Consultation with Dr. Evan Zahn

Consultation with Dr. Evan Zahn

May 23, 2005

Black font is Valerie and/or John Breder

Red font is Dr. Evan Zahn, Chief of Cardiology, Miami Children’s Hospital

How many times can you balloon the pulmonary valve without causing damage?  Is this something you will continue to do even after the Glenn surgery?

Nobody knows the answer to that. We have ballooned them 2, 3, 5, 6 times.   Remember, his pulmonary valve is very abnormal and stiff and rugged.  I don’t think it’s how many times you do it, every time you do it you’re tearing it but so far we’ve been able to do Zach’s without making it very regurgitant.

I had heard that some regurgitation can be a good thing because it brings more volume into the right ventricle.

Some people say that and we like to say that too when we have it because then we don’t feel as bad about it.  I don’t think anybody knows the answer to that.  With a shunt that Zach has it can be a very big problem because the shunt will pour blood into the right ventricle and then nothing goes forward.  I certainly wouldn’t give him leaking on purpose just to help his right ventricle grow. 

Zach has been ballooned twice.

I would think he may need something more done to that.  It’s not necessarily just the valve; the whole subvalve area is narrow.  We’ll look at his angio and you’ll see what I mean. The ballooning isn’t gonna help the muscle underneath there.  We may have to slice that open surgically and re-do that. As to future balloon procedures, yes, if he needs it.   What we try to do for each of the kids is to look at what were they given, where can we take them to, how close can we get them to a normal circulation where you have two ventricles and all of your holes are closed.  So with Zachary, I still think we don’t know.  Ideally we like him to have 2 ventricles and all his holes closed.  But the fact that when he came back for his second procedure (the second ballooning of the pulmonary valve) his right ventricle was still so small makes it less likely than on the day of life one when we say well maybe if we can just provide some flow this thing will grow.  Well now it’s gone through a period of time where it hasn’t grown.  Does that mean it never will grow? Don’t know, but with every month you become a little less encourages.   So then you say can we include that ventricle at all in the circulation.  And with Zachary I think we’re still up to maybe.  And if you can’t include, then you exclude it and you’re up to a fontan.  We don’t know if Zachary will ever need that.  So our concept would be this one and a half ventricle repair which is not a new thing. And what that does is you have blood coming from the lower half of your body, the inferior vena cava and blood coming from the upper half of the body, superior vena cava.  We say, well his right ventricle is too small to handle both of those.  So what we’ll do is take the superior vena cava blood and we’ll plug that directly to the lung arteries, that’s the Glenn and we’ll let the inferior vena caval blood, which as you get bigger and you walk around and the lower part body becomes the more important part of the circulation; we’ll let that get pumped through his right ventricle.  It’s not a two ventricle circulation; it’s a one and a half.  Then the only thing to complete that one and a half would be to close the whole between the atria which is usually something we do in the cath lab, he wouldn’t even need any more surgeries.  So, it’s possible that the Glenn would be the last thing he ever needed.  It’s also possible that we look at this right ventricle two or three years from now and see this will never handle the whole circulation.  He’s still too blue. We have ways to test this.  We can test occlude the ASD and see what his cardiac output does and his SAT’s. We might say, you know what, it’s just hoping against hope.  It’s just too little, it’s never gonna be a real player and he needs one more operation, he needs the fontan.  We can’t tell you that now because it’s not clear.  His ventricle is borderline.  A lot of kids we can tell their parents the day they are born that they will need the fontan.  Their right heart’s just not there, its too small.   Zach is sort of on the borderline.

And you’re not just saying that to make us feel better?

No, no, we don’t do too much of that.  We just don’t know yet.

We’re really as comfortable as we can be with Zach’s condition.  Having said that, please don’t candy coat anything for our sake.

I can only tell you what we know.  I can tell you what we don’t know but then I have to tell you we don’t know that.  We just don’t know.

Why are they scheduling Zach for the Glenn surgery when his Sat's are still in the 80's? What are the risks/benefits of waiting as long as possible to do the surgery?  What symptoms make this surgery advisable now?

His SAT’s aren’t gonna change with this operation.  As a matter of fact they may even be a little lower.  So we’re not just doing this for his pulse oximetry.  A shunt is not a great circulation for many reasons.  It’s the best you can do to a newborn because you can’t do a Glenn on a newborn.  But it’s not the best circulation; it’s a plastic tube which can close at any time.  That would be bad and we have seen that.   It wouldn’t be fatal for Zach but he would get very blue and very sick.  You know it’s a tiny tube and it’s not gonna grow and he’s growing. So we don’t have a choice but to replace it.

Could I interrupt you there for a second.  Do you have a model of a heart we could look at to get a better understanding of all this?  So the main artery where the valve is, is called the pulmonary artery and the ones coming from it are called the branch pulmonary arteries?

Yes, there’s the main pulmonary artery and then there’s the right and left pulmonary arteries.

