Mechanical Mitral valve replacement and posiible Ross procedure for 12yo

[sarahsunshine]

Hi,

My stepson (12yo) will be having open heart surgery in the next couple months. He currently has a prosthetic mitral valve that he received when he was 5 months old. I do not know what type of mecahnical valve he currently has, but everything I’ve found out suggests that it is a St. Jude valve (circa 1999). He will be having surgery at the U of Alberta hospital with Dr. Ivan Rebeyka. I’ve not been at the appointments this time aorund, and my husband has not been able to contact the doctor yet so all conversation has been through stepson’s mom who is notorious who hasn't aske many questions, and maybe not given us all information. I will be drafting an email soon to send to the doctor/surgery team.

Anyway, from what I’m told, my stepson is to have his mitral valve replaced. He has aortic stenosis (bicuspid) and may need to have his aortic valve replaced as well. They are talking about waiting until they are doing surgery to make the evaluation of whether to replace it at the same time, or doing a Ross Procedure or not.

Can anyone explain to me exactly what the Ross procedure is?

From the internet I’ve found that it’s to switch the pulmonary and aortic valves, though the doctor did mention something about replacing the mitral valve with the pulmonary valve and then having prosthetic valves in the location of the pulmonary and aortic valves. I guess more dicsussion needs to be done, but has anyone herad of this?

A Ross Procedure (switching pulmonary and aortic) doesn't seem that great an idea to me since he already has one prosthetic valve. Why would you put a perfectly good heart valve at risk, when aortic valves can now be replaced in day surgery? Considering I will likely never talk to the doctor (stepson’s mom does her best to keep me out), could someone try to answer this for me?

Also, the risks involved in playing with three heart valves at the same time seem rather large. Because the aortic and mitral valve are on the same side, it is very difficult to get good readings on any tests (echo for sure, but even in a cardiac cath). For that reason it is very difficult to know exactly how bad his aortic regurgitation is. To me, wouldn’t it be worth replacing the mitral valve (that is obviously too small as it was put in when he was 5mo old), and later replacing the aortic valve in day surgery if required, leaving the pulmonary valve alone?

I know, I should be asking the doctor these questions, but any suggestions would be great!


I have so many questions:
What size, type brand valve do they think will be used (I would prefer On-X but I don’t know what they use here, or if we even have a choice)?
Do they think they can put an adult sized valve in and prevent future surgeries that way?
Would it make sense to use a tissue valve for the teenage years of denial and refusal (my stepson has been on medication all his life and I can see him refusing to take it. He’s already trying no to take the right dose so his INRs are too low relatively frequently, not to mention it’s tough to monitor it when he lives one week at one house, one week at the other, and his mom doesn’t always give him the right dose and gives him Ibuprofen).
What are the risks (physical blood trauma) with having 2 mechanical valves (or 3 if the ross procedure doesn't work) that are more than having just 1? Would you need an increase in warfarin dose? Higher INR levels maintained?
What type of surgery? traditional OH through the sternum, under the ribs, through the ribs?

Are there any other questions that I whould be asking?


Lastly, is there anyone here who has had a prosthetic valve replaced and a Ross procedure done at the same time?

Sorry for the book…

 

[Duffey]

Sarah, you ask many questions that are subjective in nature. I think your best first step is to read the bicuspid aortic valve forum and the presurgery forum so you can get a better idea of what is involved in your stepson's upcoming surgery.
You say he has aortic stenosis but then mention aortic regurgitation. Do you have copies of any of his tests? Those might clarify what is happening to the aortic valve.
The Ross procedure is not in favor as much as it was at one time. I am surprised that the surgeon suggested it.
I don't know about Canadian law regarding minors and health directives but will your husband have legal authority to weigh in on valve type? Usually the surgeon recommends the valve with which he is most comfortable using, and he will most certainly make the decision as to how he gains access to the heart (full sternocotomy, minimal, etc.)
There are many things to consider, and I hope your reading the various forums will help you gain a better understanding of what is involved. As I mentioned in your other post, we have several mothers whose children have congenital issues that required early intervention and more surgeries as they became older. Hopefully they will address more of your concerns.
Good luck and best wishes!
Mary

