Gayatri A. Acharya, MD: Greetings. I'm Dr Gayatri Acharya, cardiology fellow at Mayo Clinic. During today's recording, we'll be discussing living into adulthood with congenital heart disease in the 21st century. I'm joined by my colleagues Dr Heidi Connolly and Dr Naser Ammash, who specialize in this area. Welcome, Dr Connolly and Dr Ammash.
Naser M. Ammash, MD: Thank you, Gayatri.
Heidi M. Connolly, MD: Thank you.
Living With Congenital Heart Disease Into Adulthood
Acharya: Today's conversation is very interesting. How many patients who are born with congenital heart disease live into adulthood?
Connolly: These days, we expect the majority of patients who are born with congenital heart disease to live into adulthood, and that is due to tremendous advances that have been made over the past 50 years. Our colleagues in pediatric cardiology take such great care of the patients. The cardiac surgeons and other individuals who care for these patients as young individuals and as they grow into adulthood have made this an expectation for us. We would say that 85%-90% of patients born with congenital heart disease will transition into adult care.
Acharya: Excellent. Is there any difference between the patients who have simple congenital heart disease versus complex?
Ammash: There is indeed, because there are two groups of patients, as you alluded to. Simple congenital heart defects might not appear until adulthood, when they become more symptomatic or some abnormality is noted on EKG or chest x-ray. Whereas those with complex congenital heart disease most likely were seen before, had a previous operation or intervention, and then came back to us with the residual disease and sequelae. Although we have seen adults with complex congenital heart disease not presenting until adulthood, the vast majority would have had an intervention before.
Unique Challenges in Adult Congenital Heart Disease
Acharya: What are the unique challenges that face these patients when they seek cardiovascular care?
Connolly: The challenges are quite diverse. As Naser pointed out, some patients are identified to have congenital heart disease when they are adults, and that is a big shock to them because it's something that they never had heard about or anticipated. Learning to live with the consequences and sequelae of congenital heart disease, whatever the lesion might be, may have significant implications.
With the population of patients who were identified early in life, there are obviously issues that occur in adulthood. It may be something even as minor as a bicuspid aortic valve, for example, that has major implications with regard to childbearing years, dilatation of the aorta, multiple interventions, and so on. Something that we think is a relatively minor abnormality might have major implications with regard to future care in these individuals. The very complex patients who have multisystem disorders may require comprehensive care by a subspecialty or specialty team, including specialists in and outside of cardiovascular disease, such as liver disease and kidney disease.
What Makes a Center of Excellence?
Acharya: Can you define for us what a center of excellence for care in congenital heart disease would be? Dr Connolly has mentioned that there are various aspects to caring for these patients.
Ammash: She explained it very well, because it's this multidisciplinary approach in the care of the adulthood congenital heart disease that is most important. We are the main physicians taking care of these patients, but we are supported by a variety of specialists with expertise dealing with those different medical, surgical, and anesthesia issues. The multidisciplinary approach really works well with these patients.
Acharya: As we see these patients for their incident visit, it's excellent to have that multidisciplinary approach. How do we follow these patients?
Ammash: Again, it depends on the lesion. That is what is so different about this specialty. Every lesion is followed differently because of the different problems that could be encountered in adulthood. For example, a simple lesion, such as septal defect or atrial septal defect, can have a residual defect. If it has been repaired before, patients can have arrhythmias depending on age of the operation and depending on their age. They can have residual biventricular enlargement or dysfunction or tricuspid regurgitation or pulmonary hypertension. Each lesion carries its own potential issues that we have to address every time we visit.
Now, not every patient needs to be seen every year. Obviously, the more complex patient needs to be seen every year, and sometimes we see them every 6 months if there are issues. We might see some patients with simple defects every few years, and some every 3-5 years. It really depends on the individual, and we use a case-by-case approach.
Acharya: What other special considerations do these patients have, whether their primary care provider is taking care of them or whether they visit us at Mayo Clinic for our specialized adult congenital clinic?
Connolly: Again, as Naser pointed out, it depends on the patient and it depends on the lesion. Of course, every patient needs to receive individualized care. But one example would be patients who have undergone a Fontan palliation who have liver disease. Those patients require regular surveillance of their liver, including ultrasound and blood work to look for problems that occur, such as cirrhosis and/or malignancy that can develop as a result of cirrhosis.
Another issue is patients with congenital heart disease who would like to have children. There may be genetic implications. There may be implications with regards to the ability to carry a pregnancy safely. Of course, the patient would be followed during a pregnancy should they decide to proceed. There are a wide range of issues that one would need to address.
Addressing Activity Limitations
Acharya: How would you specifically address activity limitations, especially in our younger patients such as high school and middle-school-aged children?
Ammash: We see more patients who have been restricted from activities because of their congenital heart defect. But here again, it's a lesion-dependent issue. Many patients born with congenital heart defects can exercise as much as they want up to a certain limit, whether they have been operated on or not.
I'm always against pushing patients to the extremes, such as exercising up to the point of severe exhaustion, and so I usually tell them not to do so. But we will decide depending on the lesion. We often do a cardiopulmonary stress test to see what happens to them when they exercise and gauge their response. Again, it depends on the patient and on what we are dealing with.
Connolly: And, of course, we emphasize the importance of a healthy lifestyle.
Ammash: Yes, that is very important, because although these patients are born with heart problems, they have been seeing cardiologists since they were children and infants. When we see them in an adult clinic, not only do we tackle the congenital heart defect, but we also focus a lot on lifestyle changes: blood pressure, smoking history, weight. We don't want to add any acquired element to their congenital heart defects. That is a very important part of the care of these patients.
Acharya: Do you have any other additional thoughts about the patients that we care for, especially at Mayo clinic with a multidisciplinary approach?
Connolly: I think you really emphasized one of the critical features, that having expertise in multiple different areas is so important for these patients. Of course, in pediatric cardiology, imaging in congenital heart disease has become multidimensional. We don't just think about two dimensions. but we can think three-dimensionally with imaging. We can even create and use models that can be potentially be used to do a procedure virtually by the surgeon or the proceduralist. There are lots of opportunities there.
We work with individuals from cardiac surgery, anesthesia, critical care, heart rhythm, circulatory failure, maternal-fetal medicine, and genetics every day with regard to our patient population. Having a tight network of experience in those areas is so important and all of those things are also important with regard to the patient.
What creates a center of excellence? It's really expertise in multiple different areas with the focus being the patient, and really trying to provide the best care for every patient every day.
Acharya: Wonderful. Thank you, Dr Connolly and Dr Ammash, for these very important insights. And thank you, the audience, for joining us on theheart.org | Medscape Cardiology.
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Cite this: Adult Congenital Heart Disease in the 21st Century - Medscape - Aug 07, 2018.