Hani Najm, MD, Chair of Pediatric Cardiothoracic Surgery, Head of Congenital Pediatric, Heart Surgeon, Cleveland Clinic Children’s, talks about using a 3D printed model of a patient’s heart to help prepare for surgery.
Interview conducted by Ivanhoe Broadcast News in October 2017.
Ethan Bradley was born with heterotaxy syndrome; can you talk about what that syndrome is?
Dr. Najm: It is not a very common congenital abnormally, there are types of heterotaxy syndrome; it is a combination of different abnormalities that are causing either venous returns from the right side or the left side of the body or the heart being abnormal so drainage into different parts of the wrong chamber. That usually results in the lungs then the lung blood return comes back into the wrong atrium, or for that matter, to even a different part of the body; sometimes below the diaphragm. It does not connect to the heart; and sometimes also the venous returns from the body do not come back into the appropriate chamber. In addition to that they would have some defects inside the heart, between the chambers of the heart the atriums and the ventricles, as well as they could also instead of having four valves they would have three valves, so one common valve that sits for both ventricles. So there is a combination of complexity that ranges, that is why it is called a syndrome, because it is a range of complexity sometimes some of them are simpler and some of them are more complex based on how many of these different anomalies are coming into the heart.
And then in Ethan’s case, what was his?
Dr. Najm: So Ethan was born with heterotaxy and he does not have a certain valve, so there is no way that the blood goes into that circulation from his heart. He’s had different arteries coming in from different parts of the aorta into the lungs, he has common AV canal, which is a common valve rather than two valves; usually we have a mitral, tricuspid, it is two valves now it is joint with one valve. He has ominous return of his pulmonary veins, which means that his pulmonary veins do not drain into his heart and he basically has both his pulmonary veins and his pulmonary arteries are not coming into their appropriate direction; so he needed a staged repair. He has is undergone initially a repair for these veins to connect them back to the heart, then he needed these pulmonary arteries which did not exist before that they put them back together so they can actually formulate a pulmonary circulation, a pulmonary artery then that was connected to the right ventricle, he has a right ventricle, which is called a double outlet right ventricle, which means both chambers come out and the aorta comes out of the right ventricle so with the combination of repairs that he had through his first nine years of life combined into a circulation, which brings blue blood into his systemic circulation or what is pumped into the body. That has created him to become blue and that discoloration or blueness continue to increase over the last few years to a level that made Ethan in a way unable to do majority of his daily activity down to even walking let alone being able to run or exercise, or do something more than that, so he was not able to do his daily activity. And I remember very clearly, Dr. K came into my office before I knew even Ethan case and he came and said I have a case that we are stuck with Ethan and unfortunately I see him month after month and I am following him and he is deteriorating in front of my eyes, and we need to do something for him because he is not going to survive for a long time in this condition; he is deteriorating in front of my eyes. And then he presented the case and the way we do as usual we presented in a group meeting and we entertain all the possible scenario as how we can help Ethan, otherwise he is not even a candidate for a heart transplant; so what is the thing that we can do for him. One of the areas that we are good at is imaging of the vessels and the heart from outside, we have the echocardiogram, which images the chambers of the heart but it is what we call a 2D echo even what is called a 3D echo does not give you the entire image of the heart as in gives you a section of the heart in 3D, but it does not give you the relations the different parts of the heart at the same time. So he underwent a MRI, the CT scan, and the echo; multiple of them and at one stage we had entertained that I was thinking that there could be something that we could do, but the current imaging techniques are not able to give me the ability to decide if whether this is going to be done or I could do it in the operating room; or not. So we needed something which will give us more direct imaging of what is inside the heart and that is where 3D printing comes by.
What is 3D printing?
Dr. Najm: 3D printing is a process, basically, we bring in images that we have created with the other imaging techniques the other ones like the cat scan, or MRI and we combine them set into a computer and that computer would print out a live size exact replica of the heart 3D; as in I can hold it in the same way as the heart came out of the chest of the patient; now I can hold it before I even open the chest. When I looked at the 3D structure of Ethan and I opened it, and I looked on the inside I realized that I could bring in and reroute some of his chambers and reconnect on the outside the appropriate chambers in a way that would divert the blue blood away from his systemic circulation or to his body. So we discussed this further as it would be a complex surgery, high risk, he is weak, but we thought we needed to do something good for him too, to change his life because the way he was going, he was going into a very apparent and clear deterioration as clinical status. So we discussed with his mother and he is a very smart boy also he actually wanted anything done for him to get improved. Based on the images we had we went in the operating room and one of the most innovative way of diverting this, he got what is called a common HM and a common AV valve and a very large ventricle defect, typically, when we want to separate the circulations we separate both. We divide the atrium and we divide the ventricles and we separate the two valves, with the common valve into the two; that is a standard type of surgery that is done by the thousands.
