Bi Cusp Aortic Valve Stenosis

[Marianne M]

I am 39 years old and recently diagnosed with Bicuspid Aortic Valve Stenosis – In a year and a half’s time my AVA measurement went from 1.1 to .9 - The cardiologist states I have about 5 years before I will have to undergo the surgery to have the Aortic Valve replaced with a mechanical valve.
I am curious as to the benefit of waiting for 5 years - if my heart is comparable to someone in their 70's and there is no doubt that I need the surgery, why not perform it now while I am still young and in decent shape?
The articles that I’ve read state that 4% of people with this condition die instant death as the first symptom, these odds don’t sit well as I have not been advised of any medications that may help.
As for being symptomatic I occasionally get a cramping like sensation under the right side of my chest, I also have bone deterioration in my jaw (TMJ) and now have arthritis in those muscles is this possibly related to the valve issues?
When I was in college I was diagnosed with chronic fatigue syndrome and alopecia - do these have any relation to the Aortic Valve Stenosis?
I am often tired but have three children the first of which are 9 year old twins, when they were about 3 my general doctor detected for the first time a very loud murmur it was stated at that time that I had Mitral Valve Prolapse, which apparently mimics the AVS sound –could carrying the twins have progressed the condition.  The last echo also shows the MVP but it is not major, I have taken antibiotics prior to dirty procedures since then.
Please advise as to what your opinion is on the matter.
Thank you,
Marianne

[CCF-M.D.-RCJ]

Marianne,

Thanks for the post.

Q:"why not perform it now while I am still young and in decent shape?"

Presumably, you should still be in "decent" shape at age 44!  Two reasons to put off surgery: (1) the mechanical valves, if that is what you will need, can deteriorate with time, and (2), if (God forbid) something untoward were to happen to you during surgery, you would have missed out on 5 years time without disability.

Q:"The articles that I’ve read state that 4% of people with this condition die instant death as the first symptom"

This number is too high.

Q:"... arthritis in those muscles is this possibly related to the valve issues?"

There is no known association.

Q:"When I was in college I was diagnosed with chronic fatigue syndrome and alopecia - do these have any relation to the Aortic Valve Stenosis?"

There is no known association.


Q:"Please advise as to what your opinion is on the matter."

I would seek careful consultation with a well-respected cardiologist at a large medical center.  Valve repair may be possible, and is the number one reason to go to a large center that has a surgeon skilled at this operation.

Hope that helps.

[Avel]

Hi Marianne,
My husband had a highly calcified stenotic bicuspid aortic valve. His valve failed rather abruptly, and he went into full congestive heart failure prior to having his valve replaced. He later was found to have an ascending aortic aneurysm. Only then did we learn that there can be more to bicuspid aortic valve disease than just the valve. Fortunately for him, he had a very successful surgery to replace the aneurysm with a Dacron graft. Not everyone is so fortunate. It is important to know as much as possible about this.

I want to share some information with you. Here is a paper published in the AHA journal Circulation that gives a good overview of this condition. I encourage everyone who is bicuspid to discuss this with their doctors. http://circ.ahajournals.org/cgi/content/full/106/8/900?eaf

And here are some links with additional information
http://www.cedars-sinai.edu/aorta
http://www.cedars-sinai.edu/bicuspid

Best Wishes,
Avel

[ontherecord]

I must say the responses that I've read on this board are quite remarkable, and I have no doubt that they are very useful not only to the poster of the message, but to all of us who are interested in this field of medicine (or afflicted with similar situations).  In no other forum on this board (and I read them all and even contribute where I can) are the answers to the questions as *on target* as they are on this particular forum.  

Many of the other doctors (or nurses, as the case may be on certain forums) give "general" advice and often do not speak directly to the question presented without some qualifier (i.e., without having seen you, I am unable to ...., or "this is for general educational purposes only..")  

Here, both these doctors push the envelope and seem intent to actually providing useful, reliable, comprehensive, and pertinent information to the patient.

THIS forum is exactly what the Cleveland Clinic sought to accomplish (as evinced through the Mission Statement).  Timely responses from cardiologists at one of the most reputable heart clinics in the world.  Hats off to job well done.

[Marianne M]

Thank you for taking the time to give me a reply so rapidly.  I will follow up on the links that you have provided, at this point I feel like a sponge and I am trying to absorb as much information as I can about my condition in order to have a full arsonal of questions for my current cardiologist.  
I live in the Jacksonville, Fl area and have Shands, St. Vincent's, Flagler, and the Jacksonville Heart Center for resources.  If anybody is familiar with any of these centers and knows of a specialist in this area like always any information is welcome.  
Thanks,
Marianne