Consultation with Dr. Robert Hannan

Consultation with Dr. Bob Hannan, Cardiovascular Surgeon

June 7, 2005

Red Font is Dr. Robert L Hannan, Cardiovascular Surgeon, Miami Children’s Hospital.

Blue font is Valerie/John Breder

Let me start by just saying this.  Of all the diseases we take care of, we are really in the steep part of the learning curve with this disease.  And I’m including here pulmonary atresia, intact ventricular septum, severe tricuspid stenosis, pulmonic stenosis.  And the reason we’re on the steep part of the learning curve is because people were really very ignorant about it as recently as 10 years ago.   I have to say our team at MCH, including Dr Mas, is as experienced dealing with this problem as anyone. . And what we’ve learned is that we still are learning a lot.  That we don’t know who, after their pulmonary valve gets ballooned, is going to a need the shunt.  We don’t know who’s going to need the Glenn.  We don’t know who’s going to need a 1 and ½ versus a fontan.  It’s still in a state of evolution.  And so us saying that we’re partly ignorant about it, is not just we’re ignorant, we know as much about this disease as anybody.  But the whole world is still learning about it.  And that makes it difficult for you.  And especially since there’s a lot, especially in this particular diagnosis, there’s a lot of bad information out there.   There are people who are recommending crazy stuff.  And with high risk, low reward type of stuff.   The track that Zachary is on, that we are on now, is a very safe track, it is the safest track, but also still carries a potential for high reward.  And from the last catheterization, from everything that I’ve heard about Zachary from Evan is that we have a very strong shot at what is called the 1 and ½.  The Glenn being his last operation with ASD closure later in the cath lab. We have a very strong shot for a healthy, happy boy who goes to college, does great, and doesn’t play competitive sports. And have a really good life.  There are some out there who would do crazy stuff and risk his life for what I would call a very minor possibility outcome. 

Do you want me to go down your list?

Yes, sure.

I did get it this morning and I looked it over this more.

I’m so sorry I didn’t get it to you with more time.  By the way, we are hugely grateful for the care that he’s in.  We love all the people here. It feels like family.

(personal discussion of Dr. Hannan’s daughter) I’m telling you this, because I can only imagine what it’s like to walk in your shoes with a beautiful boy, who looks normal. He’s got a scar but you know he’s going to have a continuing problem. 

And you can’t help, at least I can’t, you know I’m not a cardiologist, my way of dealing with it is to learn as much as I can and make sure I’m doing the absolute best thing.  Only it’s hard to know what that thing is because I’m not a cardiologist.

I will tell you this; I personally think that Evan is a very good cardiologist. And his judgment is very good, and Dr. Mas is a fantastic cardiologist and a personal friend and she is as good as they come. You are in exactly the right hands.

Dr. Zahn mentioned that Zach’s pulmonary valve is “very abnormal, stiff and rugged”.  Do you predict he will need replacement of the valve at some point? Dr. Zahn also mentioned that Zach has narrowing of the area below the valve (the subvalve).  How, and when, will that be treated?

I don’t think Zach will ever need a pulmonary valve replacement. The abnormal pulmonary valve, it’s just not part of the track he’s on at this time.

Even though he’s on the 1 and ½ track, you believe it’s sufficient to carry that ½ load?

You know as we discussed with the pulmonary valve replacement, some of this I’m going to say let’s not cross any bridges until we come to them because the number of branching patterns in the algorithm is way too many.  But the pulmonary valve and the subvalvular area and the size of the right ventricle is going to dictate whether he’s a candidate for a 1 and ½ repair or not.  I would say the fact that he’s born in August, so he’s almost 10 months, and he’s still doing very well with that small shunt is a good prognostic indicator.  I wouldn’t get too hung up on it.  You know abnormal appearing valves can be adequate and work ok, and normal appearing valves can wind up being degenerative.  So what he has now is not necessarily good or bad, it’s just a description, like we’re all different.

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

In a situation like this, in a set up like Zachary’s it is as close to a straight forward an operation as we get.  The success rate is extremely good; occasionally Evan needs to patch something up in the cath lab or may need to work on something. Its what we would call, never an easy operation for the mom and the dad and the baby but we would call it a straight forward operation.  We know exactly what we are going to do, we know exactly how to do it and we do it all the time.

