Bicuspid aortic valve disease: recent insights in pathophysiology and treatment.

Bicuspid aortic valve is a common congenital cardiac malformation with a broad spectrum of clinical outcomes. Bicuspid aortic valve may go undetected throughout an individual's lifetime or, alternatively, they may have devastating clinical consequences, resulting in death. Both clinicians and medical scientists have taken a renewed interest in the development, pathophysiology and treatment options for this subtle but often substantial clinical entity. Evidence is mounting to suggest that an underlying disease of the aorta is inherited with bicuspid aortic valve, although considerable controversy surrounds this theory. Novel molecular mechanisms underlying the valve and vascular pathologies, as well as new surgical therapies for these patients have been proposed in the past 10 years.