Melanie Cole (Host): Treating congenital heart defects in adults is aided by the understanding of the continuum of the disease from its infancy. My guest today is Dr. Mark Cribbs. He’s the director of Alabama Adult Congenital Heart Program at UAB Medicine. Welcome to the show, Dr. Cribbs. The American College of Cardiology and the American Heart Association have developed standards for treating adults with congenital heart disease. What are some of the challenges that you see in treating these adults?

Dr. Mark Cribbs (Guest): Probably one of the biggest challenges that we face is just the patients not coming for their appointments. In other words, there are a significant number of patients who are lost to follow-up well before they even reach their 18th birthday. A recent study has demonstrated that as many 50% of patients stopped seeing their cardiologist by the time they reached the age of 13 and that percentage does continue to climb as they get older in age. So, even by the time they reach 18 years of age, more than 60% of the patients that have mild, maybe even moderate, or even severely complex congenital heart disease have stopped going to see their doctor.

Melanie: In this continuum of care, how often should patients be seen throughout their lives?

Dr. Cribbs: That’s a great question and it’s one that patients ask me all the time. It really does depend on their cardiac history. For example, someone who has a history of a simple VSD that was closed very early in life, they really might not need to be seen but every two or three years. While someone who has a single ventricle may need to be seen as often as every three to six months.

Melanie: Because there are so many different types of congenital heart disease, Dr. Cribbs, the guidelines include general recommendations that apply to most patients but give us some examples of low risk, simple, congenital heart disease.

Dr. Cribbs: So, as I mentioned before, a VSD that’s been closed early in life is one of our more simple defects; however, a simple VSD can very much range in the spectrum. It really just depends on the size. So, if it was a small VSD that was closed early on, the risks that we expect for that person to have later on down the road are really quite low. However, if it was a very large VSD that was closed later on in life, the patient may have already developed significant symptoms such as pulmonary hypertension. Then, further on in the spectrum would be something like Tetralogy of Fallot which is considered a complex congenital heart defect which has a number of issues that can occur down the road including significant pulmonary insufficiency, dilation of the right ventricle, shortness of breath, and very significant arrhythmias.

Melanie: So, when you were talking about single ventricle, so are these along the lines of examples of highly complex congenital heart disease?

Dr. Cribbs: Absolutely. They are, by far, our most complicated patients that we take care of; however, they are doing very, very well and leading productive lives out in the community and oftentimes having families of their own.

Melanie: If cyanosis is present, what are some other health problems that can result from that?

Dr. Cribbs: So, cyanosis can lead to the ability of a clot for example that might develop in the leg after a long car trip to travel up to the heart and then scoot over to the left side of the heart which would then have free reign to either go into the coronary blood vessels themselves or up to the brain and cause a significant stroke. The other things that we see related to cyanosis are an elevated hematocrit and I think one of the long-term misconceptions related to an elevated hematocrit is that it, by itself, can cause a higher viscosity in the blood and increase in someone’s risk for having a thromboembolic phenomenon, such as a stroke. But, what we’ve learned is that having a high hematocrit doesn’t, in and of itself, put that person at risk for that, it’s when their iron levels are low. That’s what can really increase the risk. The reason why I bring that up is that patients that have a high hematocrit are often sent to our office and tell me that they’ve had multiple times when blood has been removed from their body in order to try to lower their hematocrit and that has really been seen to be more detrimental than good, more harmful than good, especially if their hematocrit has not being causing of strokes.

Melanie: What are some other comorbidities that can arise with congenital heart disease in adults?

Dr. Cribbs: Probably the most common comorbidity that we see, and actually one of the more common reasons that patients re-present to care is the issue of arrhythmia. Pretty much every person that has had a congenital heart surgery is going to be at risk for an atrial-type arrhythmia. Most often, it’s an atrial flutter. The fortunate news about that is that it’s easily treatable in the EP lab. In fact, more than nine times out of ten, it can be cured. The other types of arrhythmia that we encounter in patients with different types of defects however are ventricular arrhythmias and those can lead to sudden cardiac death. Perhaps the other very, very common comorbidity that we find in patients as they age is the issue of heart failure and actually heart failure and arrhythmias are the most common reason why adults with congenital heart disease actually succumb to their disease and die.

