Hello? New here, and frightened

[clouddancer22]

Hi, I am new here. My beautiful, wonderful, smart, 16 year old daughter needs a new valve soon. I am scared. You all seem to know what you are talking about. I am just freaking out.

She has severe aortic stenosis, and a bicuspid valve. My dad always had a murmur, and now they say he has a biscuspid valve. I always had a murmur, ummmmm bicuspid valve??? Don't know yet, don't care, I am worried about my daughter.

K, she had surgery at 3 months to open it up. Then a bunch of valvioplasties since then, last one was almost 10 years ago....... Her gradient is now over 100. No symptoms. Never. But the gradient is to high now, so probably the last valvioplasty ever next week on Tuesday. Why the last??? The valve has been leaking already, and this will probably do it in.

What are our choices???? I have heard of putting the pulminary valve over on the aortic side, then doing a replacement, maybe donor, over on the pulminary side.

Anyone know about this?? We live in California, I heard there is a surgeon at Stanford that comes to Childrens Hospital once a month.

 

[catwoman]

Hi, I am new here. My beautiful, wonderful, smart, 16 year old daughter needs a new valve soon. I am scared. You all seem to know what you are talking about. I am just freaking out.

She has severe aortic stenosis, and a bicuspid valve. My dad always had a murmur, and now they say he has a biscuspid valve. I always had a murmur, ummmmm bicuspid valve??? Don't know yet, don't care, I am worried about my daughter.

What are our choices???? I have heard of putting the pulminary valve over on the aortic side, then doing a replacement, maybe donor, over on the pulminary side.

You're referring to a Ross procedure, removing the bad aortic valve, replacing it with the native (patient's own) pulmonary valve and then replacing the pulmonary with a donor valve. (Other valvers: Please correct me if I'm wrong on the replacement for the pulmonary.) I had mitral valve surgery, not aortic, so I'm not up on AVR/BAV like others may be.

We've had several folks here who had Rosses as teenagers or young adults. Seems that some of them require replacements again and that replacement tissue valves may not have the same longevity/life span in a younger patient than in older ones.

I'm sure others will jump in here.

 

[Georgia]

Welcome to the site. I'm no help to you other than to say that you're very welcome here, and that others will be adding their knowledge and welcomes as they sneak a peek at vr.com while at work or finally get outta bed and get online.

You'll find a lot of help here; there are several moms of kids with heart issues, and quite a few people who've had Ross procedures, and those who weren't able to as well.

Search the site for "Ross procedure" and you'll find a wealth of discussion.

I'm sorry your daughter has so much to deal with at such a young age.

 

[PJmomrunner]

Hi Clouddancer. You've come to the right place for support for valve issues!

The procedure you refer to where the aortic valve is replaced with the pulmonic valve and a homograft replaces the pulmonic valve is the Ross Procedure. This is a good overview: http://www.ctsnet.org/doc/2380. There are a good number of people on this website who have had the procedure, among them PapaHappyStar, who originally posted the above link.

I sounds like your daughter has had several balloon aortoplasties and no open heart procedures yet. Is that correct? You must be beside yourself with worry...I would be too if it were my (15 year old) daughter. There are several members here who have teens who are either waiting for valve replacement or have had it. There are also some members who are now all grown up but had their valves replaced as teens (or younger!).

Hopefully, by conversing with people on this website and otherwise researching your options, you will gain confidence that your daughter is going to be okay. There are some extremely talented surgeons out there who specialize in congenital heart defects. Ask for recommendations and check out surgeons at major medical centers yourself (no doubt Stanford has someone) and find someone who does a high volume of cases like your daughter's.

I'm sorry you have already had to go through so much with your daughter and that you are facing OHS with her.

 

[Karlynn]

Welcome Clouddancer. I hope you are able to find lots of information and comfort here. This is a great group of people. We have some active members who have teenaged children who are the valve patients, so I'm sure they'll be along shortly. I have always said that I would much rather be the one with the valve problem than either of my children. You have my kudos for the strength you have in seeing your daughter through her heart issues.

