiversity. Thus, we succeeded in demonstrating biodiversity differences between the two localities, although the read numbers of the MPSs were not high enough. The corresponding analysis showed a clear seasonal change in the biodiversity of Hiroshima Bay but it was not clear in Ishigaki Island. Thus, the MPS-based technique shows a great advantage of high performance by detecting several hundreds of OTUs from a single sample, strongly suggesting the effectiveness to apply this technique to routine monitoring programs.