And the right one is where the Glenn attaches to?  And that gets blood to both lungs?

Yes, and as you might expect, more blood gets to the right one than the left.

Using a diagram of a normal heart:  Points to inferior vena cava, superior vena cava.  The blue blood is supposed to come back here go through the tricuspid valve, go into a nice big right ventricle.  With Zach there’s not enough room for all the blue blood to go out here, so he needs this shunt.  So what we are concerned with about the shunt is 1) he’ll outgrow it, 2) it can narrow, 3) its extra work load on the heart.  What you want it to get him as close to all blue blood going through his lungs as possible just like us. He has pink and blue blood.  So you do the Glenn.  Now what you have is blue blood going into  the lungs.

So all of the blood from his upper body will go directly to his right pulmonary artery?

 Right. He’ll still have a hole through his atria with some blue blood going over so his SAT’s will be about the same.  And hopefully as he gets bigger we would come back and test occlude that in the cath lab and see can his little right ventricle can be enough.  And if so, we’ll close that hole and he’ll be 100 percent saturated.  I can do that now, make him 100 percent saturated but with that ventricle so small, he’ll be 100 percent saturated but really sick.  So we’d rather have him a little blue and not sick.  So that’s the rationale for doing the Glenn.  Nobody would leave him with a shunt forever.

If everything miraculously starts growing, at the very least you’d still have to go back and get the shunt out, right?

We’d do that in the cath lab.  It’s not that big a deal, we do it all the time.  And we do it for this disease when the kids outgrow the shunt.  I just don’t see that happening with Zach.  The shunt is bad for your heart, its extra work for the heart.   Really, if we had a better thing to do to kids as newborns, we would do it, but we don’t.  So the sooner you can get that out of the picture, the better.   We also don’t want it to just all of a sudden one day close.   That would be for him.  The Glenns don’t that, they’re big, wide structures and they don’t just clot and they’re not as much work load on the heart because it’s blue blood going through it.  So that’s the why.  The typical age is somewhere between 3 and 6 months.

And we’re at 9 months already, so have we pushed it?

We would want to get that shunt out soon.  When I say 3 to 6, that’s typical.  He’s not sick and we’re not pushed to do it.  But at a year, I think it becomes pushing the envelope.   I would do it.  It’s just more wear and tear on a heart that’s already under wear and tear.

What are the hospital’s success rates, short and long term, for the Glenn?

If you mean survival through the operation, it approaches 100 percent.  You should discuss this with the surgeon.

Is there a concern with the “tenting” of Zach’s right pulmonary artery at the shunt insertion? 

The tenting means the shunt has sort of pulled up and deformed the artery a little bit.  When they do the Glenn they generally fix that.   It’s right where they’re working.  It shouldn’t be a problem.

Looking at the angiogram of Zach’s heart

Here you can see his right ventricle and there is some blood going in there.  Part of the problem is it has to come through this valve (tricuspid) which is narrow.   There is some blood going into the right ventricle but it’s muscular.

Can you ensure that either Dr. Hannan or Dr. Burke will perform the surgery?

Yes, we are not a teaching hospital and it would only be one or the other.

We also would like to ensure that Dr. Laguerella attends as the anesthesiologist.

That you have to request.  You can do that.  When you schedule surgery, you make it known that you want Dr. Laguerella.

Will they be using the "beating heart bypass" during the Glenn and how long will he be on bypass? What are the risks associated with it?

That’s a surgical question.  I would set up a separate meeting and ask these surgical questions of Dr. Hannan.  You know, I don’t want to speak for him because I don’t know what he’s planning on.  

Will they take down the BT shunt and/or close the ASD during the Glenn surgery? If not, when and how?  I believe Zach has several ASD’s.

Yes, they will take down the shunt.  No, they will not close the ASD for the reasons we spoke about.

When you do the takedown, is that an actual removal or they just tie it off?

Again, it depends on the surgeon.  Why don’t you ask him that?  Since they’re going to put the Glenn there, they generally remove it.   You can just clip it and leave it in place but usually they need the space so they take the whole thing out.  I would ask the surgeon, I don’t do it so I would ask him how he does it.  There just a bunch of different ways and they all do it differently.  They wouldn’t touch the ASD and we don’t want them to.  We’ll have to see how Zach and we can watch him on Echo.  Someday, if the right ventricle grows, we’ll take him to the cath lab, problem within preschool age and test occlude the ASD.  It doesn’t matter if he has one hole, 10 holes.  It changes the way we do it but we can do it.  Sometimes you want just a little hole.  If he has more than one hole, sometimes that’s advantageous.  You can close the big one, leave the little one.

Dr. Butler, Zach’s neurologist, wants MRI’s of Zach’s spine and head.  Can they be done with the surgery to avoid having to sedate Zach twice?

I doubt it.  But again, I would ask the surgeon. Maybe they can think of an innovative way to get him done on one anesthetic but I doubt it.

Should my husband or I plan to donate blood for the surgery?

