Bicuspid aortic valve aortopathy: genetics, pathophysiology and medical therapy.

The association between ascending aortic aneurysm (AA) and bicuspid aortic valve (BAV) has been well established. Different genetic, haemodynamic and cardiovascular risk factors have been implicated in the development and progression of AA. However, to date, definite conclusions cannot be drawn regarding the exact molecular, cellular and haemodynamic mechanisms causing BAV-associated aortopathy. For this study, we performed a thorough electronic systematic review of the literature using MEDLINE (1960-2012) and EMBASE databases. MeSH terms included: 'bicuspid aortic valve and ascending aorta', 'bicommissural aortic valve and aneurysm', 'bicuspid aortopathy', 'bicuspid aortic valve pathophysiology', 'bicuspid aortic valve and genetics' and 'bicuspid aortic valve and treatment'. We aim in this review to discuss the mechanisms, pathophysiology, genetics and modern drug therapy in the context of BAV-associated aortopathy.