Critical Aortic Stenosis (again).. Maybe

I am drinking carbonated pomegranate juice, from France, orzo salad, sushi and some pesto.  Wren has deviled eggs, sushi and madelines.  Josh has a Metropolitan market caesar salad.  If we can't kick this heart defect at least we can kick back and enjoy some fancy victuals.

To cut to the chase - Wren's first cardiology clinic in 15 months had mixed results.  It could always be worse but his aortic stenosis appears to have advanced from moderate to critical.  Critical is 50+ gradient over the valve.  His was 55.  Last appointment it was 45.

Now, that is where the clarity ends.

Dr Conwell was very amiable and calm.  He said:

1. The mitral valve looks about the same.  It has mild regurgitation, no evidence of pulmonary hypertension and is abnormal.  It is not a parachute valve but has short chordae and abnormal, restricted, motion.
2. Wren has mild left ventricular hypertrophy (thickened heart), we knew this, but he does not know if it is worse.  This is significant because they would expect some thickening if, as the numbers suggest...
3. The aortic stenosis has gone from moderate to critical and we need to talk intervention.

Actually, he said that he will review with our primary cardiologist and look at the images again to judge if this is a genuine increase or an anomaly due to angle of view.   He will call us within a week or so to discuss what they think.

They want 24 hour halter study data - Wren is wearing the halter now.  It is a set of electrodes stuck to his upper body to measure his EKG all day.  This is because Wren has left bundle branch block as a result of his last surgery - the electrical impulses are already a bit abnormal because of the damage to the bundle.  They want to know if the damage was more widespread and would indicate the need for a pacemaker.   This is not really a new thing, but if Wren needed surgery they would want it put in at the same time.

They did some chest x-rays to see if his heart is enlarged.

We will get some results back in a few weeks from the halter test, which will last until tomorrow afternoon.   Josh is teasing Wren that he is like a robot.   The nurse told him he is like an astronaut because they have to wear them all the time they are in space.

IF it seems the numbers are raised, we will repeat the echo in 2-3 months and also present to the cardiology conference.  He suspects they would recommend a cath lab intervention to try and open the bicuspid aortic valve leaflets further.  Other than a replacement, the cath lab might work as well as open heart surgery for this repair - he says.

I would want a second opinion from Dr Hanley's team and have said this.

So, we wait for:
1) The outcome of the discussion between Mark and Jeff.
2) The results of the chest x-ray.
3) The results of the halter-monitor
4) Any comments from the cardiology conference discussion.

We are disappointed but not reacting too much, yet, as there seems to be a lot of vagueness in the air.  Something bad is suggested.  Chocolate pudding is indicated.

2008 Guidelines for the Management of Adults With CHD

For parents of children with CHD's only (you won't be interested otherwise). I found this November 7th publication on guidelines for management of adult patients with CHD's. I know some of the issues have been discussed at our group before. It is a PDF file you would have to download.

Cardiology/American Heart Association Task Force on Practice Guidelines:
Executive Summary: A Report of the American College of ACC/AHA
2008 Guidelines for the Management of Adults With Congenital Heart Disease.

It outlines management issues for CHDs including patients with Shones and many issues associated with LVOT obstruction, criteria for valve replacement and issues with bicuspid aortic valves.

Heart Surgery is a Team Sport

After meeting with our cardiologist again this morning to discuss the Seattle and Stanford options we still do not know whether we are on Team Stanford or Team Seattle.
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NEXT STEPS:
Sept 18th 9am - Phone conference with Dr Hanley
Sept 18th 1pm - Second opinion with Dr Krable
Sept ?? - Early surgical consult with Dr Cohen
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Here are Dr L's responses to my questions about the Seattle Plan to save valve if possible but otherwise replace the aortic valve with a bioprosthetic:

1) If BAV is a connective tissue disorder causing dilation of the aortic root, what complications would this cause with a bio-prosthetic valve replacement?

