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The medial temporal lobe (MTL) is a hotspot for neuropathology, and measurements of MTL atrophy are often used as a biomarker for cognitive decline associated with neurodegenerative disease. Due to the aggregation of multiple proteinopathies in this region, the specific relationship of MTL atrophy to distinct neuropathologies is not well understood. Here, we develop two quantitative algorithms using deep learning to measure phosphorylated tau (p-tau) and TDP-43 (pTDP-43) pathology, which are both known to accumulate in the MTL and are associated with MTL neurodegeneration. We focus on these pathologies in the context of Alzheimer's disease (AD) and limbic predominant age-related TDP-43 encephalopathy (LATE) and apply our deep learning algorithms to distinct histology sections, on which MTL subregions were digitally annotated. We demonstrate that both quantitative pathology measures show high agreement with expert visual ratings of pathology and discriminate well between pathology stages. In 140 cases with antemortem MR imaging, we compare the association of semi-quantitative and quantitative postmortem measures of these pathologies in the hippocampus with in vivo structural measures of the MTL and its subregions. We find widespread associations of p-tau pathology with MTL subregional structural measures, whereas pTDP-43 pathology had more limited associations with the hippocampus and entorhinal cortex. Quantitative measurements of p-tau pathology resulted in a significantly better model of antemortem structural measures than semi-quantitative ratings and showed strong associations with cortical thickness and volume. By providing a more granular measure of pathology, the quantitative p-tau measures also showed a significant negative association with structure in a severe AD subgroup where semi-quantitative ratings displayed a ceiling effect. Our findings demonstrate the advantages of using quantitative neuropathology to understand the relationship of pathology to structure, particularly for p-tau, and motivate the use of quantitative pathology measurements in future studies., (© 2024. The Author(s).)

During the coronavirus 2019 (COVID-19) pandemic, children suffered severe lung injury resulting in acute respiratory distress syndrome requiring support with extracorporeal membrane oxygenation (ECMO). In this case series, we described our center's experience employing a rehabilitation-focused ECMO strategy including extubation during ECMO support in four pediatric patients with acute COVID-19 pneumonia hospitalized from September 2021 to January 2022. All four patients tolerated extubation within 30 days of ECMO initiation and achieved mobility while on ECMO support. Duration of ECMO support was 35-152 days and hospital lengths of stay were 52-167 days. Three of four patients survived. Two of three survivors had normal functional status at discharge except for ongoing respiratory support. The third survivor had significant motor deficits due to critical illness polyneuropathy and was supported with daytime oxygen and nocturnal noninvasive support. Overall, these patients demonstrated good outcomes and tolerance of a rehabilitation-focused ECMO strategy., Competing Interests: Disclosure: The authors have no conflicts of interest to report., (Copyright © ASAIO 2024.)

A 51-year-old patient with progressive right heart dysfunction was found to have a large calcified right atrial mass on echocardiography. As part of the work up for an intracardiac mass he had a cardiac computed tomogram which detailed a large coronary cameral fistula from the circumflex coronary artery to the right atrium associated with a spherical calcific pseudo-aneurysmal sac. Transcatheter occlusion of the exit point into the atrium with a vascular plug was performed directly from a right atrial approach without the need for an arteriovenous wire loop. This case details a unique presentation of a coronary cameral fistula to an unusual position within the right atrium which facilitated the rare ability to occlude the fistula from a venous approach without creating an arteriovenous wire rail., (© 2024 Wiley Periodicals LLC.)

