Bicuspid Aorta Valve

[Rick]

I am almost 25 years old and have been experiencing arrhythmia for 2 years.  I went to the Dr. had an echo-cardiogram and was diagnosed with the Bicuspid valve.  I am nervous as I have just started a high stress sales job and am very active as I workout 5 days a week.  I enjoy my job but it is a lot of pressure.  Everything I see says that this will not affect you unitl mid 40's to 50.  But nothing I can find mentions any preventative measures or anything to help avoid problems- specifically for this problem.  I still experience arrhythmia and it seems to have gotten worse (i guess I am thinking about it more).  At what point do I consider surgery?  What can I do to avoid problems?  What is the best way to deal with something this major at 25 years old?  Is there really much education on this? I have not had the consultation w/ my Dr. yet and am sure that many questions will be answered.  But any information would be of great help.  I feel that I am too young to have to worry about my heart.  Thanks in advance!

[Cleveland Clinic, MD]

The aortic valve is the valve that separates the left ventricle from the aorta.  It is made of 3 leaflets or flaps that look like an peace symbol when the valve is closed.  A bicuspid aortic valve means that there are 2 leaflets instead of the usual 3.  A unicuspid valve means there is only one leaflet.   Bicuspid aortic valve is the most common congenital anomaly of the heart.  These valves are functional at birth and remain so throughout a normal life span.  There is a increased predisposition to progress to stenosis (narrowing) as an older adult (60-70's) and surgical repair may be required at that time.  Replacement is not generally required until the valve has an area of less than 1 cm2.  There is also some increase in the incidence of infective endocarditis (infection of the valve).

Q: Are there any physical restrictions for individuals with bicuspid aortic valve?
A: No.  

Q: Is there any reason to believe that exercise can cause or exacerbated the leakage already in the valve or the dilation of the ventricle?
A: No.  Exercise does not precipitate deteriotion of valves.  

Q: Are there any special diet restrictions?
A: No.

Q: Are there some people who have had bicuspid aortic valve who will never have to have a valve replacement or will everyone with this problem eventually have to get their valve replaced?
A: Only a small percentage of people with this condition will eventually need surgery.

Q: What type of replacement valve is best should surgery be required?
A: The choices are a bioprosthetic valve (from an animal), a mechanical valve, or a homograft (from a human cadaver).  Each has benefits and risks.  The benefit of the bioprosthetic and homograft are that anticoagulation with a blood thinner is not required.  This may be useful in women of child-bearing age who desire to become pregnant and older persons with a high risk of falling.  The disadvantage of these valves is that their life-span is only about 10 years.  The advantage of the mechanical valve is that the life-span is much longer (usually longer than the patients) but requires taking an anticoagulant.  

It is a highly individualized choice as to which valve and will depend upon the patient's preference, the practice of the hospital where the surgery takes place and the co-existing medical problems.  The final decision as to which valve to use will be made by the surgeon in the OR once the valve is exposed and clearly seen.

Q: Is repair of the valve an option, rather than replacement?
A: Aortic valve repair is not a mainstream surgery but is being done at some centers. Essentially what this surgery involves is a detachment of the valve from the aorta and reattachment to a graft.  The benefit of the operation is the preservation of the native aortic valve. This type of operation is not commonly done but may be a good option  depending on the particulars of the case.   In any case it would be wise to have the operation at a major medical center with experience with the operation. The following article has additional information about valve repair and can be obtained at your local medical library.

Pepper J.  Yacoub M. Valve conserving operation for aortic regurgitation.  Journal of Cardiac Surgery.  12(2 Suppl):151-6, 1997 Mar-Apr.


Q: How do I find out if I would be a candidate for the minimally invasive techniques that are being used now?
A: You would first need to be evaluated by a cardiologist who would make recommendations to you and the surgeon.  The final choice would be up to the surgeon doing the operation. If you would like to be seen here I would recommend Dr. Cosgrove or Dr. Lytle (http://www.ccf.org/heartcenter/staff/thoracic.htm).  Surgical appointments can be made through the surgery scheduling office at [phone].


More information about congenital valve abnormalities can be found in  the web sites below.

http://www.amhrt.org/Heart_and_Stroke_A_Z_Guide/conghd.html

http://155.37.5.42/eAtlas/CV/579b.htm

http://www.mamc.amedd.army.mil/WILLIAMS/CHEST/Cardiac/Congenital/Bicuspid/Bicuspid.htm

http://www.cachnet.org/messages2/3292.html

http://www.mch.com/pihc5-06.htm

Hope this helps.

[Rick]

My son recently had a chest x-ray that revealed a "prominent aortic arch".  We have not been able to see a pediatric cardiologist yet, but are very anxious to get any information we can.  What is an aortic arch?  What are treatments and long term ramifications.  My son is epileptic and also has a metabolic disorder called long chain fatty oxidation disorder (or LCAD).  Are there any special considerations that should be given to the aortic arch in light of his other health history?  

Thank you!

[Cleveland Clinic, MD]

The aortic arch is the large blood vessel leading away from the heart.  A number of things may cause it to be prominent on X-Ray including a normal variant.  There is no relationship between arch problems and LCAD that I know of.

[Lee]

When I was 59 years old I had an aortic valve replacement with a St. Jude's mechanical valve.  At the time the measurement of my bicuspid valve was .3 cm.  I know this sounds like I don't know what I am talking about, but believe me it was true.  Afterwards the doctor told me that they considered less than .7 cm critical.  I waited much too long for my surgery, but no one knew it was that serious until the last echocardiogram, which showed the definitive measurements.  Immediately after surgery I felt incredibly better.  I was also told that the procedure had been so successful largely because I had kept myself in good shape by proper diet and exercise.  I would say to you keep yourself in good shape and have an echocardiogram taken every year to keep an eye on the valve.  And don't worry, as the doctor said, only a small percentage ever have to have the surgery!!!

[Jon]

In regards to the forum's answer to the individual inquiring about bicuspid aortic valve, the forum gave information that I commonly see on this forum:

Q: Are there some people who have had bicuspid aortic valve who will never have to have a valve replacement or will everyone with this problem eventually have to get their valve replaced?
A: Only a small percentage of people with this condition will eventually need surgery.


I am just a lay person, but I tend to disagree and feel that most bicuspid valves become problematic at some point in the patient's life and that most persons afflicted with this anomoly will require eventually.

Comments??

[Cleveland Clinic, MD]

Thanks for your comments Lee.  0.3 cm is the tightest I've heard of.  Jon - the reason it seems like more is that it is the people with bicuspid valves who have problems that get diagnosed and subsequently treated.  The vast majority of people with bicuspid valves never even know they have a valvular abnormality!

[steve]

CRC,
If it is the minority of patients with bicuspid valves that require surgery, what is the predominate cause for the problems that necessitate the surgery, i.e. what causes the leaflet deformity?
Thanks

[Cleveland Clinic, MD]

Because the leaflets are not normal there is a higher propensity to develop stenosis.

[Fred Michelson]

Hi Rick,
I am 45 yrs old, have been very active my entire life (golf, basketball, baseball, running, etc), and was recently diagnosed with a bicuspid valve.  I have lived an entirely normal life, had no special diet to follow.  I had an echocardiogram and will need surgery in the near future.  I have an appointment with a cardiologist on the 12th of Jan.

I hope your condition does not digress like mine did.  I have too much regurgitation (blood leaking back into the heart).  

Just wanted to let you know that at 45 I can still run 2 miles faster than most 30 year olds!

Fred