Yes, this could become a problem. In Wren's case we don't see any dilation of the aortic root at this point although we wouldn't expect to. In fact, he has restriction in this area. But over time if this became a problem it would affect the valve in the same way as a pulmonary graft would be affected (leaking, etc) but at least his pulmonary valve would not have been damaged - it would still be in place.

2) In your experience, what is the likely lifespan of a bio-prosthetic valve from either deterioration or being outgrown?

The growth and degeneration are two processes that occur but we expect it to last about 10 years at this point. It can be less - say from 5 years but some last longer. In the Ross procedure the pulmonary valve has lasted more than 20 years in some people but it is more likely to last 20 years in adults, say from age 20 to 40 than it is to last from Wren's age to age 20.

3) What size valve is likely to fit Wren and what are the techniques and risks of inserting a larger valve in this position?
The surgeon can make pretty much any size valve fit. There are risks - you can ask the surgeon about those. The main risk is that if the valve is too big, we occasionally see this, it can obstruct the natural motion of the mitral valve because it needs something to force against but it can't be too big. So sometimes we see this post-op and we have to go and fix it.

4) What procedure will be used for the sub and supra valvular stenosis (patch? carving?)
We would use a Kono Procedure which would cut away the septum here [will insert picture] and patch that area. It will be sewn back to muscle in about 270 degrees (this is a three dimensional area) but about 90 degrees will be a patch.

5) What is an average recovery time for this procedure?
Ah, it depends. About 10 days... 12 days?

6) Could you put me in touch with someone who can give me information on the Number of cases and outcomes for this procedure?
That is something you will have to ask the surgeon. You can speak to Dina and schedule that surgical consult sooner [currently for 2 days prior to surgery].

When I asked Dr Lewin what he would do he didn't actually give us a recommendation. He emphasized that he had been part of the decision which is now "Dr Cohen's approach" - to do the valve replacement not Ross if the valve is not salvageable, implying he is party to one side of the controversy. He also gave us a lot more background on his views - how he has worked with and respects Dr Hanley a great deal, used to propose the Ross-Kono for all patients in this situation, saw many PV failures, changed his views and now is opposed in cases of BAV.

He said that if we don't reach our own decision about where to go we really have to decide "who to trust".

On one hand he feels that we have to choose a procedure because "this is a point of divergence" between Cohen and Hanley. He feels that Hanley will say that in my patients I don't see this problem [degeneration of PV in A position in patients with BAV] and perhaps "I do the surgery in a way to.." and we will have to see whether we are persuaded.

On the other hand we can consider the institution because "heart surgery is a team sport." On this count, he argued that the institutions themselves have similar levels of infrastructure and outcomes post-op but that the determination should be on the surgeon.

"They will make similar decisions with a different set of eyes"

I said that I felt popular views were that Stanford is better equipt and does higher volumes than Seattle. He felt that much of this information was "a popularity contest" although he laughed about saying this from a lower ranking on the List of Best Hospitals and questioned what volume was necessary for expertise to build up. He feels that the 12 bed CICU constantly full gives the Seattle team enough expertise to do a good job.

But we should also consider the level of family support we have here vs Stanford "in case a 2 week hospitalization turns into 2 months".

Josh was quite persuaded by this argument - he feels the advantage of being near home more keenly. I am more impressed by Hanley's reputation of excellence.

I have asked for copies of the notes and the cath tape to take to our NEXT second opinion on September 18th. We are going to see another cardiologist - Dr Kimberly Krable - who saw Wren prenatally.

We are hoping she agrees with someone so we have help with this impossible decision.

Do you want to help? We could decide by poll. Apparently there is so much doubt, projection, controversy and big UNKNOWNS that it would be as good an approach as any.

Stanford Opinion & discussion "To replace or not replace AV"

Here is the summary of my conversation with Dr Lewin. He is sending me a copy of the letter.

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The team Looked at the cath and echo findings. They agreed that Wren did need 'intervention'. Surgery should be done relatively soon given the parameters.

In particular, he needs relief of subaortic narrowing and supra-valve narrowing.

Approach:
They were cognicent of the small size of the valve but felt could potentially be spared and were hopeful and inclined to take the more conservative approach of leaving in the valve.

