A reflection on congenital heart disease care, and where its headed – Michigan Medicine

Then lucky No. 7 came along. The baby got off the OR table and there was no ICU bed ready because the ICU nurses figured why bother. Sure enough, we eventually got the bed! The baby was fairly sick and the first thing he taught me was that too big of a shunt was the worst thing in the world. We originally thought the bigger the shunt, the pinker the baby, but it was quite the opposite.

This baby survived and I ended up transitioning to Michigan. Around 8 to 10 months old, lucky No. 7 came up to Ann Arbor for the second stage of surgery. We didnt know what to do at first because we didnt have any previous survivors get that far. So, we did a Fontan operation, the old-style Fontan, which was absolutely not the way to do it, but we didnt know.

The child eventually died after about a month, and I was ready to quit. That was it for me. But I got a letter from that childs mom and dad thanking me for the nearly one year they had with their child and told me to keep trying, to not give up. It was very emotional, and I never forgot that.

I finally came around and thought, okay, weve got to keep trying.

As a team, we started working on more cases but, at the time, there werent a lot of referrals because people didnt think it was the way to go. Slowly, we started getting better results. Then we had to learn what to do when these patients came back six to eight months after the Norwood operation.

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I had started using smaller shunts, but the babies were turning blue as they got older. Do we do another shunt? Do we do another operation rather than repeating the history? We struggled with that for a while until we finally stumbled upon the so-called hemi-Fontan or bidirectional Glenn operation what now is the second stage of the three-stage operation process.

It was in the early to mid-90s that we wrote a paper where we had over 80% survival for stage one which was incredible at the time, given the fact that no one was getting more than half of that. All of a sudden, referrals picked up. One year we did 66 Norwood operations. It was a dramatic difference, but it was step-by-step. I dont think there were any great come-to-Jesus moments, except lucky No. 7 who taught me about the shunt. It just changed the outcome completely.

Now Im getting graduation letters and wedding invitations from young adults with this condition who are now living well into their 30s. Its overwhelming when you think about it. [University of] Michigan [Health] is now up to well over a thousand Norwood operations and even more Fontan operations, the second largest volume in the world.

Bove: I started with the Glenn. In fact, one of my long-term survivors had a classic Glenn at the second stage because we couldnt figure out what to do. One of the problems I found with the Glenn was that, although its technically easier to do, it doesnt necessarily fix the branch PAs (pulmonary arteries) which very often need to be enlarged.

I didnt start the hemi-Fontan [operation], but I remember reading about it and thinking that it was really a good idea for a number of reasons. It allows you to fix the branch PAs making them nice and big, getting rid of the potential risk associated with it, then to be able to complete the Fontan [operation] in a much easier fashion because you didnt have to dissect all that out again.

Since Ive done that, weve done a number of computer flow studies looking at how the hemi-Fontan, and then subsequent lateral tunnel Fontan, distribute blood flow to each lung and minimize energy loss. Because you want to be as close to 50/50 as you can get. What our computer studies showed was that the hemi-Fontan and the lateral tunnel were by far better than a Glenn and an extra-cardiac (heart) conduit.

Ive never changed from that because Ive seen way too many now adults with lateral tunnel Fontans and their imaging looks great. It looks like its grown with them, and they dont have any or very minimal foreign material.

A number of programs have switched to [the Glenn] because they think the hemi-Fontan is too complicated and they dont like to do it. I dont blame them but after having done literally thousands, to me, its routine as it is to our other Mott heart surgeons now and we like it. Its hard to think to do it a different way when the long-term results have been outstanding.

Bove: We transitioned so much from just trying to get to survivorship into making sure now that we have a quality result and that means neurodevelopmentally and in many other ways as well. Thats probably where the next years will bring us.

As we refine our techniques, we get better and better to make the methods we use for bypass and other surgical repairs lead our patients to be healthy in every way by that, I mean mentally as well as physically.

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Were also going to be looking at the era I hope, anyway of stem cells. I think thats potentially the next biggest addition and transition we may have. Where we can grow valves, grow blood vessels, maybe even grow heart chambers, grow conduction (pacemaker of the heart) tissue theres almost no limit as you think about what potentially that could bring us. Its a difficult thing and its not right around the corner, but I think its going to be there.

I think probably the third big thing is going to be devices. Weve already gotten to the point where we have these devices that are much smaller, much more patient friendly and I think thats only going to get better. We will literally be able to support hearts with these miniature devices that will allow our patients to lead very good, useful, normal lives. Thats a reality thats happening now and I think its only going to get better.

Bove: Well, thank you. Theres no way I could ever tell you the respect and admiration I have for the parents. The parents are the heroes in all of this. I never took it lightly that parents were putting their baby in my arms to do something that is potentially lethal. I never took that lightly. The trust that they had is overwhelming it still is.

A lot of people have asked what the greatest enjoyment is Ive had, and I think the greatest enjoyment Ive had and it never got boring was coming up and talking to the parents after surgery to let them know that everything was fine and their child was going to do well. Seeing the look on parents faces and the relief and joy they had you cant beat that. You dont operate on a baby. You operate on a family. I cant ever tell you what that feeling is like. It is truly unbelievable.

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A reflection on congenital heart disease care, and where its headed - Michigan Medicine