Percutaneous carotid access (PCA) in infants has been reported in small multicenter cohorts, case reports and wider studies over the last 20 years. Compare outcomes after implementation of a systematic approach to PCA in a single center including an imaging follow-up protocol. Retrospective case-control study of PCA at Children's Hospital Colorado was performed from January 2013 to December 2022. Seventy-four patients underwent 82 PCAs for cardiac catheterization. The median age (range) was 14 days (1-359), and weight was 3.25-kg (1.9-7.9). Median sheath size was 4-Fr (3.3-6). Seventy-seven interventions performed included PDA stenting, aortic valvoplasty, BTT shunt stenting, and coarctation stenting. Vascular access was performed using a modified 21 g butterfly needle. A protocolized approach was implemented in 2020 reversing the patient head-to-toe orientation on the catheterization table, maintaining intubation and sedation for 4-h during recovery and routine use of a specific vascular ultrasound protocol. Following these changes, time to access significantly improved with no major complications. Before 2020, two access related complications occurred. One requiring surgical vascular repair and one occlusive thrombus. A significant increase in sheath time in post-era was associated with increased case complexity. Longer sheath times were not associated with increased risk of vessel injury or thrombus. No neurological insults were reported. Our experience confirms that PCA is safe and achievable with preserved vessel patency regardless of patient weight or sheath size. A protocolized planning, recovery, and follow-up regimen is recommended to establish safe practice and identify and treat complications as necessary., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

We examined the results of cardiac catheterization in infants with congenital diaphragmatic hernia (CDH) from 2009 to 2020. Catheterization confirmed pulmonary arterial hypertension in all cases (n =  17) and identified left ventricular (LV) diastolic dysfunction (LVDD) in 53%. LVDD was associated with greater respiratory morbidity. Preprocedural noninvasive assessment showed inconsistent agreement with catheterization results., (Copyright © 2022 Elsevier Inc. All rights reserved.)

We present the first clinical experience with a new hybrid cell structure covered stent, designed for congenital heart disease applications. It represents a significant redesign of the Cheatham Platinum (CP) Stent (Numed Inc.), maintaining the traditional benefits of the covered CP whilst significantly decreasing shortening and allowing controlled flaring at the ends through its combination of larger and standard sized cells. We first implanted the stent in 2 patients with superior sinus venosus defects with anomalous drainage of the right upper and middle lobe pulmonary veins. The first was a 40 year male and the second a 36 year old female. The third case was a 60 year old patient with near atresia of the aorta, with pre and poststenotic aortic dilation. The clinical result in all cases was excellent with no obstruction to pulmonary venous return and no visible L-R shunt on the transthoracic echo on 24 h and 2 week follow-up for the patient with sinus venosus defects and uniform complete revascularization of the aorta without any vascular complications in the patient with coarctation. These are the first uses of this stent in human subjects. The design is specifically aimed toward procedures where stent shortening is undesirable. Hence, coarctation of the aorta as well as stent implantation in preparation for percutaneous pulmonary valve placement are obvious use areas, as well as the growing body of evidence supporting percutaneous treatment of sinus venosus defects., (© 2022 Wiley Periodicals LLC.)

Introduction: Percutaneous pulmonary valve replacement (PPVI) continues to gather pace in pediatric and adult congenital practice. This is fueled by an expanding repertoire of devices, techniques and equipment to suit the heterogenous anatomical landscape of patients with lesions of the right ventricular outflow tract (RVOT). Contrast-induced nephropathy is a real risk for teenagers and adults with congenital heart disease (CHD)., Aim: To present a series of patients who underwent PPVI without formal RVOT angiography and propose case selection criteria for patients who may safely benefit from this approach., Material and Methods: We retrospectively collected PPVI data from the preceding 2 years at our institution identifying patients who had been listed as suitable for consideration for contrast-free PPVI from our multidisciplinary team (MDT) meeting based on predefined criteria. Demographic, clinical, imaging and hemodynamic data were collected. Data were analyzed using SPSS., Results: Twenty-one patients were identified. All patients had a technically successful implantation with improvements seen in invasive and echocardiographic hemodynamic measurements. 90% of patients had a bio-prosthetic valve (BPV) in situ prior to PPVI. One patient had a complication which may have been recognized earlier with post-intervention RVOT contrast injection., Conclusions: Zero-contrast PPVI is technically feasible and the suitability criteria for those who might benefit are potentially straightforward. The advent of fusion and 3D imaging in cardiac catheterization laboratories is likely to expand our capacity to perform more procedures with less contrast. Patients with bio-prosthetic valves in the pulmonary position may benefit from contrast-free percutaneous pulmonary valve implantation., Competing Interests: Dr Morgan is a proctor for Edwards Lifesciences Corporation. The remaining declare no conflict of interest., (Copyright: © 2021 Termedia Sp. z o. o.)

