Transcription:
Building a Lymphatic Occlusion Intervention Program

Prakash Chandran (Host): The lymphatic evaluation and intervention program at children's health builds on the teamwork established in their hybrid catheterization lab. This lab was established in 2017 and was the first of its kind in Texas The team does teleconsultation with physicians across the U S to help evaluate whether lymphatic intervention is appropriate for their Fontanne patients.

You are listening to pediatric insights, advances, and innovations with children. We are here today with Dr. Seron ready? FSC AI. He's a cardiologist at children's health and associate professor at UT Southwestern, the director of the cardiac catheterization program and the medical director of the Fontana single ventricle program.

I'm pre-cost Gendron and Dr. Reddy. Thank you so much for being here today. It's really great to have you on. I wanted to start by asking, how did you determine the need for a lymphatic evaluation and intervention program in the first place?

Suren Reddy, MD, FSCAI (Guest): Well Prakash. Thank you. It's a pleasure to be here. Determining that there's a need for the lymphatic program here in Texas was actually the most easy part. I'm a Pediatric Cardiologist, as you said. I also take care of adults with congenital heart disease and my special interest and expertise is in single ventricle physiology. And lymphatic abnormalities are one of the rare, but very serious complications of single ventricle patients. Lymphatic evaluation and interventions for patients with congenital heart disease has taken off over the past decade or so and that's mainly thanks to the pioneering work done by our colleagues at Children's Hospital of Philadelphia.

During my day-to-day practice, myself and many of my cardiology colleagues, see many patients with varying degree of lymphatic abnormality. And some of them just need medical therapies and they do well, but most do not do well. In the past, prior to these novel therapeutic options being available, they didn't really have major management protocols.

We just had to list them for a transplant. Some patients, who were lucky enough to receive a successful heart transplant did well, but we also knew and continue to know that these patients, after receiving a heart transplant, patients who have had lymphatic problems, continue to have not so great outcomes after heart transplant.

And moreover, the heart transplant itself is not a panacea as you know. There are many other problems that arises following heart transplant. So, our goal or my goal was to keep the patient's heart or the engine, if you will, as is, so that we can continue to, take care of their heart, especially those patients with single ventricle disease to keep their hearts for as long as we can.

And if we are able to provide interventions, via the Lymphatic Intervention Program, that was the best thing that could happen for such high risk patients. There's a tremendous need for us to identify various pathways, management pathways for lymphatic complications, patients with lymphatic complications. And in the past, as I alluded to, there are not many options and these patients would eventually end up on the heart transplant list. And there are not many organs available in the United States for every single Fontan patient to receive a heart transplant.

So in 2017, May, 2017, I remember that really well because it was one of my favorite patients, who suffered a major lymphatic complication in the form of recalcitrant chylous effusions and plastic bronchitis, following his third stage, which we call as a Fontan palliation surgery.

At that time, he was staring down the long list of transplant evaluation, and being listed for transplant. And after discussing with my colleagues, Dr. Yoav Dori at CHOP, I referred my patient. And I also flew with this patient to CHOP so that I could observe the procedure that he was undergoing and come up with ways to see if we can bring it home here, to Dallas.

So, after my patient, I went a very successful lymphatic intervention by our colleagues at CHOP, I was convinced that this is something that we should be thinking about, or at least starting the program here in Dallas area. And the other major impetus for me was to see that there was a long waiting list for patients from multiple states, from across the country to get their treatment at this one institution, which has done the pioneering work.

In addition, doing that trip with the patient also made me realize how extremely hard and inconvenient it is for most patients and families to do this long distance travel, especially with the child who's already suffering from major complications. So, after I came back, I checked to see if there was any center here within Texas or in the southern part of the country who do such lymphatic interventions or are on the cusp of getting better at it and I found none. And that's when I decided to start the Lymphatic Evaluation and Intervention Program here at Children's Health and UT Southwestern so that we can actually offer this highly innovative care for the most complex of our complex patients here in Dallas closer to their homes.

Host: Thank you for that, Dr. Reddy. I think we have a solid understanding of why you started the program, but maybe tell us a little bit more about the types of patients that benefit from this program and why.

Dr. Reddy: Absolutely. So, who benefits from the lymphatic program? Patients who have cardiac disease and non-cardiac patients who have generalized lymphatic abnormalities, both benefit from the lymphatic program that we have established here. To be honest, the main reason I got involved and started this program was to help congenital heart disease patients who I take care of on a daily basis.

If I may share some historical facts here, lymphatic diagnostic evaluations and interventions are not new. They were actually being done and were traditionally being performed by our interventional radiology colleagues for many years now. But their expertise, if you will, is mostly with non-cardiac patients or patients who have a normal four chamber heart.

