Research Topic

The Pathogenetic Mechanisms at the Basis of Aortopathy Associated with Bicuspid Aortic Valve: Insights from “Omics”, Models of Disease and Emergent Technologies

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Thoracic ascending aorta aneurysm (TAAA) is a localized or diffuse dilatation of aorta with a diameter at least 1.5-folds greater than the expected normal size, resulting from the maladaptive remodeling of the extracellular matrix (ECM) in the aortic wall, with an imbalance between protein synthesis and ...

Thoracic ascending aorta aneurysm (TAAA) is a localized or diffuse dilatation of aorta with a diameter at least 1.5-folds greater than the expected normal size, resulting from the maladaptive remodeling of the extracellular matrix (ECM) in the aortic wall, with an imbalance between protein synthesis and proteolysis in the medial layer, severe elastin fragmentation, cystic medial necrosis and medial fibrosis. Dilatation of aortic root and ascending aorta is frequently encountered in patients with a congenital heart disease. Bicuspid aortic valve (BAV) is the most common adult congenital heart defect, concurrent with early aortic dilatation in 80% of patients. The involvement of distinct factors in the onset of TAAA in BAV patients vs. TAV patients is well recognized.

Nonetheless, the pathogenesis of aortopathy associated with BAV is not yet well understood. A genetic predisposition can be associated in different degrees and forms to abnormal regional blood flow patterns in the ascending aorta created by cusp fusion and abnormal BAV morphology, leading to localized areas of aortopathy.

The coexistence of these mechanisms can contribute to the heterogeneous patterns of aortic dilation often seen in BAV patients. Aggressive surgical management strategies with respect to the timing and extent of aortic resection are often applied to BAV patients to prevent the occurrence of fatal TAAA. A more in-depth knowledge of the mechanisms at the basis of aortopathy associated with BAV could be helpful in a redefinition of the guidelines for surgical intervention in BAV patients.

On this basis, we would like to propose to the Editor of Frontiers in Physiology, section Vascular Physiology, a Research Topic including the multiple pathogenetic mechanisms of aortopathy, including the hemodynamics and biomechanics aspects, the development of animal models of disease, the identification of key molecular pathways involved in alterations of the phenotype of endothelial and smooth muscle cells and in mechanosensing, and the identification of potential early biomarkers of aortopathy, including microRNAs or other circulating molecules. To this aim, we selected potential contributors to this Research Topic on the basis of their well-known expertise and background in the field.


Keywords: bicuspid aortic valve, aortopathy, hemodynamics, biomarkers, remodeling


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