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Unraveling the molecular complexity of bicuspid aortopathy: Lessons from comparative proteomics.
Biochim Biophys Acta Mol Basis Dis
January 2025
Department of Animal Biology, Faculty of Science, University of Málaga, Málaga, Spain; Biomedical Research Institute of Málaga and Platform on Nanomedicine (IBIMA-Plataforma BIONAND), Málaga, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain.
Molecular markers and pathways involved in the etiology and pathophysiology of bicuspid aortopathy are poorly understood. The aim here is to delve into the molecular and cellular mechanisms of the disease and identify potential predictive molecular markers using a well-established isogenic hamster model (T-strain) of bicuspid aortic valve (BAV) and thoracic aortic dilatation (TAD). We carried out comparative quantitative proteomics combined with western blot and morpho-molecular analyses in the ascending aorta of tricuspid aortic valve (TAV) and BAV animals from the T-strain, and TAV animals from a control strain.
View Article and Find Full Text PDFAortopathy in repaired tetralogy of Fallot and David procedure.
Cardiovasc Diagn Ther
December 2024
Department of Cardiology, St. Luke's International Hospital, Tokyo, Japan.
Tetralogy of Fallot (TOF) is a condition that often leads to long-term enlargement of the aortic root in after surgery. The aortic dilation is believed to be caused by histological abnormalities of the aortic media and the hemodynamic characteristics of increased aortic flow, compared to pulmonary flow. Severe cyanosis, severe right ventricular outflow tract (RVOT) obstruction, older age at repair, a larger aortic size at the time of repair, and a history of an aortopulmonary shunt parameters related to long-standing volume overload of the aortic root were the reported risk factors.
View Article and Find Full Text PDFZ-score evaluation and inferences for management of aortopathy patients.
Cardiol Young
January 2025
Department of Pediatrics, Oregon Health and Science University, Portland, OR, USA.
Echocardiographic Z-score models play a crucial role in defining cardiac pathology in paediatric patients. There are multiple models that practitioners utilize in the United States without guiding principles to standardize their use. Discrepant interpretations can occur depending on the model chosen, even if standardized Z-score cutoffs are applied.
View Article and Find Full Text PDFMechanical circulatory support for high-risk surgical aortic valve and ascending aortic replacement in severe bicuspid aortic valve stenosis: a case series.
Eur Heart J Case Rep
December 2024
Department of Cardiology, Manchester University Hospital NHS Foundation Trust, Wythenshawe Hospital, Southmoor Road, Manchester M23 9LT, UK.
Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect (reported incidence of 0.5%-2%) and is commonly associated with proximal aortic dilation. Patients with severe aortic stenosis (AS) of BAV have been shown to have worse pre-operative left ventricular (LV) function as well as a higher incidence of post-operative heart failure hospitalization when compared with analogous patients with tri-leaflet aortic valve disease.
View Article and Find Full Text PDFHeterogeneous Cardiac-Derived and Neural Crest-Derived Aortic Smooth Muscle Cells Exhibit Similar Transcriptional Changes After TGFβ Signaling Disruption.
Arterioscler Thromb Vasc Biol
February 2025
Department of Surgery (Cardiac) (P.R., B.J., A.H., G.L., W.L., R.A., G.T.), Yale School of Medicine, New Haven, CT.
Background: Smooth muscle cells (SMCs) of cardiac and neural crest origin contribute to the developing proximal aorta and are linked to disease propensity in adults.
Methods: We analyzed single-cell transcriptomes of aortic SMCs from adult mice to determine basal states and changes after disrupting TGFβ (transforming growth factor-β) signaling necessary for aortic homeostasis.
Results: A minority of Myh11 lineage-marked SMCs differentially expressed genes suggestive of embryological origin.
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