We do have an amazing interventional radiologist here, Dr. Sheena Pimpalwar. She's an interventional radiologist who has done and continues to do multitude of lymphatic evaluations and interventions in other parts of the body, such as lymphatic cysts or malformations that are seen in chest or abdomen or like in the upper and the lower limbs. But the lymphatic complications that I encounter and I see on a daily basis are in patients with congenital heart disease. And especially those with single ventricle or also called as a Fontan palliation patients. And this subset of patients are a completely different ballgame.

And as their disease processes are very different. Patients who are born with hypoplastic, right, or left heart syndrome, commonly referred to as a single ventricle patients, they undergo a series of three open heart surgeries. And that's done usually from day zero of life to the first three to four years of life.

And eventually we come up with a system within the body where we separate out the red and the blue blood leading to a non pulsatile Fontan palliation. So, this three-stage procedure leads to a permanent increase in the systemic venous pressures, which in turn puts a lot of stress on all the other organs, including the lymphatic system.

When go through medical school, the least favorite topic for many of us, including myself at that time, not anymore, was the lymphatic chapter because we didn't really know much about reason as to why the lymphatic system existed. We only knew about two major systems, the arterial and venous systems, but the lymphatic system was a forgotten system.

We have figured out that it actually plays a very important role in the body. Even though it's called, it's a drainage system on the body, it is critical for the normal functioning of our internal milieu, if you will. This lymphatic fluid goes through a multitude of tiny little capillary like a network or a meshwork, and eventually it drains into the venous system.

Well normal venous pressures in a patient who has a normal heart, but normal four chambers are low. They're usually less than 10, but the moment we do a three-stage repair in patients with single ventricle, the Fontan patients, their pressures are high. Their pressures are usually doubled than the usual normal, less than 10 millimeters of mercury that we are supposed to see in patients without heart disease or normal four chamber patients.

So, what did this do? Meaning this fontan circulation then put in a lot of stress on the lymphatic system because now the lymphatic system has to drain against the higher pressure. And the entire lymphatic system is also on overdrive because the liver produces a lot of lymphatic fluid and that is there permanently for the rest of their lifetime.

So, congenital heart disease patients who will benefit from the lymphatic program or those who develop lymphatic complications and primarily in the form of chylous effusions, plastic bronchitis, or what we call as PLE, which is protein losing enteropathy, can essentially occurany time immediately after surgery, during the interstage period or even many years after Fontan palliation.

So, to summarize, patients who can benefit from this program would be both cardiac and non-cardiac patients. And we have the teams available here at Children's and UT to take care of all the patients with lymphatic abnormalities.

Host: So expanding on that a little bit more. Talk to us about the makeup of these multidisciplinary teams that came together to make this program possible.

Dr. Reddy: Absolutely. It's not a one man show. It is truly a multi-disciplinary effort. In general, lymphatic patients are considered as very complex patients, very high risk patients, so many service lines within the Heart Center, be it ICU or Cardiology Floor, Echo, MR imaging, every team member comes into play to take care of these complex patients when these patients are transferred from other institutions within Texas or adjacent states. But the three critical teams that are very essential for starting this program, are the Cardiac Cath Team, the Cardiac MRI Team and our Interventional Radiology Team. And I'm truly blessed to have an amazing set of cath lab staff and then a phenomenal team of cardiac anesthesia doctors that do lymphatic patients are safe while I'm doing these long procedures because typically they are a day long procedures. I consider lymphatic program, as you alluded to early on, as an offshoot of our other innovative program, which is the ICMR or radiation free MRI guided cardiac catheterization program that Dr. Hussein who's our MRI lead and I, as a cath lead, started this back in 2016, 2017 after. The first was it, my first was at to Children's Hospital of Philadelphia. I went back again and this time with Dr. Hussein so that he can actually see firsthand as to how the MR diagnostic portion of the lymphatic system is done. And after we came back, I laid the foundation with his help and the entire heart center team's help to lay the foundation for the Lymphatic Occlusion Program here. And around this time our stars really aligned because Sheena, Dr. Sheena Pimpalwar who's an excellent Interventional Radiologist joined UT Southwestern and became the Director of the Pediatric Interventional Radiology Team here at Children's Health.

She is an amazing doctor, lots of expertise doing lymphatic evaluations in non-functional heart disease patients. And it was very easy to partner with her to build a full fledged lymphatic program now that caters to the needs of all patients, both cardiac and non-cardiac patients.

Host: That definitely sounds amazing how the stars aligned and just brought together this multi-disciplinary team to make this program possible. I want to shift to what exactly takes place during a lymphatic occlusion procedure. Could you break that down us?

Dr. Reddy: Absolutely. The procedure, I consider them and our team knows that these are very long procedures. We start the day early in the morning. Patients typically are brought into what we call a zone three, which is right outside the MRI suite, where patients are intubated and placed under anesthesia by our excellent cardiac anesthesia doctors.

