Bicuspid Aortic Valve

[Nancy=Ann]

Yesterday my 16 yr old son had onset of severe chest pain, profuse sweating and tunnel vision while playing volleyball at school. (The teacher of the class said they were mostly standing around)  He is in football and has 3 hour practices daily and weight trains every other day.  They brought him to the school office where I work.  He was gray complexioned, blue lipped, and sheeting sweat from his whole body.  The paramedics took his pulse aprox 10 minutes from onset, 168bpm and he still had chest pain.  After O2, the pain left, he felt better and was hungry.  He was taken to the ER, where they did an EKG and an Echocardiogram.  The ER dr said he had a bicuspid valve and a murmer, and said to make an appt with a cardiologist right away. No football practice or strenouous exercise.  When I made the appt, I was told he COULD practice, and that they would see him two weeks later.  I am not comfortable with this, so they made an appt for Monday.  I have been reading all that I can find on the net and it seems that there is usually no restriction on exercise, but what could have caused these symptoms?  He looked BAD, and he rated the pain at an 8 or 9.  He had nothing to eat or drink that morning, but that is probably common for him as he would rather sleep the extra few minutes and usually eats at 10:00 at school.  He'd had a game the night before, drank gatorade before and after the game, and water during. He played all but a few minutes of the game.  Can you help a semi-panicing mom?

[CCF-M.D.-RCJ]

Nancy,

Sorry to hear of your ordeal.

If I had experienced what you did with my child I can tell you that I would not let him participate in strenuous sports until I knew the status of that valve.  Most people with a bicuspid aortic valve do just fine with it, but some have problems with the valve leaking or not opening properly.  Both of these problems can be easily fixed with surgery.

Also, other heart conditions besides aortic valve disease can cause problems while exercising.  The cardiologist you will be seeing will look for these as well.

The point really is: "why risk it while you are waiting".  You will have an answer soon, so wait.

Good luck, and keep us updated.

[Nancy=Ann]

Thanks Hank.  I called the ER after reading your post and looking up the definition of aortic dissection on the net.  Many of the symptoms of it my son had.  The ER told me that the x-ray did not show any signs, then I told them he hadn't had an x-ray. They put me on hold and then came back and said the echo would have shown it.  I have no confidence in the hospital I took him to.  It was the closest one, but I am definately going to have many questions for the cardiologist.  My son is going to watch a lot of TV this weekend, as there won't be much activity happening for him until questions are answered.

[Avel]

Dear Nancy Ann,
I am so concerned about your son. Because my husband had a bicuspid valve (and an aortic aneurysm), I have read a great deal of the medical literature regarding bicuspid aortic valves and the other aspects of this condition, the most dangerous being the potential for aortic dissection or aneurysm rupture. As I write this I am looking at a paper from Clinical Cardiology published in 1998 by Dr. John M. Burks. One of the patients he describes is an 18 year old boy with a bicuspid valve who was first seen in the ER - he had an enlarged ascending aorta and a dissection (tear) beginning just above the aortic valve, and was taken into surgery and successfully treated. As Hank has mentioned, I also do not want to frighten you, but timely, accurate assessment of your son is very important. If this were my son, I would request a CT with contrast be performed on him as soon as possible. Most ER's should be able to do this, but if you have a major medical center near you, they are most likely to recognize and be able to treat aortic disease. There is medical as well as surgical treatment for this. Assessment and medical treatment of your son now may avoid a surgical emergency. The important thing is to know, and leave nothing to chance. There are too many tragic stories of missed diagnoses of aortic dissections. And as the recent news has made everyone aware, even when the diagnosis is made, emergency surgical treatment of aortic dissection may not be successful.
I also want to point you to an information resource on the web regarding this. Complete information regarding bicuspid aortic disease, which includes the bicuspid valve, possible aneurysm and dissection of the aorta, and other facets of this condition is not easy to find. From the main page of the following web site you will find links describing aortic disease and bicuspid aortic disease. There is also an email interface for you or anyone else who may read this to request additional information. Here is the link http://www.cedars-sinai.edu/aorta
Please feel free to email me at ***@****  Very best wishes to you and your son.

[farmerMike]

I also have a bicuspid aortic valve.  I am 25 years old and have done all research possible.  Your son needs to have an echocardiogram and a doppler evaluated by a good cardiologist.  I played hockey, full contact and had the same feelings one day.  The doctor said it was low blood sugar.

