May 12, 2015 — With advancements in medicine, many babies born with congenital heart disease are now surviving into adulthood. With proper care and treatment, adults suffering form this disease can live full and happy lives. Dr. Colin Cowley is a pediatric cardiologist who runs a program for adults living with CHD. In this podcast, he and Dr. Tom Miller talk about the importance of adults with CHD having regular checkups and preventing problems

Interview

Dr. Miller: Congenital heart disease, what is that exactly? We're going to talk about that next on Scope Radio.

Announcer: Access to our experts with in-depth information about the biggest health issues facing you today. The Specialists with Dr. Tom Miller is on The Scope.

Dr. Miller: Hi. I'm here with Dr. Colin Cowley and he is a Professor of Cardiology at the University of Utah and he also practices at Primary Children's Hospital. Colin, tell us a little about what congenital heart disease is.

Dr. Cowley: Congenital heart disease is a form of heart disease present when a child is born. Congenital just simply means present at birth. There's a large spectrum of diseases that we see, ranging from very mild that essentially heal themselves to life-threatening forms of heart disease the require multiple surgical procedures and other types of interventions.

Dr. Miller: Do you have any estimate as to how often a child might have congenital heart disease out of the population?

Dr. Cowley: It happens in about 1% of all live births. There's a large spectrum, as I've just said, from very mild to immediately life-threatening.

Dr. Miller: And so I would think, with modern medicine since the '50s probably, a lot of these kids are alive now and living into adulthood. Is that correct?

Dr. Cowley: That is correct and that's one of the important changes in medicine, at least from my perspective. Many of these forms of heart disease, historically children at a very short live, many of them didn't survive the newborn period. Others survived into childhood very handicapped as a result of their heart disease. But with advances in surgical intervention and catheter-based intervention and imaging and medications, the vast majority of these children now survive into adulthood.

Dr. Miller: Which then presents a new set of problems, I believe. So that they need to be managed lifelong it sounds like.

Dr. Cowley: Exactly.

Dr. Miller: And it's not just regular care, they need specialty care I would assume, right?

Dr. Cowley: This is very specialized care. There are now more adults in the United States with forms of congenital heart disease than there are children. There are well over 100 large centers around the country that provide care for children with congenital heart disease and very few centers that are now prepared to take care of adults with congenital heart disease.

Dr. Miller: And my understanding is you run of these centers of Primary Children's, is that correct?

Dr. Cowley: Correct. It's a collaborative program working with people at the university hospital as well as Intermountain Medical Center.

Dr. Miller: And this involves multiple specialties or a multi-specialty orientation so you've got cardiology, thoracic surgery and pulmonary medicine.

Dr. Cowley: Yes, there are a large number of sub-specialties that become involved with these patients. In addition to heart disease, there is a very high instanced of associated diseases, kidney disease, lung disease, neurologic disease and a variety of psychosocial issues that have the potential to very seriously affect these patients.

Dr. Miller: How does a patient get into your program? How do they seek out and receive that specialty care that seems so important to their longevity?

Dr. Cowley: Many of them have been within our program throughout their life. We estimate there are about 8,000 adults in Utah alone with some form of significant congenital heart disease.

Dr. Miller: That's a lot.

Dr. Cowley: It is. Many of those have been lost to follow up and we are in the early stages of really formalizing our program and we will reach out to everybody any way we can to make sure the people realize we now have the infrastructure set up to provide them with comprehensive care across the board.

Dr. Miller: So for a patient who's involved in comprehensive specialty care, can they expect to live a pretty full life, and work, and is that generally the case?

Dr. Cowley: Many of them. It depends on the severity of the underlying heart disease. Some of these children are born essentially with half a heart and they undergo a series of operations where they have one pumping chamber that pumps blood out to their body and the blood flow to their lungs, instead of being pumped essentially has to go there through gravity. That's associated with many negative consequences including, for many of them, serious liver disease. But the expectation is that the majority of those patients will go on to need cardiac transplantation and some of them, liver transplantation at the same time.

Dr. Miller: Now that would be the minority of the patients in your group?

Dr. Cowley: That is the minority, so most of the patients that make it to adulthood with congenital heart disease have what we would call two ventricle physiologies. So they have two pumping chambers and they have problems with valves or other issues. But there are those with the more complex forms of heart disease that represent a minority.

Dr. Miller: So for patients who have congenital heart disease that live at a distance, how often would you see them and also would you transfer some of their care back to their physicians wherever they're from, say if they're from the different part of the state or a more rural part of the state? How do you deal with that, do you have outreach clinics?

Dr. Cowley: Yes, so we have outreach clinics, in fact, we're reaching into Idaho as well we have a formal clinic in Saint George and we're actually reaching into Alaska now. There's a large population up there that will benefit from our services, but we do . . .

Dr. Miller: So you have an outreach clinic in Anchorage?

Dr. Cowley: Yes, we do. Depending on the patient we might just need to see them once a year, sometimes twice a year. If they're much sicker, more frequently than that. We try to partner with their primary care physician to provide them with support to minimize the number of times they actually have to make the trip to see us, especially if they are remote.

Dr. Miller: What would be the average age of the patient that attends your adult congenital heart clinic?

Dr. Cowley: Probably in the mid-30s at this point.

Dr. Miller: Why is it important that these patients with congenital heart disease contact your clinic?

Dr. Cowley: Well, I think prevention is key here. If we can anticipate problems for some of these patients we can potentially prevent many, many downstream problems. There's a very high rate of associated problems for these patients, some of them cardiac, some of them non-cardiac. The cardiac disease can become progressive. So if they have rhythm problems, which a large percentage of these patients do, they may not know it.

Dr. Miller: You mean abnormal heart rate?

Dr. Cowley: Exactly. Abnormal heart rates or abnormal heart rhythms. There are some excellent providers in the community that can provide very good care, but the complexity for some of these adults really requires sub-sub-specialty care that I think there's nobody else really adequately qualified to provide.

Dr. Miller: So whom should they contact if they were to call Primary Children's Hospital?

Dr. Cowley: If they called Primary Children's Hospital or the university hospital operator, either number will get them, and ask for the adult congenital heart disease program. They will get put through to us and we have a team of people prepared to provide care for you.

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