Ross procedure counter-indicated by BAV?

[Isfahan]

My son is 20 months old and has complex left-side heart defects:

1) Repaired hypoplastic aortic arch
2) Repaired coarctation of the aorta
3) BAV - mildly stenotic and slightly thickened leaflets
4) Stenosis below AND above the valve (critical level)
5) Parachute mitral valve, small side of normal (no regurg at this point)

We have received two surgical opinions both of which contain aortic valve replacement as an option if the valve cannot be saved. One surgeon recommends a bio-prosthetic valve, the other opposes this opinion because:

"we are really concerned about the Ross-Kono for patients with a bicuspid aortic valve (BAV). It used to be thought that BAV was an isolated phenomenon but current dogma is that it is more of a global tissue abnormality and we have seen a fair bit of literature to show that patients with BAV have early deterioration of the (pulmonary valve) PV in the aortic valve (AV) position because it was never meant to do that sort of work in the high pressure AV position and it has similar deficiency as the BAV in terms of tissue composition.

so people are feeling more and more uncomfortable about doing the RK procedure on these patients. If you call around you will find people willing to do it, but its not widespread anymore."

We are uncomfortable with the Ross because our son has so many left-side issues anyway that we are worried about including his pulmonary valve in his pathology BUT we respect the surgeon who has recommended it.

Does anyone have advice FOR YOUNG CHILDREN regarding valve replacement options with BAV - our major considerations are bio-prosthetic vs Ross-Kono.

Thanks
Shannon
Mother to Wren with Shones Syndrome (20 months) and Frost (HH)

Heart & Family blog:
http://echobaby.blogspot.com

 

[Duff Man]

i'm just thinking out loud so to speak, but it seems like eventually your son will need re-operation anyway without the Ross procedure because of prosthesis mismatch as your child grows. It's my understanding, and I might be wrong, that the Ross gives the child an aortic valve that grows with him. The pulmonary valve might be less likely to have a problem with outgrowing the prosthesis just because there's less demand on it.

I don't really have an opinion one way or another on what should be done because I do believe there is credence to the current dogma. So it's a tough spot. I pray that you get better answers quickly. I think this is the best place to find answers though, other than your doc.

 

[Mary]

How long do they estimate before he will need valve replacement?

 

[Isfahan]

He requires OHS within two months for the stenosis either side of the valve. They will need to "address" the valve at the same time - not because the stenosis is critical but because it may be insufficient after surgery and/or may be impossible to get the blockage since it is so close to the valve.

 

[Mary]

I have/had a genetic bicuspid valve and my 22 year old son has a genetic bicuspid valve. Mine has been replaced by a bovine pericardial, but I researched the Ross extensively before the replacement. I mention this just because I am going to offer my own opinion, as you requested.

I would go with a tissue valve and not undertake the Ross-Kono. I would be concerned about the connective tissue issue and worry that the pulmonary valve wouldn't last in the aortic position. I don't know how long a porcine or bovine valve will last in a 20 month old, but it seems that you are facing a replacement later whichever way you go. The Ross is a much more complicated surgery, and I would shy away from it at this time.

Best wishes and please keep us informed through the coming months.

 

[Isfahan]

Its interesting that the surgeon who is recommending the Ross is Dr Hanley who is one of the 'gurus' in pediatric cardio-thoracic surgery, does 100's of procedures a year including some life-saving ones he pioneered:

http://www.lpch.org/findADoctor/search/doc.pl?doc=16523&resultSet=16523

We would have to travel to see him in California (from Seattle) so that is not a concern at all. We will go wherever we have to.

BUT

Surgeons seem to have their preferences in approach and as our cardiologist (also a very well regarded and awarded fellow) explained, no valve solution is right for everyone. Because my son already has two valve involvement - do we want to take the risk of THREE with the BAV complication a possibility?

He feels not.

We have a telephone conference with Hanley on September 18th and meanwhile have scheduled surgery at both centers (I have told them that we are doing this) until we have clarified the approach.

We would not ask our local surgeon to do the Ross and are leaning away from it ourselves... but this world renowned surgeon is proposing it. Argh.

