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Bicuspid aortic valve disease: an old disease with a new name?

Bicuspid aortic valve (BAV) is the most common congenital cardiac disease, occurring in 0.7-2.0% of the population.

Valvular Heart Diseases

BAV has important cardiological effects, which have been recognized over the last year. It occurs mostly in males, and is associated with coarctation of the aorta (50-80%), Turner’s syndrome (10-12%), interrupted arch (38%) and VSD (20%). Large numbers of genes could be responsible for BAV. Genes and the pathways are not well defined, although we know about three loci: 18q, 5q and 13q. Epidemiology and genetics was presented by Prof. Robert J.S. HALL (GB).

Anatomy and histopathologic basis of disease progression was presented by Prof. Jens J. KADEN (DE). Most often, a fusion between right and left coronary leaflets is seen. The observation of fusion raphe on echo should alert physicians to BAV. Aortic dilatation is observed independent of stenosis or regurgitation severity. Aortic dilatation is common even with hemodynamically normal BAV. Calcification of the aortic valve is mostly observed in BAV. Any relation between calcification of the BAV and dilatation of the aorta has not been observed. Calcification is a frequent cause of aortic stenosis or aortic regurgitation.

Natural history and clinical outcomes was presented by Prof. Bernard IUNG (FR). Clinically important points include: deterioration of aortic valve function, infective endocarditis and aneurysm of the ascending aorta, but risk assessment is difficult. Age >50 years, heart rate and valve degeneration are strong predictors of events. Aortic stenosis and less frequently, aortic regurgitation are indications for aortic valve replacement which occurs earlier in patients with BAV than in tricuspid aortic valve. One of the reasons for this is calcification of the valve. Therapeutic interventions are unclear. We don’t have validated strategies for slowing the progression. Randomized trials for validated strategies for slowing the progression of aortic valve calcification are needed. Aortic dilatation does not depend on valve function; and increases with aortic diameter. We should calculate aortic size index and the progression rate. The risk of infective endocarditis is low, but 25% of infective endocarditis occurs in BAV.

Prof. A. Peter KAPPETEIN (NL) presented the evaluation and clinical surgical management. The indications for surgery are the complications of BAV: 1) aortic stenosis (15-75% depending on age), 2) aortic regurgitation (5-15%), 3) infective endocarditis (10-30%) and complications of abnormal aortic wall, such as aortic root dilatation (40-80%), aortic dissection (5-15%) and coarctation. Careful monitoring not only of the valve but also of the aorta is necessary. Progressive aortic aneurysm dilatation should be operated on if the diameter is > 5cm or >4.5 cm during aortic valve replacement or if the rate of progression is >5 mm/year. Many operative techniques were presented. An important conclusion is that outcomes of aortic valve repair are not superior to those of aortic valve replacement.


The consequences of BAV can be serious. Prospective studies are needed to identify BAV patients at high risk of complications. We should also screen the relatives of patients with BAV.




Bicuspid aortic valve disease: an old disease with a new name?

Notes to editor

This congress report accompanies a presentation given at the ESC Congress 2008. Written by the author himself/herself, this report does not necessarily reflect the opinion of the European Society of Cardiology.

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.