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Bicuspid aortic valve is a more common congenital anomaly than widely appreciated as it may remain asymptomatic till later in life. The condition predisposes to the development of calcific aortic stenosis, usually in the 5th to 7th decades of life. They may occur alone or as part of a congenital syndrome, such as Tetralogy of Fallot. The latter is a combination of four congenital abnormalities, including a ventricular septal defect, pulmonary valve stenosis, a misplaced aorta and a right ventricular hypertrophy.
Bicuspid aortic valves have unequal cusp sizes usually as a result of two or the three normal cusps being fused together. This can lead to greater valvular dysfunction. Patients with bicuspid aortic valves are at high risk of aortic dilatation and dissection.
Bicuspid aortic valves are more likely to become calcified in older age than tricuspid aortic valves, and this is due to abnormal motion and turbulence caused by the unequal leaflet sizes.
With increasing age, patients can develop aortic stenosis or aortic regurgitation. When these become severe, symptoms associated with dyspnoea and reduced exercise tolerance may develop. This may be the first sign of a bicuspid aortic valve. Diagnosis of bicuspid aortic valves is confirmed using transthoracic echocardiogram.
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