Objectives and Background: Coarctation of the aorta represents 5-8% of all congenital heart disease. Although balloon expandable stents provide an established treatment option for native or recurrent coarctation, outcomes from transverse arch (TAO) stenting, including resolution of hypertension have not been well studied. This study aims to evaluate immediate and midterm results of TAO stenting in a multi-center retrospective cohort., Methods: TAO stenting was defined as stent placement traversing any head and neck vessel, with the primary intention of treating narrowing in the transverse aorta. Procedural details, complications and medications were assessed immediately post procedure, at 6 month follow-up and at most recent follow-up., Results: Fifty-seven subjects, 12 (21%) native, and 45 (79%) surgically repaired aortic arches, from seven centers were included. Median age was 14 years (4 days-42 years), median weight 54 kg (1.1-141 kg). After intervention, the median directly measured arch gradient decreased from 20 mmHg (0-57 mmHg) to 0 mmHg (0-23 mmHg) (p < .001). The narrowest arch diameter increased from 9 mm (1.4-16 mm) to 14 mm (2.9-25 mm) (p < .001), with a median increase of 4.9 mm (1.1-10.1 mm). One or more arch branches were covered by the stent in 55 patients (96%). There were no serious adverse events. Two patients warranted stent repositioning following migration during deployment. There were no late complications. There were 8 reinterventions, 7 planned, and 1 unplanned (6 catheterizations, 2 surgeries). Antihypertensive management was continued in 19 (40%) at a median follow-up of 3.2 years (0.4-7.3 years)., Conclusions: TAO stenting can be useful in selected patients for resolution of stenosis with minimal complications. This subset of patients are likely to continue on antihypertensive medications despite resolution of stenosis., (© 2020 Wiley Periodicals LLC.)

Background: The Edwards SAPIEN valve and its delivery system may complicate transit through the right heart during transcatheter pulmonary valve replacement (tPVR). We report our early experience using a large diameter, 65 cm delivery sheath to facilitate delivery of the SAPIEN valve to the right ventricular outflow tract (RVOT)., Methods: Retrospective analysis of all patients from three large congenital heart centers undergoing tPVR with the Edwards SAPIEN valve delivered with the 65 cm Gore Dryseal Sheath., Results: Over a 12 month period, 30 patients (17 female) with median age 17.5 years (range 8-72) underwent attempted tPVR with the SAPIEN valve delivered using the 65 cm Dryseal sheath (20-26Fr). All procedures resulted in successful valve delivery to the target area. Twenty patients had a native RVOT. The most commonly used valve diameter was 29 mm (n = 15) with the majority of cases requiring a 26Fr Dryseal sheath (n = 20). One patient with severe RVOT stenosis underwent prestenting. Median procedure time was 100 min (59-225). No patient had increase in tricuspid valve regurgitation as a consequence of valve delivery. One patient required a synchronous cardioversion for intraprocedural VT and another required ECMO postprocedure due to severe pre-existing left ventricular dysfunction. On median follow-up of 5 months, all patients had mild or less pulmonary regurgitation. Median peak Doppler velocity across the pulmonary valve was 2.2 m/s (1.7-4). There were no clinically relevant complications relating to vascular access., Conclusions: Using 65 cm Dryseal sheaths facilitates delivery of SAPIEN valves in patients with dysfunctional RVOTs., (© 2019 Wiley Periodicals, Inc.)

Competing Interests: The authors declare no conflict of interest.

Mutations in PRKAG2 gene that regulates the γ2 subunit of the adenosine monophosphate (AMP) dependent protein kinase have been associated with the development of atrioventricular (AV) accessory pathways, cardiac hypertrophy, and conduction system abnormalities. These patients can potentially be misdiagnosed as hypertrophic cardiomyopathy (HOCM) and/or Wolf-Parkinson White (WPW) syndrome due to similar clinical phenotype. Early recognition of this disease entity is very important as ablation of suspected accessory pathways is not effective and the natural history of the disease is very different from HOCM and WPW syndrome.