Ultimately there is a risk of rupture in the aortic valve due to bicuspid aortopathy which is a result of progressive aortic dilation from the stress of having only two values where three are normal. Aortic lesions
One of the most notable associations with BAV is the tendency for these patients to present with ascending aortic aneurysmal lesions. The extracellular matrix of the aorta in patients with BAV shows marked deviations from that of the normal tricuspid aortic valve. It is currently believed that an increase in the ratio of MMP2 (Matrix Metalloproteinases 2) to TIMP1 (tissue inhibitors of metalloproteinase) may be responsible for the abnormal degradation of the valve matrix and therefore lead to aortic dissection and aneurysm. However, other studies have also shown MMP9 involvement with no differences in TIMP expression. The size of the proximal aorta should be evaluated carefully during the workup. The initial diameter of the aorta should be noted and annual evaluation with CT scan, or MRI to avoid ionizing radiation, should be recommended to the patient; the examination should be conducted more frequently if a change in aortic diameter is seen. From this monitoring, the type of surgery that should be offered to the patient can be determined based on the change in size of the aorta. Aortic narrowing
A bicuspid aortic valve may cause the hearts aortic valve to narrow (aortic stenosis). This narrowing prevents the valve from opening fully, which reduces or blocks blood flow from the heart to the body.
Its heritability (
  
    
      
        
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  ) is as high as 89%. Both familial clustering and isolated valve defects have been documented. Recent studies suggest that BAV is an autosomal dominant condition with incomplete penetrance. Other congenital heart defects are associated with bicuspid aortic valve at various frequencies, including coarctation of the aorta.Bicuspid aortic valve abnormality is also the most observed cardiac defect in Turner syndrome. == References ==