 

[kfay]

Sarah, I'm sorry you all are facing so many tough decisions for a 12 year old. Clearly, it would be great if both your husband and his ex-wife could both sit down and have a discussion with all the Dr's involved at one time. I'm surprised that a mechanical valve put in when he was so young has not already had to be replaced. They must have been able to put in a reasonably sized one when he was younger. I would think replacing it during this surgery would be very reasonable. He still has a lot of growing to do (a lot of congenital patients tend to have big growth spurts after interventions like these are done), and I would really have to question if a mechanical valve put in 12 years ago would be able to handle that kind of growth.

If it were my child, I'd probably be hesitant to go the Ross route. In theory, it's great for a child because that pulmonary valve will continue to grow with them in the aortic position. However, he already has one mechanical valve, so adding another shouldn't be that big of a change I wouldn't think.

My biggest issue if I were you is his non-compliance with coumadin. You guys don't want a teenage who is having strokes because he either doesn't take his coumadin properly or he isn't being managed correctly. A stroke would be a terrible consequence in someone so young who probably really doesn't yet grasp what that could mean. I think these things need to be brought up to his medical team for discussion.

Hopefully Lyn, one of our members who has really been through a lot with her son and his heart condition will be able to come and reply to you. She is a wealth of knowledge about these kinds of issues.

Best of luck to you and your family.

Kim

 

[sarahsunshine]

Thanks Fay!

My brother has Wolfe Parkinson White! He went misdiagnosed as mitral valve prolapse until he was about 35! Everyone looks at me like I have 2 heads if I mention that, but I know you know what I'm talking about! How strange! He manages it with meds (not sure which).

Thanks for your input and hello!

Yes, the doctors have been surprised that he's had relatively little trouble with the size of his valve. He's not a small kid, but skinny and average height, and that probably helps. He only weighs 67 lbs, but healthy.

I think there was a misunderstanding. They want to replace the Mitral valve, and possibly the Aortic when they go in (or Ross procedure), not the other way around. the other way around would make complete sense. My hesitation is that the Aortic may or may not need replacing at this point, they aren't sure (from what I've been told).

I guess the thing that really irks me about the whole step-mom thing is that I'm the person who does all ther research and asks all the questions. Then, I'm not "supposed" to take part in the conversations! Oy! Fortunately last time I spoke to the new cardiologist he gave me his email address and told me to email anmy questions any time. I just found his card so I will.

 

[tobagotwo]

It's not unusual to have both aortic stenosis and aortic regurgitation at the same time. It means the valve doesn't open fully and it doesn't close fully. I understand that the mitral valve is a separate issue that they are expecting to handle at the same time, "while they're there."

I would have a concern about using the Ross Procedure to replace a bicuspid valve in a child who has congenital heart issues, especially including an aortic valve that is failing at 12 years old and an already-replaced mitral valve.

In the Ross procedure, they replace the poorly-working aortic valve with the heart's competent pulmonary valve (because they are almost exactly the same), then replace the pulmonary valve with a human donor valve or a tissue valve. When it works, it's a beautiful thing. There is sometimes a crisis point with the human donor valve, which usually clears up, and then the valves function perfectly for the rest of the patient's life.

When the patient is a child, the added bonus is that the new aortic valve (that used to be the pulmonary valve) is the child's own tissue - so it actually grows with him or her, which prosthetics can't do. That can keep the child from having to have an extra operation to replace a perfectly functional prosthetic valve for another one in a larger size. This can be a wonderful advantage.

But the pulmonary valve needs to be a strong one to do this, and the more evidence there is of weakened (mixomatous) valve tissues elsewhere in the heart, the more likely the pulmonary valve is to have a touch of it, too. That means it's less likely to go the distance. Most Ross Procedures that fail do so after a few years because the transplanted pulmonary valve begins to leak and deteriorate like the original aortic valve did. Left alone in the less demanding pulmonary position, a borderline valve will most likely still serve for a lifetime. Placed in the high-demand aortic position, it's more likely to fail early, leaving him with yet another operation, three replaced valves instead of two, and further scarring of the heart.

