Next Article in Journal
Roles of PDE1 in Pathological Cardiac Remodeling and Dysfunction
Next Article in Special Issue
The Role of scaRNAs in Adjusting Alternative mRNA Splicing in Heart Development
Previous Article in Journal
cGMP Signaling and Vascular Smooth Muscle Cell Plasticity
Open AccessReview

Enlightening the Association between Bicuspid Aortic Valve and Aortopathy

Bristol Heart Institute, Bristol Medical School, University of Bristol, Bristol BS2 89HW, UK
Department of Medicine, Division of Cardiology, University of Verona, 37100 Verona, Italy
Structural Interventional Cardiology Division, Department of Experimental and Clinical Medicine, University of Florence, 50100 Florence, Italy
Cardiac Surgery, University Hospitals Bristol, NHS Foundation Trust, Bristol BS2 8HW, UK
National Heart and Lung Institute, Imperial College London, London SW7 2AZ, UK
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;
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
Show Figures

Figure 1

MDPI and ACS Style

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.

Show more citation formats Show less citations formats
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

Back to TopTop