Then Dr. Pimpalwar from radiology and I use an ultrasound machine to basically place tiny little needles just underneath the skin in these small channels called lymphatic channels within the lymph nodes in the groin area. Previously, we used to place needles all the way down by the toes in between the toes.

It takes a long time. And we have come up with better ways of imaging, which is the diagnostic portion of the lymphatic procedure. So, we place the needles in the groin area where lymph nodes and lymphatic channels are, and the patient is then carefully transferred so the needles do not fall out, to zone four, which is where Dr. Pimpalwar and I inject dilute gadolinium is the MRI based contrast agent. And as we inject, the MRI team with Dr. Hussein and his team, they do these specialized scans that lights up and delineates the entire lymphatic channel, including the main lymphatic duct called the thoracic duct.

It takes about half an hour, or one hour for the entire process. After that we'll review the MR images. We decide and evaluate if the thoracic duct is open or not. If there are any abnormal connections to the chest or the abdomen and come up with a game plan, if you will, and then transfer the patient to the cath lab for the next step, which is the interventional aspect of the procedure.

Sometimes these patients do have lot of breathing difficulty and we might have to stage them, meaning the diagnostic portion on day one and clean up their airways, using bronchoscopy with our lung doctors, pulmonary doctors cleaning up their airways, and then coming back the next day or a few days later to do the interventional part.

But most of the times, the diagnostic and interventional part is done on the same day. So, the moment the patient's transferred for the second half of the procedure to the cath lab under the same anesthesia, we do our basic diagnostic evaluation to make sure that there is nothing else wrong inside the patients.

In this case, let's say a Fontan physiology, the Fontan anatomy is okay. There is no other problems. And if there are areas that have a direct right to left shunt, meaning a blood clot or glue that we will use during lymphatic interventions has a direct access from where we would inject and going to the heart and the brain, then we have to temporarily or permanently occlude that channel during the lymphatic intervention. So, once that step is done, then we have essentially two ways that we can enter the thoracic duct. It's like finding a needle in the haystack, if you will. That is the diagnostic portion. MRI would have already told us where to look and once we identify the needle, if you will, the thoracic duct, we need to thread it. We need to put a wire and then go down into the thoracic duct and identify areas of abnormal connections so that we can selectively take care of them. And sometimes we take care of the entire thoracic duct, meaning occlude it using devices, coils, glue, et cetera.

And when we use glue, it's considered a high risk procedure. We want to make sure that the glue doesn't go into the lungs or to the brain cause those are the complications that needs to be avoided. We take a lot of precautions to prevent that. So, that's in a nutshell for the lymphatic procedures and after the procedure, patients typically recover in the ICU for a day or two and then go to the floor and the recovery is much quicker and faster post lymphatic intervention for chylous effusions or patients after surgery, who drain for weeks, sometimes months. You see this drastic reduction and complete resolution of their chylous effusions, or the plastic bronchitis, which is a life-threatening complication. That's gone in a few days. And patients recover really fast and are discharged home in no time.

Host: Yeah, it's amazing just how much faster the recovery time can be. I was wondering if you can share some of the outcomes from the procedures that you've done?

Dr. Reddy: Within a short period of time, we were able to successfully intervene on more than 10 patients so far with lymphatic complications who either presented to us with chylous effusions or plastic bronchitis. We are at the present time building the team that is needed to offer diagnostic and therapeutic options for patients with PLE, which is protein losing enteropathy.

So, at this time we offer services for patients presenting with chylous effusions and plastic bronchitis, and all the patients who have been referred to us so far from locally within DFW area, from adjacent institutions and also multiple states, Oklahoma Tennessee, et cetera; patients have come here gotten their plastic bronchitis or chylous diffusion treatment successfully and have returned back to their homes and are currently being followed up with their primary cardiologists.

This is a testimony to the multidisciplinary team that we have established here that within a short period of time, we've had patients referred from multiple other hospitals, not just within Texas, but also from adjacent states. And I actually want to share a fun fact here because the system or the program that I was building was for a lymphatic occlusion procedures for lymphatic occlusion program.

But the first patient ended up being a patient who actually needed the lymphatic system to be dilated. This was a patient who was referred to a different institution before we started our lymphatic program here had a lymphatic intervention done when one of the glue materials had done migrated up to a wrong place and occluded the thoracic duct, if you will. And this patient became very high risk for a transplant after that intervention because needed a lot of support and stayed in the hospital. And while I was doing a routine cardiac catheterization procedure we figured out that the glue material was actually in the wrong place.

And found out that the thoracic duct was severely occluded by this glue material. So, we went down through that pathway, dilated it. So, in program, the Lymphatics Occlusion Program actually started out with a patient getting a dilation and opening of that lymphatic vessel.

