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Background: Congenital aortic and truncal valve disease is challenging in infants and children given the lack of available prostheses in very small sizes and the limited durability of homograft aortic valve replacement., Methods: A comprehensive literature search was performed using the PubMed database. Studies were included either if the report included patients less than 1 year of age or if the technique was tailored to accommodate for somatic growth., Results: Techniques for aortic and truncal valve repair addressing each aspect of the aortic valve complex - the aorta, aortic annulus, commissures, and cusps - were reviewed. The incidence of reoperation following aortic or truncal valve repair is significant at 10-years ranging from 30% to 70% depending on the underlying diagnosis and the repair technique utilized. A significant challenge in interpreting the published literature relates to the lack of anatomic data available in the publications limiting the ability to make direct comparisons between operative techniques and also limits the ability to draw conclusions regarding these techniques as applied to varied etiologies., Conclusions: A comprehensive understanding of the aortic valve complex is necessary to achieve adequate results in pediatric aortic valve repair given the high variability in these valves., (Copyright © 2024. Published by Elsevier Inc.)

Background: von Willebrand factor (VWF) is a multimeric glycoprotein critically involved in hemostasis, thrombosis, and inflammation. VWF function is regulated by its antigen levels, multimeric structures, and the state of enzymatic cleavage. Population studies in the past have focused almost exclusively on VWF antigen levels in cross-sectional study designs., Objective: To identify subjects in the Atherosclerosis Risk in Community study who had persistently low and high VWF antigen over 10 years and to quantify longitudinal changes in the biological activities and cleavage of VWF in these subjects., Methods: We measured VWF antigen, propeptide, adhesive activities, and cleavage by ADAMTS-13 quantified using a mass spectrometry method that detected the cleaved VWF peptide EQAPNLVY, as well as coagulation factor VIII activity., Results: We determined the mean subject-specific increase in VWF to be 22.0 International Units (IU)/dL over 10 years, with 95% between -0.3 and 59.7 IU/dL. This aging-related increase was also detected in VWF propeptide levels, ristocetin cofactor activity, and VWF binding to collagen. We identified 4.1% and 25.0% of subjects as having persistently low (<50 IU/dL) and high (>200 IU/dL) VWF antigen, respectively. Subjects with persistently low VWF had enhanced ristocetin cofactor activity, whereas those with persistently high VWF had elevated levels of ADAMTS-13, resulting in a comparable rate of VWF cleavage between the 2 groups., Conclusions: These results provide new information about the effects of aging on VWF antigens and adhesive activity and identify a functional coordination between VWF and the rate of its cleavage by ADAMTS-13., Competing Interests: Declaration of competing interests There are no competing interests to disclose., (Copyright © 2023 International Society on Thrombosis and Haemostasis. Published by Elsevier Inc. All rights reserved.)

It has been long appreciated that anesthetic drugs induce stereotyped changes in electroencephalogram (EEG), but the relationships between the EEG and underlying brain function remain poorly understood. Functional imaging methods including positron emission tomography (PET) and functional magnetic resonance imaging (fMRI), have become important tools for studying how anesthetic drugs act in the human brain to induce the state of general anesthesia. To date, no investigation has combined functional MRI with EEG to study general anesthesia. We report here a paradigm for conducting combined fMRI and EEG studies of human subjects under general anesthesia. We discuss the several technical and safety problems that must be solved to undertake this type of multimodal functional imaging and show combined recordings from a human subject. Combined fMRI and EEG exploits simultaneously the high spatial resolution of fMRI and the high temporal resolution of EEG. In addition, combined fMRI and EEG offers a direct way to relate established EEG patterns induced by general anesthesia to changes in neural activity in specific brain regions as measured by changes in fMRI blood oxygen level dependent (BOLD) signals.