And what about with Ethan?
Dr. Najm: The issue is with Ethan is that he had a high pulmonary artery pressure that would not allow us to do all of his procedure, therefore, we needed to improvise and only separate where the blue blood and pink blood goes to the wrong chamber and reroute them through the atrium into the appropriate chamber then switch whatever comes out of the chambers. It is a little complex in a way to explain it in lay words, but the gist of the operation is rerouting on the inside to allow the blue blood to go to the lungs; that is where it is suppose to go and the pink blood to come out of the heart to the body into the aorta and that would make him pink and would allow it to work so he could go about his daily living. And that was executed, so we went into the operating room with a saturation, oxygen saturation, anywhere between fifty and sixty percent and we came out of the operating room with a saturation of ninety five percent; and that has through the months even after we repaired him have stayed also. We knew in the operating room, where usually things are perfect, but so were around ninety five or ninety six percent like normal people. He is in the pink range that would allow him to do substantially more and live a fairly active life, but he has one thing to claim from previous years that he has not been able to play and do what he used to do with his peers.
And is this surgery, have you done anything like this before, has it been done on someone like Ethan?
Dr. Najm: I have not done. I am sure that there are surgeons who have; I can’t claim that no other surgeons done something like that. There are plenty of very smart, good surgeons around the world who has done some sort of procedures that are innovative. What is special about Ethan is the dramatic change from literally going into the operating room with a saturation of 50 in the morning coming out with a saturation of 95 after being nine years in that situation by simply using first innovative thinking, innovative imaging and innovative surgical techniques. All of these together you always want to help and a child with such a very complex heart, that is rare. I would say that it is likely not done by many centers if any, but I would not say that no one else has done it like that.
It is definitely a novel approach. What does the future hold with Ethan I am sure he will need somewhat lifelong care but with this surgery are we hoping that it kind of takes him years?
Dr. Najm: I would believe that the way he is going and his clinical status how it is progressing and so on this is going to carry on for years. Now, we did implant a conduit, that conduit is variable with time it is going to became narrow and is going to be replaced, or an intervention needs to done. We have left a hole inside the heart purposely because we knew that he would not tolerate closing it at this stage. But that hole is needs to be addressed, it need to be closed in future. All these things are going to have to be evaluated and assisted in a way that would basically put him into an area that would gave him longtime survival, but as it is with all congenital heart disease patients they have to be followed for the rest of their lives and that is why most of what we do is we call it palliative surgery, because we are except for very few leads we do with congenital heart, it is palliative they will come back and then we need to continue to follow them for the rest of their lives. That is why it is a growing population, and that is why we have some specialty code. The kids we operate on them after 10, 15, 20, 30, 40 years they will come back and their 50’s and 60’s with new problems that we have not seen before because now we are following and they are living up to 50’s or 60’s, so they are coming back with different set of problems that we have not seen before. This is a very fascinating specialty and it is a growing specialty the adult congenital heart surgery and as we stand now we have more adults with heart disease, with congenital heart disease, then kids with congenital heart disease; if we take the cutoff ages being 18. So, once you cross the 18 year old labeled as an adult the patient is labeled as an adult with a congenital heart disease they just continue to grow and they become into that bigger population that needs to be followed up.
For now Ethan is healthy, he is able to be active, he is able to be a normal kid and he was never able to do that the whole nine years of his life?
Dr. Najm: Yeah he has never been able to do that for sure for the first nine years of his life and for the last year before this surgery he was disabled, he came into my clinic with a wheelchair. So he could not even do a long distance walk, let alone getting up the stairs or doing something that kids want to do. So now his life is close to normal but it is not like kids with normal hearts of course, but he is close to normal.
Is there anything else about his story or his case that general population should know about?
Dr. Najm: First of all general comments is never give up; always look for what is best for your child or for your relative. There are always ways that medicine has evolved over the years. Things we do now we did not even know that we could do five years ago, let alone 10 years ago. So even if there are people who are being labeled inoperable it is very important not to take that; I know that many times apart from Ethan we have been presented with some cases that were labeled two years ago as inoperable and they just left. I think families should seek advice from multiple professionals because this label as being inoperable is not permanent and it is not true all the time, because we all have new techniques, new innovations, new technology that we use like this 3D printing. We have new devices that may address these “inoperable” diseases even if I look at my surgical experience in the last 20 years, since I was in training and I became a surgeon; many things that just in my period we never used to able to touch or operate on, now we do it routinely, so that is what I am saying to families; do not take things for face value keep searching, look for the right people, for the right center, for the right surgeon. What is called inoperable or we cannot do anything about, someone else may be able to because there are always ways we could actually help a child, or an adult with what is called an inoperable congenital heart disease.
END OF INTERVIEW
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