Patient
Information
Subaortic
stenosis
What is it?
Subaortic stenosis is a tightness located below the valve that lets
the blood out (the aortic valve). There are two problems that result from
subaortic stenosis: The heart has to work harder to pump the blood
out to the body,
and the high-pressure jet of blood can also damage the aortic valve
as it passed through it. Children with subaortic stenosis can have
problems with shortness of breath, poor growth, fainting and leaking
at the aortic valve (aortic regurgitation), and later in life they
can have problems with abnormal heart rhythms. Untreated subaortic
stenosis, when severe, can be a risk factor for sudden unexpected
death. Subaortic stenosis is rarely present at birth, and generally
gets gradually worse throughout life. Even after surgical
correction, it is not uncommon for subaortic stenosis to recur and
continue to progress.
Some patients with
hypertrophic cardiomyopathy, in whom the main problem is an abnormal
thickening of the heart muscle, can also have subaortic stenosis.
This is a somewhat different problem from subaortic stenosis with a
normal heart muscle, so the rest of this article refers to patients
who do not have hypertrophic cardiomyopathy.
What causes it?
No one knows what causes subaortic stenosis. It often occurs in
children who have other types of congenital heart disease (heart
problems one is born with), but is can also occur with no other
heart problems. About 1 in 1,200-10,000 people has subaortic
stenosis, and boys are 2-3 times more likely to be affected. No
genetic inheritance is known for subaortic stenosis, and few
familial incidences have been reported, but it is slightly more
likely to occur in a family if a prior child has had heart disease.
There is nothing a mother can do during pregnancy to make subaortic
stenosis more or less likely to occur.
How is it diagnosed?
Most children with subaortic stenosis are identified because of a
heart murmur. The diagnosis is confirmed with an echocardiogram
(ultrasound test of the heart).
How is it treated?
Not all children with subaortic stenosis need surgery. There are
two reasons that subaortic stenosis might require repair: If the
tightness is severe enough that it causes too much strain on the
heart, or if the stenosis causes damage to the aortic valve. Even
mild stenosis can damage the aortic valve, so sometimes even mild
subaortic stenosis may require repair. The only effective treatment
for subaortic stenosis at this time is an open heart operation,
although it may be possible in the future to use non-surgical
catheter-based technology to reduce or eliminate subaortic stenosis.
For more information about
subaortic stenosis, check the following Internet links. These links
should be of pretty good quality, but as always with the Internet,
it is a case of “browser beware.”
eMedicine -
http://www.emedicine.com/PED/topic2485.htm
Southern Illinois University
-
http://www.siumed.edu/peds/teaching/Cardiology/subaortic%20stenosis.htm
Albany Medical Center -
http://heart.amc.edu/subas.htm
PediHeart -
http://www.pediheart.org/
Includes a "Parent's
Place" with information resources.
March of Dimes -
http://www.modimes.org/HealthLibrary2/FactSheets/Congenital_heart_defects.htm
The Children's Heart Society
-
http://www.childrensheart.org/
Specializes in support for
families and information.
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