Melanie: If they were coming to you on a regular basis, are you doing testing on them? Is there exercise testing, stress testing involved?

Dr. Cribbs: Absolutely. So, it really, again, gets back to what their heart history is like. For someone who has a history of a VSD that was repaired early in life, they’re at a small but present risk of atrial arrhythmia and so if they are complaining of palpitations, we would certainly do monitoring such as a Holter monitor or an event monitor, certainly an EKG. Someone who has a history of Tetralogy of Fallot, where the risk of atrial arrhythmia of even ventricular arrhythmia is much higher, we will routinely screen them with monitors to make sure that we can catch something that they might not even feel. And then, when it comes to stress tests, we do that on a number of patients, particularly when they complain of symptoms such as shortness of breath or chest discomfort when they’re trying to be active or exercise.

Melanie: Are patients more at risk for infective endocarditis?

Dr. Cribbs: That’s another good question that patients ask me a lot and what we’ve found is that the risk of endocarditis is actually probably lower than what we believed it to be in the past. Those who are at risk for endocarditis are those who have prosthetic valves, those who have a history of endocarditis are certainly at risk for having it again. Those patients who remain cyanotic and then those who have a small, residual defect where the surgeon tried to close a hole, for example, a VSD that might not have been closed all the way where there’s a residual defect. Those patients are also at risk for endocarditis. But, by and large, the majority of our congenital heart patients are not at an elevated risk of endocarditis.

Melanie: How important is it that these patients see an experienced, adult congenital disease heart center?

Dr. Cribbs: The adult congenital center that’s experienced with these types of patients is going to be able to offer them a full range of things that they would need--everything from an EEG to an echo to advanced imaging such as cardiac MRI or a cardiac CT to stress test to CAT and so forth. It’s really the experience and the knowledge of the congenital heart defect that helps these patients the most. There’s a number of places in Alabama, for example, where a person could get a cardiac MRI but then the person who’s reading the test may not fully understand the congenital heart defect itself. So, they might get good pictures but they may not be able to interpret all the details the way that someone here at UAB could.

Melanie: And, in just the last few minutes, Dr. Cribbs, how could a community physician refer a patient to UAB Medicine ACHD Center?

Dr. Cribbs: Right. So, one of the easiest ways is to go online to our website which is at UABMedicine.org. However, perhaps the easiest way to do it is just to call. The number is (205) 996-9000 and our schedulers work very hard to get patients in quickly and we really do try to “bend over backwards” to get these patients in on a timely basis because, as I mentioned before, a lot of these patients haven’t been seen for years and years and so getting them in quickly and trying to figure out what’s going on, really is important.

Melanie: Tell us about your team at UAB Medicine.

Dr. Cribbs: Our team at UAB most directly involves myself and, then, I have two other pediatric cardiologists, Dr. Colvin and Dr. Johnson, who have been taking care of children and adults with congenital heart disease for years and they’re certainly and very important part of our team. We have a nurse, an adult congenital nurse, but really and truly, the team is really the entire division of cardiology on the adult side as well as the pediatric side. I would add to that, that the congenital CV surgeons that we work with because each of these patients may have something that goes on that involves CV surgery or involves the electrophysiology folks on the adult side, or involves a congenital cath that would be done on the pediatric side. So, it really is a system-wide team effort.

Melanie: Thank you so much for being with us today, Dr. Cribbs. You’re listening to UAB Medcast. For more information on resources available at UAB Medicine, you can go to UABMedicine.org/Physician. That’s UABMedicine.org/Physician. This is Melanie Cole. Thanks so much for listening.