Patient Information

Bicuspid Aortic Valve and Aortic Stenosis

The aortic valve is the valve leading from the heart to the body.  Normally it has three little flaps, or cusps, that open to let the blood go forward, but close to prevent it from going backward.  In up to 1-2% of all people, two of these cusps are fused together long before birth, resulting in a "bicuspid" aortic valve.

 stenosis.gif

A bicuspid aortic valve with little or no tightness (aortic stenosis) or leaking (aortic regurgitation) should not affect the heart's function in any significant way.  This is a common condition that usually occurs randomly, but can run in families as well. 

Over time, children may or may not develop more tightness or leaking, but this is quite variable, and often does not become a problem until late in adulthood if at all.  Generally there is no need to do any procedure on the valve unless the tightness or leaking becomes severe or, in some cases, the artery leading away from the valve (the ascending aorta) may become enlarged (dilated).  The usual recommendation is for periodic visits to check for any progression.

Should a child develop moderate or severe tightness (aortic stenosis) or leaking (aortic regurgitation), or severe dilation of the ascending aorta, he or she might show signs of shortness of breath, exercise intolerance, fatigue, chest pain, dizziness or fainting.  Because these symptoms are also common in otherwise healthy children, a careful evaluation by a cardiologist would be prudent for any of these symptoms in a child with significant aortic stenosis to be sure that they do not indicate cardiac difficulties.  Of particular concern is fainting or near-fainting with exercise.  This symptom warrants restriction from athletics until the patient can be evaluated by a cardiologist. 

Severe aortic stenosis or aortic regurgitation may require a medical or surgical procedure to improve the functioning of the valve.  Aortic stenosis can sometimes be treated without surgery using a specialized balloon catheter, whereas in other cases surgical repair or replacement of the valve is necessary.  Severe aortic regurgitation can often be treated with medications alone, but sometimes surgery may be required to repair or replace the valve in this case as well.  There are several different surgical procedures commonly used to replace the aortic valve, including one that uses the patients own pulmonary valve to replace the aortic valve (Ross Procedure). 

Children with moderate or severe aortic stenosis or regurgitation may need to be restricted from certain athletic activities, but those with mild aortic valve disease generally need not be restricted.  This decision is best made by a cardiologist who has evaluated the child recently.

You can get some additional information about bicuspid aortic valve at some of the links below.  The quality varies a bit, but I have tried to focus on fairly good sources:

American Heart Association
http://www.americanheart.org/presenter.jhtml?identifier=1659
A good site to see a brief description of the different types of heart disease and the treatments available

Society of Thoracic Surgeons
http://www.sts.org/doc/3620
Information about bicuspid aortic valve and surgical treatment of aortic valve disease.

University of New Mexico
http://hospitals.unm.edu/childrens/CHHC/patients/heart-disease/stenosis.shtml
A brief discussion, but a nice figure of a bicuspid aortic valve.

eMedicine
http://www.emedicine.com/PED/topic2486.htm
A more technical but thorough discussion of bicuspid aortic valve and aortic valve disease.

Updated 2/23/03