Ali Zaidi, System Director for Adult Congenital Heart Disease at Mount Sinai, talks about the importance of a transition of care for patients with congenital heart disease from childhood to adulthood.
When you talk about transition of care, I wanted to start first because we have a very general viewing audience. When you’re talking about congenital heart disease, how many people in the United States does this roughly affect?
Zaidi: That’s a great question. Let’s just talk a little bit about congenital heart disease. What congenital heart disease means is that you’re born with this. Children are born with congenital heart conditions. They could be either mild or severely complex. There is a whole plethora of different disease processes. Now what happens with adult congenital heart disease is that these kids now survive into adulthood. So now they’re adults, but they were born with congenital heart disease, so they’ve had multiple open-heart procedures or catheterization, but they survive. What we know now, in this day and age, is that there are more adults with congenital heart disease than children with congenital heart disease. If you just think about that statement for a second, what that means is that because of the success of pediatric cardiologists, these kids are now all grown up and they’re adults. You have more adults now than kids. So that’s sort of where adult congenital heart disease lives. We also know now that there are more than 2 million adults with congenital heart disease in the United States. That’s what we estimate it at. The interesting caveat is that only a fraction, a small percentage, single digit percentage of those 2 million actually get the required care from an adult congenital heart disease center or specialist. If you look at the numbers, there are 2 million out there and less than 10 percent get the specialized care.
Why is that?
Zaidi: That’s where transition comes in, is that if you look at transition of care, what transition of care means is that these children with congenital heart disease or any chronic illness, be it cancer or sickle cell or blood disorders, autoimmune conditions, cystic fibrosis, congenital heart disease, is one of those chronic conditions you’re born with. Transition of care is that these children who were born with congenital heart disease are slowly ready, or they slowly sort of transition from pediatric health care, from pediatric cardiology, into the world of adult medicine. And that’s a process of transition. So, you’re taking someone who’s 15, 16, 17 years old, maybe even less, with a heart condition and you’re going to ready that patient through a process along with their parents and you will move them from pediatric heart health care to adult health care. And that’s a process.
Why are they falling through the cracks?
Zaidi: Several of them fall through the cracks. If you look at the numbers, as I said, two million adults with congenital heart disease and less than 10 percent reach adult congenital heart specialists. So, what goes wrong? The onus is that they are not transitioned appropriately. They are not transitioned timely. If I was a pediatric cardiologist and I would be sitting with a 15-year-old patient and the family and say well listen, when you become 18 or 21, you will find an adult congenital specialist for you. Now there are several reasons why that doesn’t happen, and I’ll go through some of the salient reasons. One of the problems is there are not enough adult congenital heart specialists in the country, and that has been a phenomenon maybe over the last several years. If you look at the numbers again, there are maybe a few hundred who are what we call board certified in this field, which means they took an exam. They got certified and are now certified to practice in adult congenital heart disease. Only a few hundred in the country for two million people. There are very few who are trained exclusively in adult congenital heart disease. What that means is that you take a physician who then goes through med school and then residency and fellowship and then adult congenital heart disease. Very few people out there who are uniquely trained in Adult Congenital Heart Disease. So now, the number of physicians out there is very, very small. So that’s the health. That’s the health care arm of this. That’s where the problems are. A lot of patients, not enough doctors. The second part of this is really two parts. One is we as physicians or health care providers haven’t done due diligence in teaching the patients or teaching the parents about transition. Somewhere along the lines, that message didn’t get across uniformly and constructively, that these young children eventually do need specialized care and they need specialized care lifelong. That message doesn’t go across clearly. The onus is on us, as physicians, as pediatric cardiologists, or health care providers that we need to tell the patients and their families that you need somebody down the road for the rest of your life, and that’s, again, part of the process of transition. The third part is the patients and the families themselves. The reason why there is a gap there is because these are young patients, adolescents and teenagers, and you know I’ve done this for a long time now. They think they’re invincible. They’re running around. They’re feeling good. They go to school. They’re getting ready for college. They’re going to get the first job. Why do I need to see somebody? Why do I need to see somebody for the rest of my life? That message doesn’t resonate, and sometimes, it doesn’t sit with the families either, the parents. Keep in mind these parents have taken care of this child since birth. The first operation, the second, the third, and the fourth. So the parents are very, very intricately involved in this process, but they see their kids now in their adolescence and teenagers and go they’re fine. They’re doing okay. That’s again a false perception because even though they’re not sick every day of their lives, they can get sick maybe down the road. So it’s really a three pronged process. It’s the health care system itself. It’s the patients and then it’s the physicians where we just haven’t done the due diligence in getting that message across.
