Options for my son, age 13, bicuspid Aortic valve + server Aortic Regurgitation

[m1ffb]

My son was diagnosed with bicuspid Aortic valve when he was 3 months old.
We have been monitoring his condition and lead a mostly worry free life till 2 months back when our Cardiologist said that my son now has severe aortic valve stenosis.
He recommended getting a balloon valvuloplasty/catheterisation to provide some relief to the valve.
After the procedure at CHOP he now has severe aortic regurgitation.

My son was mostly symptom free before the above procedure, but now I can see the AR symptoms like high heart rate, tiredness and sometimes shortness of breath.

I have recently been reading messages in this forum, most of which are for AVR in adults, and could not find posts related to children with age around 13 years.

After doing my amateur research it looks like my son's options are

1 - Try to get his aortic valve repaired.
2 - If repair is not possible then get the Ross Procedure.
3 - If both the above, cannot be done then go for a mechanical valve.

Regardless of the options, it seems to me that he will have to undergo an Open Heart surgery (OHS).

Has anyone gone through or heard of someone with a similar situation ?
Do any of you have any recommendations for us ?
Are there any options other than those listed above ?
I have read about stem cell based valve generation and testing in animals.
Does anyone know anything new about it.

Given that my son is only 13 years old, my goal would be to get something done which will
- grow with him
- minimize or extend the timeline for any subsequent OHS.

Any and all suggestions are welcome and would really appreciate your support.

Thanks,
Worried Dad

 

[dick0236]

........... would really appreciate your support.

Thanks,
Worried Dad[/QUOTE

You have my support. There have been some parents of teens on the site although I don't know of any that are currently active. There are a number of current posters who went thru surgery in their teens and I'm sure they will come on with some good advice and insight. I will mention a support group dedicated to young patients.....look up "mended little hears" on line. That is a sub-group of the national "Mended Hearts" organization that is dedicated to heart surgery patients.

FWIW....I was also diagnosed with a heart problem in my teens, but back then surgery was not an option and I made it into my 30s before surgery. Modern medicine makes getting young people "fixed" and able to enjoy a normal life without hickups.

 

[pellicle]

Hi Worried Dad

first I'd like to say that a long time ago I was pretty close to being your son in terms of condition.

I had a (then called) valvotomy which may be the same thing that your option 1 is. That was done by OHS at that time (1974) and I think that the valvoplasty is done by catheter these days. That lasted me until I was 28 when I had another OHS which was a homograft (living tissue cryo-suspended) and then I had my most recent surgery back in 2011 to put in a mechanical.

To my mind there is no solution that gives you a one stop shop right now. Even the Ross will not deliver that.

Being younger its surprising how well one recovers from surgeries, I can say that my last one (my third) wasn't too bad but left me with a few lessons to show me how its good to try to minimise the surgeries.

I started in Australia I'm currently in Finland and I've lead a good life ... I say this to let you know that (as you're a father) that your son has every chance of leading a good and happy life.

Message me if you feel like a chat, (or email me, username @ hotmail . com) I'd be happy to call and talk or dig through some of my older posts here if you want (but there's probably too many for that)

Best Wishes

 

[Superman]

Your post really brings me back. I was also diagnosed in infancy. When I was 10 - 15 years old the balloon procedure was relatively new. I was being monitored fairly frequently (going in every six months if memory serves). We talked a lot about doing that very thing during those years, but elected not to. We were able to put off surgery until 2 days prior to my 18th (weird, I know - I don't like saying I was 17, because I turned 18 in the hospital. But I wasn't 18 at the time of surgery either). My activities were very restricted growing up though. Because of that, sometimes I wish they had done something so I could have a little freedom in that regard. But there's risk in everything, and now you've got some tough decisions.

As pellicle said - if surgery is needed, there really isn't a one stop fix at that age. Too much growth ahead. For me, it was put off as long as they could until they felt most of my growing was done. I was 6' 2" at 14 years old, but they still waited.

Of the options indicated, I would think valve repair would be best, but with a back up plan in place if it isn't going as well as expected. Reasons being:

- It would hopefully buy time and grow with him until a more permanent solution could be done (either mechanical or tissue replacement).
- and it potentially wouldn't require any medication longer term.

