Management of Children and Adults with Congenital Heart Disease

Podcast discussion with Shailendra Upadhyay, MD, CEPS, FHRS, and Irfan Warsy, MD

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In this episode, EPLD talks with Drs Irfan Warsy and Shay Upadhyay from Connecticut Children's Heart Center about updates in the management of children and adults with congenital heart disease (CHD), including evolution of their program, development of their hybrid lab dedicated to children and adults with CHD, and more. Shailendra (Shai) Upadhyay, MD, is the Division Head of Pediatric Cardiology and Co-Director of the Cardiovascular Institute at Connecticut Children’s; he is also an Associate Professor of Pediatric Cardiology at the University of Connecticut School of Medicine. Irfan Warsy, MD, is the Director of Cardiovascular Electrophysiology at Connecticut Children’s and Assistant Professor of Pediatric Cardiology at the University of Connecticut School of Medicine. 

Irfan Warsy, MD: I wanted to thank EP Lab Digest for inviting us to talk about the program at Connecticut Children's. My name is Irfan Warsy, I'm one of the pediatric cardiologists here and also the director of pediatric electrophysiology (EP). I’m joined by Dr Shay Upadhyay, director of the pediatric cardiology program at Connecticut Children's. He is an electrophysiologist and adult congenital heart disease (ACHD) specialist. It's one of a very small number of programs in the country where we cater to both children and adults with CHD, and one of the few examples of centers where patients of all age groups are treated. Last year, I was hired by Dr Upadhyay to come on as director of EP at Connecticut Children's. I’ve had experience from various institutions and been able to see the good and bad about programs at freestanding children's hospitals versus programs where there are shared facilities with adult patients. Coming to Connecticut Children's was a wonderful experience, because I am now at a program where everything is under one roof. I want to pass this conversation over to Dr Upadyay and have him go over his experience in the evolution of the program at Connecticut Children's.

Shailendra Upadhyay, MD, CEPS, FHRS: Thank you, Irfan, for the kind introduction. Yes, the program is very close to my heart. I came to Connecticut Children's in 2014 with prior training in both ACHD and EP. I am also an internist by training and certification as well. When I came here, my goal was initially to focus and grow the ACHD program. Connecticut Children's has an excellent history. Leon Chameides established the Connecticut Children's program in 1957. In the EP world, we all know that maternal lupus can cause heart block in babies, and he was the first to describe this in his New England Journal of Medicine paper decades ago. He is also regarded as the father of pediatric advanced life support (PALS). So, we're lucky to have this program that he built, which eventually transitioned into the program that we have today. When I came here in 2014, we did a lot of organization of the ACHD service. At the time, there were shared cardiac surgery and EP services with another program within the state, and over the years, we consolidated the program into one. Now, we have our own fully functional cardiac surgery and EP program and interventional cardiology programs. So, I think we've come a long way. When it comes to the adult congenital services, the program was consolidated to have more than one board-certified adult congenital cardiologist, electrophysiologist, interventional cardiologist, and surgeon. We were accredited as an ACHD center. Having received this accreditation by the Adult Congenital Heart Association in 2018, we were the first program in Connecticut to receive that accreditation, and we have continued to provide excellent service to all the CHD and adult congenital population. 

Irfan Warsy, MD: One of the interesting things for me was the hybrid laboratory that you have developed at Connecticut Children's. It is state of the art and it's quite a point of pride to have a laboratory like this under the roof of a children's hospital, owned and functioned by the children's hospital. We have had the good fortune to share the laboratory with the adult cardiologists. It has its benefits and downsides as well. Obviously, the benefits are that we are introduced to and have access to a number of high-tech advancements that our adult colleagues are utilizing, and we can gain experience from that. One of the things that drew me here was that we had all those technologies and continue to gather those technologies under the roof of pediatrics and the adult congenital service. Could you talk about the development of our lab, where it was and where it is now?

Shailendra Upadhyay, MD, CEPS, FHRS: As we started to get our programs accredited and as we started to provide state-of-the-art services, it was crucial for us to stay on top of not just skills but also with the latest technologies, to make sure that our patients receive the best and safest care with minimum exposure to x-rays. One of the things on top of my agenda was to make sure we developed a laboratory that was state of the art and could provide all complex interventions such as transcatheter valve replacements, device implants, and 3-dimensional mapping, pacing, and ablation in this population. We were very much supported by our leadership, and we got our hybrid laboratory built, which is dedicated to children and adults with CHD. Being a freestanding children's hospital, there are advantages and certain disadvantages, but I see most of the things to our advantage, as we try and keep pediatric and adult patients with CHD within our health system. When it comes to medical or interventional care, there are certain scenarios where we would consider sending them to our adult partner hospitals, such as if the patient needed a lead extraction, which is a service we do not have here at Children's, or if they had multiple medical comorbidities that were predominant over their cardiac issues, we tend to provide those services at the partner adult institution.

Irfan Warsy, MD: One of the one areas in which we really are pioneering here is in the use of zero fluoroscopy for certain procedures. I've rarely had to wear lead this entire past year that I've been here. Again, that's a testament to the vision and culture of the program itself. It is said that there is no such thing as good fluoro and that there is no amount of fluoro that is acceptable. So, we have continued with that. We still use fluoro for certain procedures, such as transseptal puncture, but this year we are working to eliminate even that with certain new technologies that have been acquired. Shai, could you talk about the cases that we're now performing with zero fluoroscopy and the numbers that we've done?