And after the procedure I was corrected to change the name of the program to a Lymphatic Intervention Program, both occlusion and dilatation if we will. So, at this point we offer services for both lymphatic evaluation, diagnostic and interventions, both dilatations and occlusions.

And we have had many patients who have had good results. One patient who has had a recurrence, which is again, common with patients who have lymphatic occlusions. Some patients do have a recurrence of their symptoms, and we have to go back in and do a second procedure. But so far we have been blessed not to have any major complications and pretty much every single patient that we have done a procedure on has gone back home and has done well.

Host: It's amazing. Dr. Reddy. I kind of want to shift now to some of the Teleconsultations that I know that you're a part of. So can you talk a little bit about those Helena Consultations that you're currently offering to providers?

Dr. Reddy: We offer Teleconsult. It's basically phone consultations to physicians across the country and the world, if you will. We've had a couple of phone calls from Europe and South American countries. Primary cardiologists, pediatric and adult congenital heart disease cardiologists who are taking care of their own patients in different states and different parts of the world.

If they have patients with lymphatic complications they reach out to either CHOP or to us and a few other centers who are trying to establish their lymphatic programs. At our institution here at Children's Health UT Southwestern, the way the process works is the primary cardiologist calls our office line.

Because I also lead the Fontan Single Ventricle program, I either do a Teleconsultation with the primary cardiologis,t get all their records, review their records in detail and call the referring cardiologist back and discuss our impressions and the next steps. If the patients are deemed to be a suitable candidate for an lymphatic intervention, we then arrange for these patients to be transferred to our institution and after the initial diagnostic portion, take them to the cath lab for the interventional aspect of the lymphatic intervention.

Prakash Chandran (Host): It's amazing to have that resource available. And I think I just wanted to ask more just about the future goals of the program. You know, you talked about currently how you offer evaluation and diagnostic services for the Kyla's a fusion, the plastic bronchitis, and soon to be PLE or protein losing enteropathy.

But what other future goals do you have for this program and what can other providers look forward to?

Dr. Reddy: I think the big picture is for us to be a program where patients from other parts of the country could come for the lymphatic evaluation and treatment, which I think is already happening. There is a lot of pent up demand for patients, especially patients who could not travel because of COVID and other related concerns or the past year or two.

There has been a historically, only one center in the country who is offering these services. So, if we can become that second center and hopefully many other centers can come up in the country in the near future, we want to be that center in at least in the Southern part of the United States where patients and providers can come or send their patients to get their loved ones taken care of at our institution. That'll be goal number one. Goal number two, which is critical for our entire field to move forward is to streamline the steps for lymphatic interventions. Lymphatic intervention just by itself is a very high-risk procedure.

It's a long day procedure, lots of moving parts. So, we want to make sure that we work towards trying to identify better and easier ways of delineating the lymphatic abnormalities. There are some patients who have pacemakers or ventricular assist devices who cannot actually get an MRI done.

So the first part of the lymphatic evaluation, which is a critical first step, is not possible. So, in those patients we have had such a patients here where we basically had to go directly to the cath lab. The patient on a VAD, ventricular assist device was taken to the cath lab without knowing exactly what type of lymphatic abnormal channels they had or didn't have.

And it was like finding a needle in the haystack and not just finding the needle, but also trying to thread that needle in the haystack. It was a very challenging procedure. Thankfully, we got lucky and we were able to successfully do that procedure, but that shouldn't be the case. We should not be doing the same old routine techniques that are current standard of care.

We should be able to push the field forward and identify alternate ways to delineate these lymphatic abnormalities. Maybe we can take these patients to the CT scanner or come up with the ultrasound techniques that other institutions have started looking at. And we have also started looking at, but think there is more that you will or we all will see in the next few years. And we are on the cutting edge along with three other centers to move this field forward.

Host: It absolutely sounds like that. Dr. Reddy, so just before we close, is there anything else that you'd like to share with our audience today?

Dr. Reddy: I think all pediatric and adult congenital heart disease providers who take care of single ventricles, and even those patients who have two ventricles, the moment you have a lymphatic complication, all of us in the past, we were knocking on our heart failure, heart transplant team to start evaluating our patients, to put them on the list.

And I want all of us to know that now there are newer therapeutic options for patients with lymphatic complications and please call us or call the local center that has a Lymphatic Interventional Program close by so that you can get these patients evaluated and get their treatments so that we can continue to keep these patients with their own hearts for as long as we can. And that'll be my plea.

Host: I think that's the perfect place to end Dr. Reddy. Thank you so much for your time today.

Dr. Reddy: Absolutely. It's a pleasure. Thank you very much.

Host: And thank you to the audience for listening to Pediatric Insights. You can find more information at children's.com/heart. My name is Prakash Chandran and we'll talk next time.