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.
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 |