Shailendra Upadhyay, MD, CEPS, FHRS: That is another great area of progress. I was able to perform the first zero-fluoroscopy ablation in a child in 2015 here in Connecticut, and since then, we have pretty much done zero-fluoroscopy ablations for nearly all our cases. When we look at the impact data on our fluoroscopy times, we are way below the national average for fluoroscopy use. Some of the arrhythmias that I routinely use it for are AV node tachycardia in children and most right atrial arrhythmias in children, including ventricular tachycardia in some patients with CHD. So over 90% of our cases are without fluoro, and we have only been helped with your addition to our program. Those numbers keep growing as we stay away from radiation.

Irfan Warsy, MD: I also wanted to stress that despite this, we've had almost no complications. A part of it also is a great deal of experience that both of us have had in ablation, even before instituting the zero-fluoro policy. But with that experience, it has been quite a smooth set of procedures that we've been able to do. Shai, one of the challenges that I've experienced a lot in my more than a decade in different institutions has been what we do with our pediatric EP patients once they reach adulthood, specifically, our pediatric patients with CHD. This is something that is really plaguing a number of practices around the country, where we, as pediatric cardiologists, have a great deal of experience and knowledge of ACHD itself, and electrophysiologists have a great amount of knowledge about electrophysiologic abnormalities that occur in these patients with CHD. But once the patients reach adulthood, after a certain age, we need to transition them to the adult service, which may not be as comfortable with the anatomy, though they may be comfortable with the electrophysiologic issues. One of the great strengths that I've seen at Connecticut Children's is we have a number of ACHD specialists who deal with that. Shai, could you talk about the adult congenital service at Connecticut Children’s and how many practitioners we have currently?

Shailendra Upadhyay, MD, CEPS, FHRS: We are really proud of our adult congenital service here. It was initiated by Dr Felice Heller, who just recently retired, and then it was consolidated into a full ACHD program from 2016 onwards. Since then, we've done some remarkable things in the program's growth. When it comes to caring for these patients, whether they're best served at a children's or adult hospital, numerous models exist within the country. There are certain programs where they're all managed on the adult side, there are programs where they're all managed on the children's side, and there are hybrid models. There are unique challenges to the freestanding children's hospitals, which is what we are. While we have all the expertise from the cardiac side, meaning the cardiac surgeons, interventional cardiologists, sonographers, echocardiographers, and electrophysiologists who have expertise in their conditions, we may have nursing staff, anesthesia, or PACU recovery people who may not be as comfortable in caring for these patients. I feel like we are quite lucky in our program in that we've had full support all along, from the physicians to the support staff to leadership, in making sure that we are able to deliver the best care to adults with CHD. That is sort of our rationale of keeping all the adult patients here with us; if their major medical comorbidities are predominant, they would go to the adult partner institution. Having said that, we work very closely with our cardiac intensive care unit and nursing, and provide them periodic education in terms of how to specifically deal with adult patients. We also have protocols in place as to the times when these patients need to be transferred to the adult hospital, either on an urgent or elective basis, if their medical issues are becoming predominant. We have a wonderful group of cardiologists on our team, including 18 cardiologists who do critical care. We have 3 adult congenital cardiologists and a dedicated advanced practitioner for the ACHD program and one for the pacemakers. We have 2 nurses who are dedicated to the ACHD program as well. So, it's a well-rounded program that provides all the services needed for the pediatric to the adult age group. We also have a formal transition program, so for our pediatric cardiologists who take care of children or adolescents, as they start evolving into young adults or adults, they get transitioned and are seen by the ACHD service.

Irfan Warsy, MD: I think that that is something that is really quite a model for institutions around the country. One of the aspects of pediatric EP that is really quite underdeveloped, and we discussed this in depth when I arrived here, was the development of an autonomic dysfunction program, which traditionally, has fallen upon either neurology or EP, both in the pediatric and adult side, not so much because we're trained in it, but mainly because the buck ends with us in a sense. We have nowhere else to send them. We talked about this at length before I joined, and upon arriving, Shai and the leadership were tremendously supportive of the development of what we've now instituted as the Connecticut Children’s Center for Autonomic Dysfunction in the Young. Over the last year, we've seen more than 800 patients at the program, including more than 200 new patients with POTS, which has seen quite a dramatic increase in numbers since COVID-19, although it has been around for decades. There are really no centers of this kind in the country, apart from a small handful scattered across the country. So, in coming here, one of the goals was to develop the program. We will hopefully be adding an APRN by the end of this year or beginning of next year to the program. We've gone from building volume to now trying to expand the program itself. It's a program that utilizes the help of and expertise of 12 different subspecialties, including GI and sleep medicine. What we're working to do now is to develop protocols and hopefully add to the research that's out there about this very pertinent but little researched condition. One of the directions in which we are going is the use of a procedure known as cardioneuroablation, which has both pros and cons, and which is very actively being debated upon. I have a particular interest in heart rate variability and measures of heart rate variability, and I think that the union of measures of heart rate variability and post-procedural clinical follow-up will be a direction which we will be focused on in the future in terms of the direction we're going. 

Shai, I think this has been a wonderful conversation. It has really highlighted the program itself and the direction in which the program is going. I want to thank EP Lab Digest for inviting us to talk about our subspecialties, and I want to thank Shai for being available to discuss the program and how his experiences have helped to shape the program.

Shailendra Upadhyay, MD, CEPS, FHRS: Thank you.

The transcripts have been edited for clarity and length.