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Enlightening the Association between Bicuspid Aortic Valve and Aortopathy

1
Bristol Heart Institute, Bristol Medical School, University of Bristol, Bristol BS2 89HW, UK
2
Department of Medicine, Division of Cardiology, University of Verona, 37100 Verona, Italy
3
Structural Interventional Cardiology Division, Department of Experimental and Clinical Medicine, University of Florence, 50100 Florence, Italy
4
Cardiac Surgery, University Hospitals Bristol, NHS Foundation Trust, Bristol BS2 8HW, UK
5
National Heart and Lung Institute, Imperial College London, London SW7 2AZ, UK
6
Cardiorespiratory Unit, Great Ormond Street Hospital for Children, NHS Foundation Trust, London WC1N 3JH, UK
*
Author to whom correspondence should be addressed.
J. Cardiovasc. Dev. Dis. 2018, 5(2), 21; https://doi.org/10.3390/jcdd5020021
Received: 22 March 2018 / Revised: 16 April 2018 / Accepted: 16 April 2018 / Published: 19 April 2018
(This article belongs to the Special Issue Genetics of Congenital Heart Disease)
Bicuspid aortic valve (BAV) patients have an increased incidence of developing aortic dilation. Despite its importance, the pathogenesis of aortopathy in BAV is still largely undetermined. Nowadays, intense focus falls both on BAV morphology and progression of valvular dysfunction and on the development of aortic dilation. However, less is known about the relationship between aortic valve morphology and aortic dilation. A better understanding of the molecular pathways involved in the homeostasis of the aortic wall, including the extracellular matrix, the plasticity of the vascular smooth cells, TGFβ signaling, and epigenetic dysregulation, is key to enlighten the mechanisms underpinning BAV-aortopathy development and progression. To date, there are two main theories on this subject, i.e., the genetic and the hemodynamic theory, with an ongoing debate over the pathogenesis of BAV-aortopathy. Furthermore, the lack of early detection biomarkers leads to challenges in the management of patients affected by BAV-aortopathy. Here, we critically review the current knowledge on the driving mechanisms of BAV-aortopathy together with the current clinical management and lack of available biomarkers allowing for early detection and better treatment optimization. View Full-Text
Keywords: bicuspid aortic valve; aortopathy; molecular pathways; hemodynamics; clinical management; microRNAs bicuspid aortic valve; aortopathy; molecular pathways; hemodynamics; clinical management; microRNAs
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Sophocleous, F.; Milano, E.G.; Pontecorboli, G.; Chivasso, P.; Caputo, M.; Rajakaruna, C.; Bucciarelli-Ducci, C.; Emanueli, C.; Biglino, G. Enlightening the Association between Bicuspid Aortic Valve and Aortopathy. J. Cardiovasc. Dev. Dis. 2018, 5, 21.

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