*personal opinion* I'm not a fan of the Ross procedure. Takes one bad valve, and potentially creates two bad valves in the long run. The only plusses are that it's the patients native material and potentially avoids a lifetime of medication.

If a repair is not possible and growth is an issue - I would consider a tissue valve at 13. Even if he only gets five or ten years out of it, he can switch to mechanical when his growth requires it.

I'll add that this really depends on his size now. If he's a small 13 year old with a lot of growth ahead - whatever they do now will need to be redone. If he's an adult size 13 year old, and they feel strongly they can put in an adult size valve that will last him a lifetime, then go mechanical.

 

[cldlhd]

First very sorry to hear about your son's situation. So was the procedure the cause of the regurgitation? CHOP is a great hospital but I guess they're not perfect. If repair is an option my personal recommendation would be to try to have Dr Joseph Bavaria do it. He did mine last year at HUP and although they have a few great surgeons according to the staff my wife and I spoke to the consensus was he's their best at aortic valve repair. I had mine done in February 2015. It was overwhelming enough having it done to me I can't imagine if it was my son. Any questions I may be able to help with feel free to ask. We're practically neighbors, I'm in Bucks county.
Sorry went back and read again so I see my question about the procedure was already answered. I was given the impression that repair was only possible if there was little or no stenosis.

 

[neil]

sorry to hear about your situation, for me I would be asking all the known experts in this field. Cardio AND Surgeons would be my main port of call ,

 

[m1ffb]

Thanks all, for your support.
We are planning to meet our regular Cardiologist tomorrow and will come back for any follow up questions / advice.

After the first couple of days of denial it has finally struck me that this is going to be a very long term ordeal especially for my son.

 

[dick0236]

............. this is going to be a very long term ordeal especially for my son.

Long term - yes............ordeal - no

Most of us felt that life would never be the same in the beginning, but found that most of our fears never occurred. Good luck to your son.....and you.

 

[Superman]

As a parent, I don't envy your position. It's definately harder to see our kids face challenges than to face them ourselves.

I cant pretend to know exactly what your son is thinking or feeling. All I can offer is my own perspective as a lifer. I can say that surgery, for me, was not a scary thing. It was a path to relief from a lifetime of restrictions. It's a tough few weeks, but as a teenager recovering, family friends that took me for my walks couldn't keep up. My brother and sister were both smokers and couldn't raise the spirometer as high as I could.

Since my open heart, I ran several 5k's, a 25k, was a pretty active mountain biker, and I still focus on more active vacations. I have an amazing wife and five wonderful children. Depending on how you define "long term", I don't see it like that. It's just my life with a couple big hurdles thrown in that I've cleared.

I hope his appt goes well and you have clearer direction after tomorrow.

 

[pellicle]

Hi

Thanks all, for your support.
...
After the first couple of days of denial it has finally struck me that this is going to be a very long term ordeal especially for my son.

firstly I'd like to mention that children do not see things like adults. You need to be careful that you are not projecting your own anxiety onto your son as he will see this entirely differently to you (until he starts to soak up your feelings and emit them as kids do).

The hospital staff will not be treating this as an ordeal, they will seem happy, supportive and confident, and your son will see your anxiety and trauma. He will be confused.

I was

Life is a process of learning, Auschwitz is an ordeal, medical treatments to save your life are not. I suggest you familiarise yourself with all the stuff about kids battling with cancer (who will in the main die) and observe that and learn.

Then grok that this is not a death sentence, its a life sentence.

Myself thought my life I have often said that my experiences (angiograms at 9, OHS at 10, other angiograms, lifelong checkups (till I became an independent adult) I felt it all made me who I am. Even while I was a 12 year old looking up to my cousins who were big and strong (10 years older), footballers, party going, drinking. I was at first wanting to be like them.

It did not take long for me to form a totally different view. This view has panned out exactly as that 15 year old kid predicted. They are all really ill from a life time of self abuse and I'm the stongest and fittest.

Being around hospitals provoked an interest in science in me. Soon I was sure that I wanted to do that with my life. By the time I started University (doing biochemistry and microbiology) I was focused on what was at that time not yet established. I wanted to work on research on genetic engineering to contribute to solving this issue because I was sure it was genetic. I was right: it is.

My "ordeal" focused me on learning, learning about the world, learning about science and helped me to become a thinker.