What’s the likelihood that a child who had been treated for a congenital defect 10, 20, 30 years – at some point will something pop up that will require additional assistance?
Zaidi: That’s a fantastic question. I always tell my patients that based on the condition they were born with, the congenital heart condition they were born with, there are some patients who may go many decades and nothing is needed. There are some patients who have a greater complexity of an underlying congenital heart defect where they do need something done sooner than other patients do. Is it a one size fits all? It isn’t. It’s not that every patient will have a complication. I think that is where we try to explain to our patients is that because of your complexity, you need to be followed because you could develop abnormal heart rhythms. You could have what we call heart failure. The other caveat in what we are also finding out is that these patients are getting older, remember they’re surviving to adulthood, but in adulthood they’re living longer. Now, there are 50, 60 and 70 year old patients with congenital heart disease. Now, they have the spectrum of adult acquired heart disease coming in, so they have diabetes and hypertension and coronary disease strokes. They have liver disease. They have kidney disease. So now you’ve taken congenital heart disease that is thought to be just with kids. You’ve moved into adulthood and you’ve added a layer or more than one layer of adult comorbidities. When I speak to my patients, I tell them, I can tell you that you’re gonna do fine for many years, but you will need follow up because something may happen down the road. The other part I’ll bring up and I don’t know if you have a question down the road for this is pregnancy. These are young women who were born with congenital heart disease who sometimes have had one or two or three open heart procedures. They go through their transition years or what we call the teenage years and they’ve really not thought about pregnancy. They have not thought about what pregnancy will do to their heart condition, what will happen to them when they get pregnant, what will happen to them at the time of delivery What will happen to their children? Is there a genetic risk? Is this something that’s transmitted or not transmitted? More importantly, what will happen to the second pregnancy or the third pregnancy? Can they carry a second or third pregnancy? When we talk about transition and adult congenital heart disease, pregnancy is a big part of this because we again have to ready these young women for what lies ahead. They want to have families, want to have their own children. This is again a big part of transition.
What do you recommend to patients? This will go on across the country. Not every patient may be close to a center where there’s a specialist. What do you recommend to parents who have kids with a congenital heart defect and also to those young adults who were treated when they were younger?
Zaidi: That’s a fantastic question because it goes back what we just talked about is that there are not enough adult congenital specialists in the country. That’s number one. Number two is you can have an adult congenital specialist, but you also have to have a good adult congenital heart center that’s working with that adult congenital specialists. What that means is that it’s not just the physician itself. You need a team. You need the right surgeons. You need the right catheter docs. You need electrophysiologists, folks who do pacemakers and ICDs, you need heart failure transplant physicians, pregnancy or what we call maternal fetal medicine. Will every city or town have that? No. But what we do know now that we didn’t know many years ago was that now, with adult congenital heart disease, there are centers or clinics that are in every state in essence, so physicians who are working with adult congenital patients can be found when patients or parents look. There is an Adult Congenital Heart Association. It’s called the ACHA. That is a wonderful organization which has a medical advisory board and it caters to patients’ needs, and they have a directory where patients can actually look at that directory and find an appropriate adult congenital heart physician state wise. So, it’s doable. It is doable. It may take some extra steps if you’re not in a big city or you’re not close to a big city, but it is doable. The Adult Congenital Heart Association is a wonderful organization and they give the resources.
