Enlightening the Association between Bicuspid Aortic Valve and Aortopathy

Froso Sophocleous, Elena Giulia Milano, Giulia Pontecorboli, Pierpaolo Chivasso, Massimo Caputo, Cha Rajakaruna, Chiara Bucciarelli-Ducci, Costanza Emanueli, Giovanni Biglino

Research output: Contribution to journalArticle (Academic Journal)peer-review

17 Citations (Scopus)
299 Downloads (Pure)


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.
Original languageEnglish
Article number21
Number of pages26
JournalJournal of Cardiovascular Development and Disease
Issue number2
Early online date19 Apr 2018
Publication statusPublished - Jun 2018


  • bicuspid aortic valve
  • aortopathy
  • molecular pathways
  • hemodynamics
  • clinical management
  • microRNAs


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