Dr. Zahn mentioned in his letter to Dr. Fricker that there is some “tenting” of Zach’s right pulmonary artery at the shunt insertion.  Do you foresee any problem or treatment needed for this?

The tenting of the pulmonary artery is more common than not.  I wouldn’t even be concerned about that.

We have become comfortable with you as Zach’s surgeon.  Although we know that Dr. Burke is also highly qualified, we’d like to request that you perform the surgery.  We also would like to ensure that Dr. Laguerella attends as the anesthesiologist.

Once a surgeon operates on a patient, typically unless there’s a personal problem, Zachary’s my boy now.  So I’m going to be doing the rest of his operations.  It’s a relationship that’s hard to describe.  But after my own kids, Zachary’s my boy.   We can also include Dr. L.

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?

Unless he needs something else we would do the operation warm and beating.  We don’t anticipate needing to stop the heart. 

How will they take down the BT shunt during the Glenn surgery? Dr. Zahn mentioned removal vs. clipping it and leaving it in.

We usually take down the BT shunt by dividing it in half.  We leave the upper end in, the end connected to the artery.  It’s a little stump because we don’t want to take it off the artery.  But the end at the pulmonary artery gets completely removed.  And the little stump is never an issue. And it gets closed off.

Zach had several incidences of his lungs collapsing before and during surgery last time.  Is this something we should be concerned with during the upcoming surgery?

I wouldn’t worry about the lung collapse, that shouldn’t be an issue.

How long is the surgery expected to take?

Surgery takes half a day primarily because it’s a re-operation. 

Will any foreign materials be used, goretex, etc?

The only thing would be if Zachary needs augmentation or enlarging of his pulmonary artery, we may use some bovine pericardium.  It’s the shrink wrap of the heart, but from a cow instead of him because his pericardium is going to be all scarred.  It’s something we use all the time. That’s the only foreign material I would anticipate.

And that’s not something you have to worry about with rejection or anything?

Nope.

How long will Zach be on the vent after surgery?

Zach will be on the vent, we would hope, he may even come out of the operating room with his breathing tube out.  Or a minimal amount of time.

After Zach’s shunt surgery, you mentioned that the scar from the Glenn would be much “neater”.  Will he have the railroad tracks this time?  Will he have new chest tube scars?  Is there any way to minimize the scarring?

I’m going to excise all the scar that I can. So we should be able to get rid of the railroad tracks.

I know it seems shallow.

No, no.  And we’ll use the same chest tube holes.

Did I understand, because he’s doing this as a baby, skin grows, scars don’t? Right now the thing goes from his belly button to his sternum.

The thing about a scar, you know, it looks worse in the 6 week to 6 month range and then 3 years or 5 years later, the scar will continue to get smaller.  It remodels.

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

This biggest risk of arrhythmias and one we work extremely hard to avoid is that when we divide the superior vena cava, we’re quite close to the sinus node.  And sinus node dysfunction is a known complication of the Glenn operation.  Our incidence here is lower than at any institution I’ve ever worked at.  We work very hard.  In other words, we clamp it; we make sure he stays in sinus rhythm.  There is some incidence of late sinus node dysfunction.

Late, meaning after we go home?

Years later.

May I interrupt?  You said you split the superior vena cava?

We divide it in half so that the head end gets sewn to the pulmonary artery and the bottom end attached to the heart is over sewn. So all the head and arm blood goes into his pulmonary artery.

So you’re blocking off the bottom part?

Correct.

I see, I thought you picked it up and moved it.

No because then we’d have the heart end open.

Are there any common complications associated with this surgery?

On a re-operative situation, bleeding is always an issue but we never leave the operating room unhappy about that. If there are pulmonary artery problems, it’s typically something that can be treated in the cath lab.  I would think that Zachary’s operation is going to be very straight forward.  Again, not easy for the mom and dad, not easy for him, but very straightforward.

Dr. Zahn has said that he thought Zachary’s pulmonary branch arteries are good sized.

Yes, they’re very nice.

Also, I have read about the occurrence of headaches until he gets used to the new circulation.

I think with his hemodynamics, he’s going to be fine.  Babies cry a little bit, the worse their hemodynamics are.  Zachary compared to many of our patients, with his good left ventricle is in a better situation.  For instance, babies with hypoplastic left heart syndrome are in a much worse situation.  We don’t want anyone to have heart disease, but if they have heart disease, we want them to have their left ventricle. 