If your son has a BAV with no mild or no stenosis he will be fine.  All he will need is anti-biotics prior to dental work.  I highly doubt that it is an aortic aneurism or dissection. If he was feeling better after a few hours and continued to, he probably wouldn't be in a fatal situation.  These primarilly occur with people in their 40's to 70's.

The most important thing for him is not to worry about it.  I spent a year thinking I was in for it because of a bicuspid aortic valve.  Most BAV cases pose no problem through a normal lifespan.

[valger]

Hi everyone, I was just wondering, is the condition you are talking about the same thing that John Ritter just died from?  They said they think it was the aortic valve that tore.  I was wondering what the risk factors are for this, I heard it was high blood pressure, but I don't think someone so young would have that.  I get PVC's all the time, and I have had echo's, and most recently, a thallium stress test, but I don't know if those things would pick up something like that.  I worry sometimes that I have something structurally wrong with my heart since I have such frequent PVC's.  Anyway, good luck, and thanks, Val

[Avel]

As the posts from farmerMIKE and hankstar indicate, the underlying cause of chest pain and other symptoms can be very difficult to determine. It is so important that nothing be left to chance because there is no room for error when it comes to the aorta. The possible involvement of the aorta when there is chest pain in anyone with conditions such as bicuspid aortic valve disease, Marfan syndrome, Ehlers-Danlos, or any family history of aortic aneurysm or dissection must be considered. There is so much variation in how these conditions affect people that it is important that nothing be assumed. What may be perceived to happen to many or most people does not matter much to the one experiencing the problem - be it dissection or the slow silent growth of an aneurysm. Perhaps it would be of interest to list the Aneurysm Support web site address here. The variety of experiences shared there include those who survived dissection as well those who have not. Some people who experienced dissection were found to have evidence of a prior dissection that they can associate with chest pain at an earlier time. However, it went undetected and untreated at the time it happened. If it had been found, they could have been treated medically and perhaps have avoided a second life-threatening event. Here is the URL to the support group http://www.westga.edu/~wmaples/aneurysm.html
For those with bicuspid aortic disease, an additional helpful resource fully available on the net is a paper published in the AHA journal Circulation. Here is the URL http://circ.ahajournals.org/cgi/content/full/106/8/900?eaf
For those with bicuspid aortic valve disease, it is important to be well informed, and then, as farmerMIKE has said, continue living a normal, productive life while being followed on an ongoing basis. Along with periodic monitoring of the aortic valve, the aorta should also be monitored for enlargement. Ideally, with ongoing follow up, medical treatment, and proper timing of elective surgery, emergency situations may  be avoided.

[Nancy=Ann]

Thank you so much for your comments, everyone.  The EMT's tested his blood sugar at school and it was 107 with no breakfast.  As soon as the pain stopped, he was smiling and really back to his normal self, the first question from his mouth was 'when can I get something to eat, it's nutrition break?' so it doesn't seem like this was an anxiety attack.  It was severe pain.  I hope and pray it can be explained away as something non-important, but the question of dissection will be thorouly discussed with the pediatric cardiologist tomorrow.  I just want to make myself as informed as I can before speaking to him.  The paramedics made me feel like I was being overreactive, and didn't even transport him, then the ER doctor told me I was 'lucky he didn't drop, he could never do any vigorous exercise again, etc.' then two minutes later after talking to the cardiologist, told me he could go to football practice.  I just want every possible test to be done to make sure this was not a dissection or any other abnormality with his valves.  He had the echo done, what other tests would be valuable to look for this?
Thank you all again so much.

[Avel]

Please go to this URL and under "Diagnosis" read about the different tests that can be done. http://www.cedars-sinai.edu/3866.html The two different types of echocardiograms (TTE and TEE), CT with contrast, and MRI with contrast are described here. Your son has already had an echo, which detected the bicuspid valve.  To get a clear look at the entire aorta, CT with contrast is fast and widely available. Depending on the facility available to you, MRI with contrast may also be an alternative - the test takes much longer but if one is not in an acute crisis it is also very accurate. Don't let anyone tell you that you are over reacting....what you are doing is hopefully ruling out something that can be very serious....too often the focus is on the bicuspid valve and not the aorta. You might want to print out the Circulation article I mentioned previously and share it with your doctor. I do hope your son's aorta will turn out to be just fine. In doing this testing now there will be a baseline to compare with, for both his valve and aorta, going forward. Best wishes!