 

[Emma]

Hello,
I'm a heart mum too although my daughter not a teen yet, shes only 6. But I am with you on how scary and worrying it is having your child go through surgery of any kind, let alone major heart surgery!
Chloe had a replacement valve following 2 failed repair attempts on it. Shes not had the Ross as hers was the mitral valve that failed but I do know a lot of people whose children have had very successful Ross procedures if you'd like me to put you in touch with them.
Sending you and your daughter lots of love. Am here if you need to talk!
Love Emma
xxx

 

[MikeB]

Welcome to the site! I also had Aortic Stenosis when I was born, you can read my sig, but I had my valve replaced at age 20, about 3-1/2 years ago. The Ross procedure was an option for me, but I chose not to get it because I didnt want to have to go thru another surgery or worrying about how long it was going to last. You can PM me if I can be of any other help to you.

Mike

 

[Mary]

Hi, I am new here. My beautiful, wonderful, smart, 16 year old daughter needs a new valve soon. I am scared. You all seem to know what you are talking about. I am just freaking out.

She has severe aortic stenosis, and a bicuspid valve. My dad always had a murmur, and now they say he has a biscuspid valve. I always had a murmur, ummmmm bicuspid valve??? Don't know yet, don't care, I am worried about my daughter.

K, she had surgery at 3 months to open it up. Then a bunch of valvioplasties since then, last one was almost 10 years ago....... Her gradient is now over 100. No symptoms. Never. But the gradient is to high now, so probably the last valvioplasty ever next week on Tuesday. Why the last??? The valve has been leaking already, and this will probably do it in.

What are our choices???? I have heard of putting the pulminary valve over on the aortic side, then doing a replacement, maybe donor, over on the pulminary side.

Anyone know about this?? We live in California, I heard there is a surgeon at Stanford that comes to Childrens Hospital once a month.

Since your father and daughter both have a bicuspid valve, you might want to contact Dr. Sharro Raissi at Cedars-Sinai for a consult. Sometimes there are more issues with a bicuspid valve than what is first assumed.
There have been some very successful Ross procedures performed on members. I don't recall off hand the age of the youngest member having one, but the Ross is certainly a good option. As your daughter ages and considers having children, the type of valve will certainly impact her decision.
Good luck; we're here if you need us.
Mary

 

[Perry]

Familiar with Ross

Familiar with Ross

Clouddancer22,

You are describing the Ross Procedure for your daughter. Her healthy pulmonary valve is moved over to replace her faulty aortic valve. This way her own pulmonary valve will live and grow with her as her new aortic valve. For her pulmonary side a human donor valve is usually used, though some other tissue valves can be used. The benefits are that her own transplanted valve will live and thrive and, I am told, a future issue with her donor pulmonary valve may not need an operation or perhaps even catheter treatment. A failed aortic valve replacement does need intervention.

This procedure made perfect sense to my wife and I in 1999/2000.

My 1st grade son had the Ross Procedure when 5 months old (following 2 valvioplasty procedures and then a valve replacement that didn't last). It was a tough time for sure.

The Ross still makes sense for me. I am scheduled to have the Ross Procedure in March 2006 - I am 42 and was diagnosed only 5 years ago.

I am convinced that the Ross provides the best chance for never needing open heart surgery again. My son may as his donor valve had to fit his little heart and my not be the right size as his heart (with his own transplanted aortic valve) grows into his teens.

We reacted so fast in 1999 we didn't have time to consider anything but the next best step to keep our little guy alive. I have met with surgeons at Hopkins and in Northern, VA and the choice is mine. It's a grand issue for your family and mine. The surgeon, on the other hand, may perform several Ross Procedures per week and many open hearts per week. We just need to have faith in the people and technology that keeps us going when we need help.