Oh, the one complication we see very occasionally after a Glenn is that sometimes children can have a lymphatic leak.  The excess fluid from your feet and legs and the fat that you eat, all goes into the lymphatic system.  It comes up and drains into your left IJ (??).  Sometimes increasing the pressure by asking that blood to go through the lungs can cause a lymph leak.  Its called a chylothorax and it can build up in one side of the chest or the other.  After the bi-directional Glenn, its extremely rare, it’s a 1 percent complication.  It’s the one that I’d warn you about because it can be vexing.  Almost always its because we need to do something else like do a cardiac catheterization. I can’t even remember that last time we had a baby in the hospital for a prolonged chylothorax who had a good left ventricle and good pulmonary arteries. But it is the complication that would delay him the most.

What do you do for it?

Drain it and make sure the hemodynamics are okay which is where the cardiac catheterization comes in.  Usually they seal up on their own.   Sometimes, very occasionally they need to go on a fat free diet for a couple of weeks.  But really with the good left ventricle, the low pressures in the LV, the good arteries are good against that.  And I only mention that and the sinus node dysfunction because even though they’re very rare, you want to hear about it.  That’s why you’re here.

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?

No, we can’t do it.  It would be too much. 

In that case would you recommend doing the MRI’s before or after the Glenn surgery?

I would do it after the Glenn.

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

The nurses are experts on pain management.  We have some new techniques with a catheter that we actually put local anesthetic in.  We’re actually doing a study on it right now.  Depending on when he’s operated on, he may be in the study.  The pain from this operation is really the headaches that some kids get and I don’t think he’s going to have a problem with that.  They don’t routinely give benydryl but again, the doctors and the nurses in the ICU are expert at pain management and they’ll be able to tell if he’s itchy.  They are really experts at it.

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

7 – 10 days.  It really depends on how he eats afterwards, getting the chest tubes out.

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

You will be able to go with him right to the room and be with him virtually right afterwards.  You’ll be able to stay with him until he’s sedated.  You folks were up in the ICU long enough before that you know who really runs the ICU and its not the doctors.  The nurses will get you in there as soon as possible after.   Typically, you’ll come in in the morning to pre-op holding.  You be able to kiss him goodbye in pre-op holding.  He’ll get versed orally. He’ll be loose as a goose.

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?

I really don’t anticipate his saturation to increase too much.  We would expect his saturation in the 80 percent range.  And that depends on his pulmonary blood flow, the amount going through the valve.

He’s running about mid 70’s now.

That would be an indication to jump ahead to the chase.  After you see Dr. Fricker and Evan gets Dr. Fricker’s letter, we’re going to re-conference Zachary and at that point we would probably proceed with a date, especially since you’re telling me his SAT’s are dropping.   He’s in a different circumstance than a baby whose entire pulmonary blood flow goes through the shunt.  Because if they have a shunt problem, they’re going to die.  So those babies get operated on between 3 and 6 months of life.  Zachary, we can wait, because he may get blue, you know, we may need to do his Glenn one evening.  I doubt that even.  Because he’s not going to die because he’s still got plenty of pulmonary blood flow.   So we’re giving his right ventricle the biggest chance to grow.  But again, Evan and I talked this morning and after you see Dr. Fricker, which is the 13^th, Dr. Fricker will send Evan a letter which we’ll have at conference.  Evan, Dr. Mas, myself, Dr. Burke, Dave in Orlando, our Orlando surgeon who just joined us from CHOP, we’ll discuss it.  But I’m certain we’ll say, let’s call the Breders and give them an OR date.   I’ll be out of town over July 4^th.  Its possible we can do the surgery the last week in June but I wouldn’t want to do it and then go out of town.  Again, I wouldn’t want to do the surgery and then go out of town. 

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?

If you look on the internet and call enough cardiologists and cardiac surgeons, you can find a world’s supply of advice.  For my money, the Glenn operation for him, it’s the safest, it’s the best and certainly we’ll discuss all the options in conference with Dr. Zahn, Dr. Mas and the other doctors. But I think all the other options are, again, we’re on the steep part of the learning curve.   And what we’re learning is he’s going to have a great result with the Glenn and even if he goes onto a fontan, he’ll have a great result. I personally, in my crystal ball, I suspect in 2 years, Dr. Zahn will be closing his ASD in the cath lab.  That’s my suspicion. 