In leaving the valve behind they know its not going to be functional long term...

[Dr Lewin's comment: Of course it is preferable to leave in the AV in a young child because the alternative is not optimal but I am very worried about the valve....]

After the surgery for the repair of the SAS and supra-valve stenosis we will test the AV valve gradient and the fallback option, should we have an unacceptable gradient will be the Ross-Kono procedure.

[Dr Lewin spoke with Dr Cohen (Seattle Surgeon) about this approach and they are concerned about the issue of multiple bypass runs and potential cardiac dysfunction as a result of this strategy. If the AV gradient is too high they would have to put Wren BACK on bypass and expose him to further risk of heart, brain damage. Overall, a much longer run on bypass increases the risk considerably. Obviously, they are tolerant of a higher degree of risk in surgery while we feel more conservative there.

[Dr Lewin continues...."In a small child I am always in favor of saving the valve...BUT in this case we have already had intervention done on the valve -so I have added concern about leaving a valve that already have intervention on.

Also, Dr Cohen said that he can potentially get in an adult size valve which would delay the need for replacement...]

HANLEY: Go ahead with Ross-kono

[Dr Lewin: "Up here 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.

It may be that that was a more generic statement about replacing the valve.. but...

Now, the Pacific NW seems be to endemic for AV disease so we have a lot of experience seeing what happens long term when you intervene in the AV and we are pretty nervous about that whole procedure and what Dr Cohen was recommending was a bio-prosthetic valve - a metal scaffolding with a valve in the middle which is better than pure animal valve - it degenerates slower than pure animal valve.

His answers to some of my Qs:

Q: How do they decide whether the valve should be saved?
A: In making the decision on whether to keep the valve they look at:
Real valve size.
Looking at leaflets, how thick, how pliable, how much motion.]

Q: Would he be on Coumadin with the bio-prosthetic?
a: No, It tets to the point of what is the best choice - mechanical valve you get adult valve size but you require anti-coagulation but not with a bio-prosthetic.

Q: What is the timing? You said "soon" - what does that mean
A: Its not a set time. If we did nothing he may be OK in a year or he may have a rhythm disturbance which would be [long pause] catastrophic.
Q: You mean he could have a heart attack and die?
A: Well, yes... or serious damage to the heart...

Q: Dr Hanley is very highly regarded. Why would he recommend the Ross-Kono if it is falling into disrepute? Could we call and ask whether he would do another kind of valve replacement?
A: Well, he wrote ROSS-KONO on the report and he didn't just write this off the top of his head so that is what he thinks we should do. We could call and ask but... well, this is what he thinks here. I presume he thinks that the articles, the data, is flawed and that we can 'get away' with the Ross Kono in the presence of BAV disease. I don't know....

My thoughts:
I am out of my depth in making this decision. Clearly, Dr L leans toward Dr Cohen's views. He is concerned about the Ross-Kono. I don't like the idea of a Ross-Kono either but I trust Dr Hanley's reputation. I like the idea of staying here but if there is a judgment call to be made I err on the established judgment of expertise - Stanford BUT I confess I have been swayed by this information about Ross-Kono and BAV PLUS neither of us want the R-K and another valve-replacement. I like the idea of a longer term more conservative appraoch.

BUT both surgeons present their approach as more conservative. Dr H's team are more conservative because they want to save the valve. Dr Cohen will also save the valve if he looks at it and thinks it may last a while [he said that too] BUT he is more suspicious of it right now. He feels that avoiding a long period on bypass AND another re-operation soon is more conservative.

Who is right? I Dunno.

I have set up an appointment with Dr Lewin for MONDAY 5th at 9am. Josh and I will talk through the issues.

AND

A Second Opinion on this mess with Dr Krabill at Swedish. Her earliest opening is September 18th but I can phone for cancellations "as often as you need to" said the receptionist.

Wait, I need to call again immediately.

Perhaps I am too wary but I feel that while our doctor here is fabulous he cannot be the source of the dispassionate advice I need right now.