Perry
(if you prefer, reach me at [email protected])

 

[Lynlw]

hi my 17 yo old son

hi my 17 yo old son

Justin just had his 4thOHS to get his conduit replaced and get a pulm valve in May. If your daughter would like to talk to him, just email me and I'll give you his AIM screenname.
I know it's a scarey time, he went into his cath last jan , being pretty sure it wouldn't work, but hoping to put off surgery a little longer.Are you on any groups for CHD families? I belong to a few if you are interested, lyn www.caringbridge.org/nj/justinw

 

[clouddancer22]

OMG you guys are awesome!! So much info. Um, I am having a hard time processing all of this. But I have to hurry up and not be stupid. 16 years we have been waiting, and now, here it is.

Yes, she had open heart surgery 16 years ago. And a bunch of angio's since then. But we have been waiting for the final diagnosis that it is not working anymore. Here it is. A week. I think I need to take you guys up on talking. Not doing that great, but I have to, I am the MOM. Yep, I am the MOM. Single mom of four, they need me, and here I am, wigging out. Sorry.

 

[OldManEmu]

As you have suggested a Ross may well be a viable option for you daughter. I suggest that if this is what you are interested in for your daughter that you approach a surgeon that is experienced with Ross procedures. Not everyone is suitable, about 1% of those with BAV also have a bi-cuspid pulmonary valve and this should preclude a Ross. However if you have a surgeon that isn't versed in Ross's like one of our members did JCDavis81 and have a Ross done with a bi-cuspid pulmonary valve you are definitely setting up more problems. I have attached a link to a New York surgeon that specialises in Ross procedures and his web site explains them quit well. http://ps4ross.com/index.shtml If you have murmur you should also be checked for a bi-cuspid valve so you can take appropriate precautions. I was originally scheduled to have a Ross however my condition deteriorated to much before surgery so it was considered to risky as it is a long operation so I got a conventional AVR. I may get a Ross next time when the tissue valve wears out.

 

[PJmomrunner]

Single mom of four, they need me, and here I am, wigging out. Sorry.

WHO WOULDNT be wigging out??? YOU, my dear, are wigging out here! And that's the perfect place to do it. You've got nothing to be sorry about!

 

[clouddancer22]

OK, we do have a great surgeon. Someone who comes down to Children's Hospital from Stanford University once or twice a month to do special surgeries. I just am not sure what to ask for, or ask about.

Her gradient is 100 now. I am so worried.

 

[Lynlw]

my only concern would be if the great surgeon only comes to do the special cases, what happens if there is a post op problem? who would take care of that? Is it possible to go to one of the centers when the surgeon practice fulltime? If I was you I would talk to a few docs and hospitals, especially if you are interested in the Ross. When Justin had his last surgery, we needed a new surgeon since his wasn't in practice anymore and I talked to several different centers (actually 4) we ended up going to CHOP, but one of the centers I was looking at was Boston.
Is the surgeon that did the first surgery still around? Lyn

 

[Karlynn]

Clouddancer, I just happened to watch the movie "What the Lord Made" today and was so awestruck. It caused me to be so very grateful for living in the time we live in. The movie is the true story of Dr. Alfred Blalock and his assistant Vivian Thomas. Dr. Blalock and Mr. Thomas pioneered the "Blue Baby" project at Johns Hopkins. It was the first successful surgery performed on the heart and began the incredible field of cardiac surgery. To watch a portrayal of how doggedly these two gentlemen pursued a way to save TOF (Tetrology of Fallot) babies was inspiring.

We have come such a long way. Your daughter has great odds on her side! Our little web community here is a huge gathering of people with success stories and I know that your daughter will be one of them.

Take deep breaths. Study the different types of valve choices. Ask any and all questions of the doctors, and of us.

I pray for continued strength for you and a wonderful outcome for your daughter.

 

[clouddancer22]

Thank you so much. I never knew that having a bicuspid valve was a problem in itself. My father is 74 now, and I have always thought him quite healthy, except for the murmur. Me too, except that the doctors keep mentioning a murmur. Now I am getting worried about my other kids . . .

 

[Mary]

Thank you so much. I never knew that having a bicuspid valve was a problem in itself. My father is 74 now, and I have always thought him quite healthy, except for the murmur. Me too, except that the doctors keep mentioning a murmur. Now I am getting worried about my other kids . . .

