Bicuspid aortic valves may be present in as many as 1-2% of the population. Because the bicuspid valve may be entirely silent during infancy, childhood, and adolescence, these incidence figures may be underestimated and are not generally included in the overall incidence of congenital heart disease. Recent studies have proved this condition to have a genetic component. This is also associated with coarctation of aorta, patent ductus arteriosus and Turners syndrome.
Bicuspid aortic valve is often observed with other left-sided obstructive lesions such as Coarctation of the aorta or interrupted aortic arch, suggesting a common developmental mechanism. With degeneration of aging valves, sclerosis and calcification can occur. The changes are similar to those in atherosclerotic arteries. The bicuspid valve may also be completely competent, producing no back flow. However, redundancy and prolapse of cusp tissue can lead to valve regurgitation.
Complications arise in as many as one third of patients over their lifetimes, thus necessitating constant follow up and evaluations. Patients with Bicuspid aortic valve are usually asymptomatic, but 30 % of them may develop complications. The symptoms that they may present are those of aortic stenosis, aortic insufficiency or both. Rarely, the congenital bicuspid aortic valve may be the cause for critical aortic stenosis in infancy, leading to cardiac failure.
No specific medical care is required for individuals with bicuspid aortic valve unless they have progressive clinical deterioration or infection. Serial follow-up evaluations are important for early recognition of potential complications (valve insufficiency, valve stenosis, progressive aortic root dilation) and the prevention of possible bacterial endocarditis. Because hypercholesterolemia and other coronary artery disease risk factors may accelerate the sclerosis and deterioration of a congenitally bicuspid aortic valve, a heart-healthy diet is recommended for all patients. Prophylactic antibiotic therapy can be institute in these patients, at times of minor surgical procedure, against infective endocarditis.