I know from reflection how hard it must have been for my mother and father ... Dad never really got it (kept shitting me off with crap like "poor little ******* went through ..." but it was always about him really. Mum was terrorfied (she was a farm girl so knew nothing about this stuff) but had nearly died from a ruptured appendix as a little girl so had faith that the medical system would save her boy. She did have a massive tendency to "wrap me in cotton wool" ... my responce to that was what every teenage boy would do. Rebel against that.

I've had a good life which was given to my by the work of many in the medical system. You should look at it that way too.

But now ... well ... I'm going out cross country skiing on the local tracks ... Think about what I've said.

 

[ashadds]

Hi



firstly I'd like to mention that children do not see things like adults. You need to be careful that you are not projecting your own anxiety onto your son as he will see this entirely differently to you (until he starts to soak up your feelings and emit them as kids do).

The hospital staff will not be treating this as an ordeal, they will seem happy, supportive and confident, and your son will see your anxiety and trauma. He will be confused.

I was

Life is a process of learning, Auschwitz is an ordeal, medical treatments to save your life are not. I suggest you familiarise yourself with all the stuff about kids battling with cancer (who will in the main die) and observe that and learn.

Then grok that this is not a death sentence, its a life sentence.

Myself thought my life I have often said that my experiences (angiograms at 9, OHS at 10, other angiograms, lifelong checkups (till I became an independent adult) I felt it all made me who I am. Even while I was a 12 year old looking up to my cousins who were big and strong (10 years older), footballers, party going, drinking. I was at first wanting to be like them.

It did not take long for me to form a totally different view. This view has panned out exactly as that 15 year old kid predicted. They are all really ill from a life time of self abuse and I'm the stongest and fittest.

Being around hospitals provoked an interest in science in me. Soon I was sure that I wanted to do that with my life. By the time I started University (doing biochemistry and microbiology) I was focused on what was at that time not yet established. I wanted to work on research on genetic engineering to contribute to solving this issue because I was sure it was genetic. I was right: it is.

My "ordeal" focused me on learning, learning about the world, learning about science and helped me to become a thinker.

I know from reflection how hard it must have been for my mother and father ... Dad never really got it (kept shitting me off with crap like "poor little ******* went through ..." but it was always about him really. Mum was terrorfied (she was a farm girl so knew nothing about this stuff) but had nearly died from a ruptured appendix as a little girl so had faith that the medical system would save her boy. She did have a massive tendency to "wrap me in cotton wool" ... my responce to that was what every teenage boy would do. Rebel against that.

I've had a good life which was given to my by the work of many in the medical system. You should look at it that way too.

But now ... well ... I'm going out cross country skiing on the local tracks ... Think about what I've said.

dick0236 and you are inspirations along with the many site members here..... my friends dont even think about having a health insurance at 26

 

[AZ Don]

Sorry to hear about your son's situation. I often say that the anticipation of surgery is the worst part of it, but as a parent I think that anticipation of a child's surgery may be worse. In any case I can tell you that OHS, for me, was not much different than other surgeries, and I've had a few. I was walking a mile a day the next week and swimming in under 3 months. I certainly felt up to doing more, sooner, but I held back because it was not advised. Surgical techniques and capabilities have vastly improved over the years. If valve repair is an option that sounds like a good choice. Perhaps a chance for no blood thinners or future surgeries. If another surgery is needed you buy time and there may be better options available then. I'm not aware of any that are ready yet but valves grown from stem cells and mechanical valves with no need for blood thinners are in the works. A mechanical valve is a sound choice but if a future surgery is required because of growth, perhaps a tissue valve is also an option?

The important thing to remember is that your son's condition can be treated successfully. I'll also mention that I had a surgery at 15 that scared me more than any others because adults kept trying to re-assure me that I would be ok and that the chance of dying was very small. It was not my first surgery and it was for my foot! I wasn't worried until everyone started talking so much about it. If it were my son facing surgery, when talking to him about it I would probably want to focus not on the surgery so much but what to expect afterwards. Many find it difficult to lie flat and so sleep in a recliner for a time (I did), coughing and sneezing hurt, the pain is generally managed well but some movements/positions may cause some pain for a couple months. The spirometer (breathing exercises) are important for recovery, as is walking, as is taking it easy and giving the breast bone time to heal.

 

[Agian]

It's definately harder to see our kids face challenges than to face them ourselves.