If you like him to have your blood, then yes. You organize that through the surgical office.

Is there any advantage one way or the other?

Most people feel better about giving their own blood.  The blood is heavily screened.  Having said that, we had a recent case of hepatitis getting through.  Of course, it can even happen with your blood.  People don’t always know what they’ve been exposed to.   I think families tend to go that route.  Zach would in all likelihood need blood for the surgery.  Even if the surgeons tell you he won’t get blood.  What they’re thinking of is during the operation.  I can tell you when you’re in the ICU, he’ll get blood.

They’ll also need it to prime the heart/lung machine, right?  I remember when we met with Dr. Burke before Zach was born, he discouraged us from it. 

Most of the time I tell parents it very safe, you just have to know there is a risk.  There was a study several years ago that looked at those sorts of issues years later.  And it turns out that the rate of infection was higher with the families that donated blood.  The theory behind that was, people asked family members to donate and what family member is going to say “well I once had this”.  So it’s a double edged sword.  And really I don’t know if it makes a gigantic difference.

How long is the surgery expected to take?

You know the answer to that one right? Ask the surgeon.  Usually it’s a half day, you start in the morning and the surgeons are eating lunch.  So it’s not a whole day.

Will any foreign materials be used, goretex, etc?

No, but you might want to talk to the surgeon about it to make sure.

 How long will Zach be on the vent after surgery?

If everything goes well, we extubate most kids in the OR, that’s typical.  Now, is that every kid?  We had a Glenn last week that took 3 days on the ventilator.  We will explore the possibility of doing it at the time of the surgery. And if not then, usually it’s a short period of time after that, a day.

 Zach had a problem with collapsing lungs last time.  Should we expect to see that again?

No.

 This is where the tape ended so the rest is from my written notes.

 Is there a risk for arrhythmias after the Glenn?  If so, how are they treated?

There is a low risk. They are not operating on this part of the heart.  If so they treat it with medications.

Are there any common complications associated with this surgery?

Headaches for several days to a few weeks.  Effusions, a few weeks is possible.  It is treated with diuretics and diet.

What is the hospital’s policy on pain management after surgery?  If you use morphine, do you routinely give benydryl to counteract itching?

There is no set policy, it is given as needed. The nurses watch closely.

What is the expected length of stay in the CICU and/or step down?

Usually 4 – 7 days total with 1 to 2 of those in the step down.

How long can we stay with Zach before the surgery?  How soon can we be with him afterwards?

Check with Dr. Hannan

What changes should we expect to see in Zachary after recovery from the Glenn?  Increase in SAT’s, increased growth, increased energy?  How will the Glenn surgery improve his health or quality of life?

The shunt is palliative.  The SAT’s will be the same or lower.  He should have more energy.

What (if any) are our other options besides the Glenn procedure? What about replacing the tricuspid and/or pulmonary valves and giving the right vent a chance to grow? What about expanding the right vent with a patch to allow for growth? Are any of these (or other) options producing "better" long term outcomes? What are the risks/rewards of each choice?

There is no consensus among surgeons.  Different surgeons prefer different methods.  Our hospital doesn’t do scraping of the right ventricle. It is considered to dangerous and can damage the heart.  A right ventricle outflow patch may be considered.

After the Glen, what is the long term health care plan for Zach?

Watch his progress, probably re-cath in 1 year or more.

If Zach only requires the Glenn, I've read that this can leave the blood pressure "unbalanced" between the upper and lower body and that exercising upright isn't well tolerated in older kids/adults who haven't had the full fontan.

Closing the ASD should solve this.

What determines whether Zach will eventually need the fontan?

The ability of the right ventricle to handle the lower body circulation.

Is Zachary’s left ventricle and systemic blood flow considered normal?  Yes. Are there anticipated concerns with the left ventricle in the future? None expected.  Coronary arteries can complicate the problem.  Zach doesn’t have them and isn’t likely to get them.  They would usually be present by now if he were going to get them.

I know it’s impossible to predict Zach's future.  Based on what you know of children similar to Zachary, can you tell me if Zach will be able to go snow skiing, scuba diving, etc. In what ways will he be limited?

This will be determined by his right to left shunting.  We will have to wait to determine his endurance.  Commercial flying should by ok.  Scuba diving but no real depth.  No organized football, no hockey, no martial arts, in other words, nothing high impact.  We have lots of kids who live in Denver with heart conditions.  You can take him to higher altitudes and see how he does.   Symptoms would be getting exhausted.

What is the best current prediction for life expectancy for Zach and other kids with HRHS?

We predict normal or near normal life expectancy with very, very good quality of life.   Coronary arteries would be a concern.

Is there any reason Zach shouldn’t father children?  Can this defect be passed to his children?

He’s not likely to pass it on.  If there were other family members with heart defects then the chance increases but still only to 2 or 3%.

Can you recommend any reading materials geared toward “non-Doctors” so we may learn more about our son’s heart and future?

To Mend a Broken Heart.