The fact that we have this problem for older adults – does that speak to the success of what you and your colleagues are doing?
Zaidi: Fantastic question. Adult Congenital Heart Disease or ACHD has grown over the years because of the success of pediatric cardiology and pediatric congenital cardiothoracic surgeons. Over the last 50 to 60 years, the progress in surgical outcomes and catheter-based outcomes in the world of pediatric cardiology has been exponential, and because of that, these kids are surviving, and because of that, these kids are getting to be adults. That’s where we come in is that we take these young adults and we want to take them to 60 and 70 and 80. Who knows where? The success doesn’t just stop with being an adult. It doesn’t stop there because now it’s you want to take them much further. So you’re right. If I were to wear my pediatric cardiology hat on, it is truly kudos to the folks who came decades before me because they laid the groundwork. They laid the groundwork.
We’re going to be speaking to Michael Parnack.
Zaidi: Yes. Mike’s a fantastic young man. He’s a great guy. Mike has congenital heart disease. His story is fascinating. He was again operated on when he was a kid and he did really well. He lived in New York and then somewhere along the way he fell out of care. He fell out of health care, and then he got sick. That’s how he came back into the world of adult congenital heart disease and he met the right physicians and one thing leads to another. He’s doing really well now, but his story reflects what’s happening in adult congenital heart disease across the country, is that these young patients don’t always appropriately transition. The one point I want to make is not that they all get lost. It’s not that all of them are sort of dispersed, but the majority don’t make it appropriately into adult control. When I’m even giving lectures, I don’t use his name, but I say that he’s a young man now who worked his way through law school. He has a job as an attorney in Manhattan. He works full time. He goes to the gym. He enjoys his life. He now has a significant other who, you know, they’re thinking of starting a life together. That is important because that’s what we as adult congenital physicians would like our patients to do, is that we want them to have full lives. It’s wonderful to see him.
Do you know what kind of congenital heart disease he was treated for?
Zaidi: Yeah. He had what we call a cyanotic congenital heart condition and he had initial operation when he was a child and then he had a pulmonary valve replaced, and then he was lost to care for a while. Somewhere along the lines, he actually got an infection of his heart, and then he needed to get surgery again.
Does he have any restrictions?
Zaidi: No he doesn’t. He’s doing well. He’s done really well. I try not to restrict my patients until it’s an absolute restriction. I tell them, you know, if you want to go bike and hike and jog and run and swim, go for it. There are some places I restrict patients, but I want them to have full lives as much as they can. That’s what I want Mike to do as well.
Is there anything I didn’t ask you that you want to say?
Zaidi: I think you hit the salient features. I think the important thing out there is that this is a field that Adult Congenital Heart Disease needs a spotlight in the sense that we need this to get out. There are too many patients out there who are not being seen by appropriate physicians. There is a gap as we’ve talked about. We have to focus on transition. Transition is essential for these patients to not get lost. One of the things I didn’t highlight is that we know there is national data that when patients don’t transition from pediatric cardiology to adult congenital heart disease or adult cardiology, there is what we call increased morbidity and mortality, which means the data out there is that if they don’t transition, if they don’t transition appropriately, they tend to get sicker and then they get hospitalized. One thing leads to another. There is what we call higher morbidity. I think it’s preventable because if it’s done in the right way, it’s done in a timely way, you can prevent those morbidities or those complications from happening.
Do you know what the percentage is?
Zaidi: It’s dependent on a couple of different things and it’s going to be hard to say what the percentages are because it depends on the complexity of the lesion and it actually depends even state wise. Some states have higher than other states, so it’s difficult to put a number on it. But I think those are the salient things. If I can try and think if there is anything else that comes to my mind. I think we talked about pregnancy. Those are the big ones.
Interview conducted by Ivanhoe Broadcast News in February 2020.
END OF INTERVIEW
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