Obviously, I'm not a doctor, and I don't know what his heart looks like now, have his history, x-rays, or any substantial knowledge of his actual case, so I'm not competent to give you more than general information and my layman's opinion. The doctors there are qualified to make this type of decision. However, it should be fine for you to bring this up as a question or concern.

It's so difficult and emotionally draining to have a child in this situation: I don't know how people manage through it. I hope you have great success with his treatment.

Best wishes,

 

[kfay]

Thanks Fay!

My brother has Wolfe Parkinson White! He went misdiagnosed as mitral valve prolapse until he was about 35! Everyone looks at me like I have 2 heads if I mention that, but I know you know what I'm talking about! How strange! He manages it with meds (not sure which).

Thanks for your input and hello!

Yes, the doctors have been surprised that he's had relatively little trouble with the size of his valve. He's not a small kid, but skinny and average height, and that probably helps. He only weighs 67 lbs, but healthy.

I think there was a misunderstanding. They want to replace the Mitral valve, and possibly the Aortic when they go in (or Ross procedure), not the other way around. the other way around would make complete sense. My hesitation is that the Aortic may or may not need replacing at this point, they aren't sure (from what I've been told).

I guess the thing that really irks me about the whole step-mom thing is that I'm the person who does all ther research and asks all the questions. Then, I'm not "supposed" to take part in the conversations! Oy! Fortunately last time I spoke to the new cardiologist he gave me his email address and told me to email anmy questions any time. I just found his card so I will.

I'm sorry, I didn't mean to imply because you are the step-mom you shouldn't be involved in the decision making process! I really just meant that it shouldn't just be his "mom" alone making these decisions. Of course you should all be able to meet with the Dr's and have some input.

 

[AgilityDog]

I will add two cents here, being a double valver with a third repaired, myself. I'd try to get the Onx for the mitral position, and for the aortic also if they decide against the Ross. It creates less turbulence than the St. Jude, being a newer model. Less turbulence helps preserve red blood cells. One potential hazard of mech valves is hemolytic anemia, i.e., damaged red blood cells caused by the turbulence.
Also, the Onx's better hemodynamics (better flow) appears to reduce the chances of clotting, which is very important in the mitral position, and will be doubly important if the patient has a reputation for non-compliance with his anticoagulation medication.

Personally, while I understand the attraction of a Ross for some patients, I figure if it's not broke, don't mess with it. Poor kid's gonna have enough scar tissue in there anyway.
And BTW, don't be surprised if they do a full sternotomy. He's a re-do, and if they have to do 2 valves, they need the access.

 

[sarahsunshine]

I would have a concern about using the Ross Procedure to replace a bicuspid valve in a child who has congenital heart issues, especially including an aortic valve that is failing at 12 years old and an already-replaced mitral valve.

Actually, my step son is an unfortunate case. Yes, he has aortic stenosis, but the mechanical mitral valve is due to a surgeon's error that wrecked his perfectly healthy mitral valve. For that reason, I don't know that one could truly say that there is evidence of weakened valve tissue other than the Aortic valve.

Thanks for the summary of the Ross procedure. It seems like a good idea in theory... But I don't know how it applies to the whole situation. I hope we can talk to the doctor soon.

 

[hannahsmom]

Hello

My daughter's (10 yrs old at the time) surgeon (Dr Bove of UofM) recommended her for the Ross procedure last year with mechanical being plan b. Within a short period of time in the OR we were informed that he decided to proceed with mechanical as he felt her pulmonary valve wouldn't last more than a few years as an aortic valve. As said above she was only 10 and he was able to give her an adult sized valve, that with all hope, will keep her put of surgery for decades or life.

I agree that the misuse of his coumadin should be a huge concern. Maintaining a stable target INR is the vital part of avoiding preventable health issues down the road. I cannot believe a mother would take such risks with such a delicate situation!

I wish the best for you and your family!