[Nancy=Ann]

We're back from the cardio office, and I am more conflicted than ever.  The echo didn't show up from the ER, so they did a quick one (5 min) in the office.  The technician made the comment 'that's a generous one' while measuring his aorta, which to me said 'enlargement'.  The dr said, it's just because he's a big boy (6'1" 190lb) There is an ejection click and murmur.  The dr said there was mild stenosis and flow disturbance, along with regurgitation.  I drug most of this out of him, and he seemed disturbed that I had been researching anything on the internet. He said let him play football, it would break his heart not to play.  He also said most dr's would recommend that he not play. He also said the attack that he had was not related to his heart, but was a muscle spasm in his chest. I don't see how a muscle spasm could make his heart go into arrythmia, beat at nearly 200 bpm, cause sweat to pour from his body and his skin to turn gray. I asked several times about a possible dissection, but he said, nope, not that.  I asked if he should have a CT with dye or and MRI, and he said, nope.  He did order a stress test for next Monday, and said if he felt any chest pain to leave the game.  We will have the stress test done, but then I believe we will seek another cardiologist.  We live near Boise Idaho, and there are only 3 pediatric cardiologists in our area, and the one we saw is retiring Oct 1.  He looks to me to be in his mid 50's.  What would you do in my shoes?

[Cathy12]

It is reasonable in your case to seek advice from another cardiologist after another stress test. Since your son is 16, he is at the age when he can see an adult cardiologist as well (even though adult cardiologist see patients over 21 but in terms of pathology that he has and general body habitus he can see an experienced adult cardiologist in a major heart center). In terms wheither he should be active in sports, as a mother you know best what he should do. Many pediatric cardiologist allow children with much more heart disease than your son to be active in sports with no restrictions however it does come with risks. Basically if you let your son to become couch potato in a long run it is not good for his heart either, but if you let him overexert himself it is not good for his heart as well.

[shooie]

Hi Nancy:

I live in Boise, Idaho!!  Where does your son play football?  Which cardiologist did he see here?  So sorry to hear about his problems - it is soooo stressful as a parent.  I would definitely get that second opinion.

[Avel]

Hi Nancy Ann,
If this were my son (or myself!), I would be concerned about having a stress test done at this point in time. Someone with bicuspid aortic valve disease who has just had an acute incident that has not been explained should be handled very carefully. Were you told what the purpose of the stress test is and why it is being ordered?  Doing some gentle, minimally invasive testing first will tell a lot at this point in time, and that is what I would want for myself.
It is premature to make any assumptions right now about the degree or nature of your son's heart/aorta issues, and what that may mean in terms of activity level. Whatever happened to him the other day was a warning - and fortunately you can now go ahead with getting help to find out everything about your son's condition. Just knowing that he has a bicuspid valve puts you ahead of where you were in the past. What you do next is very important.
There will be time enough to figure out what level of activity is ok for him once there is a clear picture of his condition. If this were my son, I would not allow sports activity at this time. Gloom and doom about being a couch potato right now is just not appropriate. What is appropriate is to have an expert evaluation that is carefully and accurately done.
You are in a relatively small area, and it is very possible that no one in your entire state will be fully familiar with this condition. (Many centers in heavily populated areas would not be either.) If I were you, I would get a copy of the echo tape done in the ER and have it evaluated by an expert. I would also ask that expert to work with me on what testing should be done, and where it can be done. Even given your son's large size (which may be significant in evaluating him regarding this condition), I am concerned about the comment that his aorta is large - his aorta needs to be evaluated by an expert. Please get in touch with me, and I will be happy to share my thoughts on options for you to find that kind of expertise. It does exist! My email is ***@****
If you are interested in reading our experience with bicuspid aortic valve disease it is on the Aneurysm Support Group site at http://www.westga.edu/~wmaples/velebir_arlys.html and I can be reached via email from there also. Best wishes.

[ace4355]

I have a bicuspid aortic valve. I just had an ecg because i'm an applicant for the police force. When i read the ecg it said potential left axis deviation. Does this mean anything. I've never had problem in my life and have never been treated. i'm 25 years old. do you think this means bad news? Is it enough to terminate my application to the police force? Do you think it will slide in terms of their medical expectations. Finally even if its a minor defect, can it always be picked up on an ecg by any cardiologist? I"m lost and worried....

[Nik1]

My 42 year old brother just had value replacement surgery due to a BAV, my 48 year old Aunt had the same surgery 4 months ago.  Does anyone know BAV is genetic?  Thanks for the help.