Dr. Zahn mentioned they may consider a right ventricle outflow patch?  How does this work?  What is it intended to accomplish?

If his right ventricle was big enough and we thought it was the pulmonary valve and the subvalvar region, and that was the right limiting step for the one and a half, but that’s way down the road and there are a bunch of options at that point.

Okay, so we do the Glenn and we wait and see what his right ventricle does?  Isn’t his ventricle less likely to grow after the Glenn because its not carrying the full load anymore. 

That’s one reason to leave the shunt in as long as we have.  Why ventricles grow and why they don’t grow, again that’s universal, global ?-based ignorance.  No one can predict.  I’ve been in the cath lab when they balloon the pulmonary valve and I’ve thought to myself I’m going to put a shunt in that baby, 100 percent and they totally go home and they come back and get their ASD closed.   We’ve been surprised by that many times.

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.

The blood in the superior vena cava is under higher pressure than the inferior vena cava  blood because it has to go through the lungs and there’s a thing called pulmonary vascular resistance or transpulmonary gradient.  So the blood in the SVC has a pressure of 9, the transpulmonary gradient is 6 and there’s a pressure in the left atrium of 3.   So the lower body has a pressure of 3 and the upper body has a pressure of 9.  When you exercise that can increase. And it depends, everybody’s different. I know people, young athletes, with very similar setups who couldn’t swim because they needed to be upright to get blood through their SVC and when they swam, their heads ballooned up.  When they rode on a stationary bike they were fine.  I think he’s going to be fine. He’s going to exercise.  There’s a little boy I know with hypoplastic left heart syndrome and I very complicated Glenn and he kicks the ‘you know what’ out of his older brothers all the time.  His mom says he’s the most active of them all. And he’s a little bit blue, and he’ll get his fontan one of these days but it doesn’t limit his exercise at all.  He’s a beautiful boy, we get a Christmas card from his family every year.

So basically you’re saying it can happen but you don’t think it will?

I wouldn’t fret about it.  I think he’ll be running around, fishing with his dad and roughhousing with his mom, just great.

We’re hoping that if he doesn’t get it before this surgery, he’ll learn how to crawl after the surgery.

My daughter never crawled.  She scooted around then she walked. Some kids never crawl.

What determines whether Zach will eventually need the fontan?

As some point in the future, he’ll go into the catheterization lab, Dr. Zahn will occlude his ASD and measure all his pressures with a balloon. It the pressures and the saturations look good, he may close it permanently.  If the pressures and saturations don’t look good, then we’ll go through the whole conference, re-evaluation process again.

But at some point, he’ll need to have the ASD closed?  You can’t go on forever with it open?

No.  Because he’s got flow through the pulmonary artery, his saturation, again we don’t have a crystal ball.  What if the saturation was 94 percent after the Glenn?  We might say let’s leave that alone.  You know all the numbers are good.  We’re not going to make him any better with the fontan, he’s sort of got a natural one and a half and we might leave the ASD open for a prolonged time then.  Again that’s another case where you open the door and there’s so many choices it gets to be information overload.

Is Zachary’s left ventricle and systemic blood flow considered normal?  Are there anticipated concerns with the left ventricle in the future?

I don’t have any concerns about his left ventricle in the future based on the information that I have. 

I think we’re done.  I don’t really expect to hear anything different from Dr. Fricker.  As a mom, I hope to hear him say “give this pill to Zach and he’ll be fine”

I totally encourage you to get the second opinion.  Again, as a father, my daughter gets a cold, throws up a little bit and I freak out.  Only because of my experience as a surgeon can I imagine what its like walking in your shoes.  Your tremendously loved son has heart disease and it changes your life. I will say that I think here, we take care of this spectrum of diseases as well as anywhere in the world.  Our surgical results for the Glenn, I’m not going to tell you there’s 100% survival, but there is.  We’ve never lost a baby in a Glenn with this set up.  There is an occasional baby with the Glenn who dies but its with HLHS.  There can be complications.  There are some extraordinarily rare complications after Glenns that you’ll find on the internet.  And any time you use the heart lung machine you can have a stroke, or brain damage or bleeding or any of those issues. But, again, I would never tell this operation is easy but its very straight forward.  We know exactly what to do; we’ve done it many, many times before with minimal complications.  And the commitment is always the same, to take care of Zach like he was my little boy or my little girl and that’s what happens.