~Kelly

 

[sarahsunshine]

Kelly,

I am so glad that I have finally spoken to someone whose child has had surgery! This definitely helps for having to go through this with my step son.

I assume from what you said that Hannah had her aortic valve replaced. Is that correct? How was the surgery performed? Did they go through the sternum? How was her activity pre and post surgery?

Thanks in advance for your patience. Is there a particular place to find parents of children having heart surgery on this site, or even another?

 

[hannahsmom]

Good morning,

I only know of a couple of other moms on here. They post regularly, so I wouldn't be surprised if they eventually respond.

Hannah was born with a bicuspid aorta along with a narrow aortic arc. Her father has a very similar condition but has never needed surgery. She was diagnosed at 6 mos old and had open heart at 4 to dilate/repair the narrowed, stenoic valve.

She was involved in 2 different types of dance, gymnastics, and also is an avid swimmer. She was asymptomatic as far as we knew/observed, but luckily after her 1st
surgery her dr watched her closely, so we knew another surgery was coming.

Yes Hannah had her aortic valve replaced the Konno procedure (enlargement of her
aortic root due to subaortic stenosis). They went through the sternum. As far as I am
aware this is the only option for aortic valve replacement.

She had her surgery on a Friday (5hrs of surgery). We finally got to see her around 2pm and by 6pm she was off the vent. Catheter and the some other lines were
removed the next day. Left the ICU Sunday (was ready Saturday, but no beds available). The most important part of her recovery was to get get walking and
breathing exercises to expand her lungs. Coughing is painful, so she hugged a pillow when ciughing. The most painful part was removal of the chest tube, it's the last thing
they remove - taken out on day 4 I believe. We went home Wednesday. They have to make sure there's not a lot a fluid in the lungs and that her INR was within a safe range to travel (we were 3hrs from home).

Once we were home she improved very rapidly. Only took Tylenol before bed so she
would sleep. Appetite was very low for at least another week. White rice and Gatorade were her favorites. She was most comfortable on the couch surrounded by pillows. Was working on school work the morning after we got home. Definitely napped a lot and slept much better than at the hospital. She returned to school 3-3 1/2 weeks post op and returned to her dance and gymnastics 6 weeks post op. I didn't think she could have more energy than she did pre op but she does!!!!

We did weekly blood draws for the first couple of mos (she was not a fan of finger poking), then every 2 weeks, and about 5 mos post op we got get INR stable and she now goes once per month.

Overall she's doing great!!! All of her check ups have satisfactory, she has regular heart rhythm, and any damage done prior has repaired itself (yea!). The surgery was
necessary and was a blessing. I cannot stress enough how important it is to maintain a stable INR. The biggest risk for her stems from medication - keeping it in range keeps her safe and healthy. She has had a few minor cuts and bruises but nothing that was troublesome or caused any issues. No side effects as of yet. Per her dr we only give her Tylenol if needed and shouldn't take it at the same time as her warfarin.

On a side note she gained 15lbs post op and by the time we met with her cardiologist 2 weeks later she was 11lbs under her pre op weight. It took a while for her weight to get back up but she has since grown 3 inches and is at a healthy weight.

I hope this has been helpful. Feel free to send any additional questions.

 

[sarahsunshine]

Thanks for your summary. Hannah is one lucky girl.

My stepson (Skyler) had the same thing (narrowed aortic arch and bicuspid stenoic aortic valve). He had surgery to enlarge the valve at 9 days old and the surgery went wrong, ripping out his healthy mitral valve. They then did open heart surgery to fix the valve. It worked for 10 days, and then it blew out. They kept him alive for the following 5 months, through every infection in the book, until he went into heart failure. At 5.5 months, at 10pm on a Sunday night, they gave him the smallest mechanical valve they could find. He hasn’t looked back since. He has lost a lot of hearing due to the gentamicin (vicious antibiotic they use as only a last resort) that he was on and is mostly deaf in his left ear, and has high frequency hearing loss in his right ear. This is improved with the use of hearing aids.


So, she lost 15 lbs post surgery? That’s a little worrisome. Skyler ways 67lbs at best, and doesn’t eat much…