Figure 1.

Nosology of the congenital BAV condition. Left: anatomically and prognostically complex presentations of the BAV valvulo-aortopathy are those associated with syndromes, left-sided obstructions, significant aortic coarctation, early/accelerated valve dysfunction (stenosis or regurgitation) and/or early aortopathy. Middle: the anatomically and prognostically typical valvulo-aortopathy is usually diagnosed in young and middle-aged adults although it may be diagnosed in children as well and comprises various degrees of progressive valvular dysfunction (mostly high incidence of aortic stenosis), with a high cumulative incidence of aortopathy over the long-term, manifested as thoracic aortic dilatation, without other major associated conditions. Right: the undiagnosed form is diagnosed retrospectively (without any BAV-related complications, some are diagnosed post-mortem), or may present for the first time with a life-threatening complication such as aortic dissection or infective endocarditis. Modified from Michelena et al. (1-4) with permission. BAV, bicuspid aortic valve.