Don't get too worried right now. Aussigal has three children, with two boys having a bicuspid, while I have five children and only one has a bicuspid. I ran a thread almost two years ago asking how many bicuspid aortic valve members had children with bicuspids, and there weren't very many.

There will be plenty of time later on to consider getting your other children tested. If none of them has a murmur, I wouldn't be too concerned at all.
Mary

 

[PapaHappyStar]

Hi cd22,

I had the Ross Procedure done for a stenotic aortic valve, your daughter may be a good candidate for it. The Ross Procedure could be a longer term cure for aortic valve disease than any other bio-prosthetic valve, but involves two heart valves so is a more demanding procedure in terms of surgical skill and practice. It is often done in children and young adults for multiple reasons:

-- no requirement for long term anticoagulation, there is a small but cumulative risk of adverse events for people with mechanical valves who are on anti-coagulants, the younger you are the longer you must be on anti-coagulants and the higher the risk.

-- the new aortic valve is native tissue, there are no issues with gradual calcification as in other bio-prostheses. Also a native tissue valve has the possibility of growing along with the rest of the heart.

-- the pulmonary valve is often a pulmonary homograft ( donor human pulmonary valve ), problems on the pulmonary ( right ) side of the heart are usually better tolerated and there may be surgically non-invasive ( catheter based ) ways of fixing pulmonary valves.

-- the hemodynamics of the new valve is very close to native valve hemodynamics, so the heart adapts better to changes in demand for pumping capacity on the left side ( for oxygenated blood to the body ). This makes the RP a good choice for athletes and for other people who might undergo unusual added stress to the heart for prolonged intervals ( e.g. pregnancy ).

There are some cautions as well:

-- There have been findings that the autograft sometimes dilates -- the reasons for this dilation have been debated in the literature, one may be that the pulmonary valve has a thinner wall than the aortic valve and so is less able to handle the added wall stress from the higher pressure circulation in the aorta ( compared to the pulmonary artery ), also if there is already some stress which acts to dilate the aortic root -- the new aortic valve may be suseptible to it ( these stresses sometimes cause aortic regugitation, so an RP is more favorable in people with primary aortic stenosis ).

-- There is more time spent on the heart-lung machine, this is usually tolerated better in younger people

-- The aortic valve disease becomes a pulmonary valve disease and you still have a higher possibility of aortic valve disease than in people with "normal" valves.

-- The operation is technically trickier because of the need to explant and re-implant coronary arteries and to explant and prep the pulmonary valve on the fly. You need an experienced and techincally superior surgeon, preferably one who has extensive experience with fixing congenital valve disease.

I think the Ross Procedure may be a good choice for your daughter provided you can go to one of the premier Ross surgeons -- she is too young for Paul Stelzer in NYC the author of http://www.ps4ross.com ( he does adult RP's ) -- you may want to check on the Ross Procedure list on yahoo groups ( website: http://health.groups.yahoo.com/group/rossprocedure/ ), you can send an e-mail to the list subscribers at [email protected] -- I am not sure whether you have to be subscribed to be able to do this though. They might have good info on RP surgeons. There is also a "ross registry" online: http://www.rossregistry.com/fcMain.asp.

Best Regards,
Burair

P.S. My max pressure gradient was over 100mmHg around 3 months prior to surgery, mean around 60mmHg -- its a serious matter but not critical, you and your daughter have some time to make the decision, hope you can get the best guidance possible -- please dont hesitate to ask for help of anyone on his forum, we all understand your worry and anxiety.

 

[Perry]

Ross Surgeon

Ross Surgeon

CD22,

Your Cardiologist ought to be able to hook you up with an experienced Ross Procedure surgeon. It's not like you are going to ask a surgeon to give it a shot.

I'm a bit confused myself because there are options (mechanical, tissue, Ross) and results differ - couldn't the hospital just tell me what to do? But once the Ross procedure became the primary focus for me, the first question to the surgeons I spoke with was "how many have you performed"? Both said about 100 with no operative or post operative issues.

Perry