 

[Duff Man]

I lean toward the Ross because the child will probably outgrow that prosthetic valve quickly, whereas the intent of the ross is to have the valve grow with the child. Pulmonary valves don't have the kind of demands an aortic valve has, so the prosthetic will probably last longer in the pulmonary position.

I personally think the chances are higher for earlier reoperation with the aortic bioprosthetic.

 

[Isfahan]

The surgeon advocating the bio-prosthetic believes he can get a 'small adult' size in so they expect degeneration not size to be the complicating factor in re-operation.

 

[Duff Man]

the way I understand whats going on though is that on either side of that valve, there's an obstruction of tissue that needs to be removed... which might mean that the annulus is going to be smaller than usual for his age under normal circumstances. So if it's too small for his size right now, I dunno.

 

[Mary]

Its interesting that the surgeon who is recommending the Ross is Dr Hanley who is one of the 'gurus' in pediatric cardio-thoracic surgery, does 100's of procedures a year including some life-saving ones he pioneered:

http://www.lpch.org/findADoctor/search/doc.pl?doc=16523&resultSet=16523

We would have to travel to see him in California (from Seattle) so that is not a concern at all. We will go wherever we have to.

BUT

Surgeons seem to have their preferences in approach and as our cardiologist (also a very well regarded and awarded fellow) explained, no valve solution is right for everyone. Because my son already has two valve involvement - do we want to take the risk of THREE with the BAV complication a possibility?

He feels not.

We have a telephone conference with Hanley on September 18th and meanwhile have scheduled surgery at both centers (I have told them that we are doing this) until we have clarified the approach.

We would not ask our local surgeon to do the Ross and are leaning away from it ourselves... but this world renowned surgeon is proposing it. Argh.

Sorry that I didn't catch that you are weighing a cardiologist's recommendaton with a surgeon's. If Dr. Hanley is a true guru, he should be up-to-date concerning the BAVD connection. Why don't you inform him of your concerns at the phone conference and see what his response is?

When I was discussing the Ross with Dr. Stelzer, he said I was a good candidate for it since I had aortic stenosis without regurgitation and no dilation of my aortic root or aorta. Sometimes surgeons change their mind on doing the Ross when they actually open the heart and view the tissue and structures. Dr. Stelzer did switch to a bioprosthetic valve, rather than performing the planned Ross procedure, on one of our members for this very reason.

I personally would go with the most skilled surgeon I could find and then go with his recommendation.

 

[Duff Man]

I don't want to inject catastrophic thought in to the mix, but as long as we're ruminating.... The bundle of His is right there near the annulus that is, from what I can make of what I've read, too small in either case. If the surgeon that proposes the bioprosthetic does some kind of corona procedure to make a larger valve fit in the insufficient annulus, it could damage the bundle. Maybe that's really far out there... I don't know.

 

[Isfahan]

Sorry to confuse you. We are weighing the opinions of two surgeons. The view of one surgeon is in writing and the other one has been conveyed to us through our cardiologist. with whom he works. So the cardiologist seems to favor the local surgical approach although he was encouraging us to consult the expert for his opinion.

I am going to raise these issues on the 18th but as far as I have read the surgeon is the one who makes the call on what kind of valve to use and the written opinion only mentioned his recommendation, the Ross.

Ideally, we would simply go to him but ask him to do the bio-prosthetic if that is what we decide is best [with cardiologist].

 

[Andrew'sMom]

What a tough situation you are in. I have seen a few posts mentioning Dr. Stelzer. I don't believe he does pediatrics, but Dr. Jan Quegabur (I AM ABSOLUTELY SURE I DID NOT SPELL THAT CORRECTLY! I spelled it how is is pronounced, but if you do a search google or on here with at least part of his name I am sure you will find him!) is supposed to be very highly regarded when it comes to pediatric Rosses. After reading through this thread, I tend to lean toward whoever said -- we have 2 valve issues already, why make it 3. I'm sorry that you are in this difficult position. I'll keep you in my thoughts & prayers and watch for updates on your son. My very best wishes to you all.