Bicuspid Aortic Valve

A bicuspid aortic valve (BAV) is a defect of the aortic valve that results in the formation of two leaflets or cusps instead of the normal three. It is the most common congenital cardiac anomaly. About 1–2% of the population has bicuspid aortic valves (BAV). Bicuspid aortic valve is an inheritable condition. The incidence of bicuspid aortic valve can be as high as 10% in families affected with the valve problem. 

This disease can lead to other heart complications such as aortic stenosis, aortic insufficiency,  and an enlarged aorta.

Aorta Aneurysms and Aortic Insufficiency 

An important associated problem is that the aorta of patients with bicuspid aortic valve is not normal in strength or size.  Aortas of patients with a bicuspid aortic valve do not have the same histological (cellular) characteristics of a normal aorta. In particular, the tensile strength of the aortal wall is reduced, which puts these patients at higher risk for dilation of the ascending aorta (aortic aneurysm) which may result in a leaky aortic valve (aortic insufficiency) or a tear in the aorta (aortic dissection) and  formation of the ascending aorta. 

The size of the proximal aorta should be evaluated carefully during the work-up. The initial diameter of the aorta should be noted. An 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 Stenosis 

BAV may become calcified later in life, which may lead to varying degrees of severity of aortic stenosis. If the leaflets do not close correctly, aortic regurgitation can occur. If these become severe enough, they may require heart surgery. The heart is put under more stress in order to either pump more blood through a stenotic valve or attempt to circulate regurgitation blood through a leaking valve. Due to poor heart performance, people with BAV may become tired more easily than those with normal valvular function, and they  may have difficulty maintaining stamina for cardio-intensive activities.

Most patients with bicuspid aortic valve whose valve becomes dysfunctional will need careful follow-up and potentially valve replacement at some point in life. Regular ECG and MRI may be performed. For diagnosed patients, genetic testing is done to allow for future offspring with the disease to be monitored and treated early in life.

Congenital Defects  

Other congenital defects are associated with bicuspid aortic valve at various frequencies, including coarctation of the aorta, mitral valve leakage, kidney or liver cysts,  near-sighted vision,  musculoskeletal degenerative changes (scoliosis, degenerative spinal discs, flexible joints, flat feet, etc.), and aneurysm or dissection of smaller arteries (berry aneurysms)
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Dr. Russo in the News