This is so true

 

[Agian]

Worried Dad, you will be anxious and sad. It's ok to cry, even for a man.
Your son will be ok.

 

[cldlhd]

Hopefully your next appointment will give some guidance as to what direction to go. If he was 5 or 6 years older and the valve couldn't be repaired durably I'd say the choice would be a mechanical valve but as he still has a lot of growing left to do it's a more difficult choice with different pros and cons.
A tissue valve -pros: Less likely to need warfarin during those teenage years when most boys think they're indestructible. Cons: Will definitely need another operation but if the valve lasts until he's fully grown he could then get a mechanical.
Mechanical - pros: the valve is unlikely to wear out .
Cons: He would need warfarin and assuming they would need to fit a much smaller valve ( may not be the case ) it would need to be replaced when he's full grown or he'd have restricted flow. So if they would need to replace it anyway that would negate what I think is the main advantage of a mechanical valve.
Repair- Pros: Likely no need for warfarin. Also since it would be his native living tissue it would continue to grow with him and there's a possibility for a lifelong repair although that's dependent on the condition of the valve and obviously you can't count on it.
Cons: There's always the possibility the repair could fail before he's big enough for a full size mechanical. That could happen with a tissue replacement also.
Then there's the possibility of future aneurysm that would require a reop one day regardless which way you go now. It's a lot to think about and I'm sure you don't need me to tell you this but do your research and write it all down so you remember to ask the surgeon what you need to. It's easy to forget something in the moment and the few meetings I had with the 2 surgeons from Penn they seemed to be in a hurry. Their time is important but so are your concerns so make sure you get the answers you need.

 

[tom in MO]

Your goals are:

Given that my son is only 13 years old, my goal would be to get something done which will
- grow with him
- minimize or extend the timeline for any subsequent OHS.

Your goals have some pre-conceptions that may not be realistic. As noted by others, OHS is not the worst thing in the world and generally valves don't grow. Your goal should be life for your son. Much simpler.

My suggestion is simple too. Find the best team to care for your child that you can afford and physically get to. Trust them, but educate yourself. Take notes and prepare questions before your appointments. Pray. It helps with the worry. I like rote prayer, such as the rosary.

Good luck, take heart that people with bicuspid valves live long lives once their problem is discovered. Your choices will be primarily between treatments that will all provide "life" to your son, so there is not really a bad choice. A philosopher once said that if the choice is hard, take heart because that means both decisions are good.

 

[cldlhd]

Well that was my point ,that IF the valve can be repaired with an expectation of reliability it will grow with him. CHOP is #2 nationwide and third in pediatric cardiac surgery so he's in pretty good hands.

 

[m1ffb]

My son is now stable for his condition if we greatly restrict his physical activity.
There is a very wide blood pressure gradient and he somehow does not complain about physical strain.
Doctor confirmed that he does need surgery and the question now is "when" instead of "if".
Given his age the Cardiologist wants to see if we can postpone the surgery by some time. We have a follow up appointment in 2 weeks to assess his condition again.

 

[pellicle]

Hi

Given his age the Cardiologist wants to see if we can postpone the surgery by some time. We have a follow up appointment in 2 weeks to assess his condition again.

I recall being on the waiting list for some years. I had regular xrays done and they were always wanting to defer surgery that bit longer .. without increased risk.

I had assumed from your first post it was a when not an if

Stay strong and it will all work out.

It's a lifelong journey

 

[Superman]

Given his age the Cardiologist wants to see if we can postpone the surgery by some time. We have a follow up appointment in 2 weeks to assess his condition again.

Given that it's your son's life, I would involve him in the decision as much as is reasonable. One of my one true regrets of youth is that my parents opted for the restrictions route instead of the earlier fix route. In hindsight - there are many times I wish I would've have that surgery at 12 so I could have played high school basketball or ran or did something instead of sit on the sidelines and wait.

I would talk to the doctor's about their view of his teen years if the surgery is done now. It appears from your post that no surgery = restrictions. So does surgery mean more surgery down the road, but freedom from restrictions now? If I had it to do over again - I would do that.

P.S. - I feel like this is some kind of time loop and I'm being offered the opportunity to talk to my parents then. Almost tearing up a bit here. Unfortunately no internet message boards in the mid 1980's.