Congenital Heart Disease and the Pulmonic Valve

Podcast Transcript

Announcer:

Welcome to Love Your Heart, brought to you by Cleveland Clinic's Sydell & Arnold Miller Family Heart, Vascular and Thoracic Institute. These podcasts will help you learn more about your heart, thoracic and vascular systems, ways to stay healthy, and information about diseases and treatment options. Enjoy.

Dr. Joanna Ghobrial:

Hello everyone. My name is Dr. Joanna Ghobrial. I am the Director of Adult Congenital Heart Disease at Cleveland Clinic. I specialize in adult congenital heart disease as well as interventional cardiology. I want to talk to you today about pulmonary valve disease, which is oftentimes something that you are born with, i.e., a congenital heart defect that can cause an abnormality in your pulmonary valve. There are times when the pulmonary valve can become abnormal, whether leaky or tight as you grow up, and it's not necessarily related to a congenital heart defect. So what are the congenital heart defects that can have the pulmonary valve affected?

Some of these terminologies may sound very medical, but some of you may actually know that because you have it. Things like Tetralogy of Fallot, that's quite common. You can also have congenital pulmonary valve stenosis or congenital pulmonary valve regurgitation. And it also can be affected by the surgery that you undergo for other congenital heart defects. So some patients, for example, who have a tight aortic valve can then undergo a surgical repair that actually uses their pulmonary valve in the aortic position and then their pulmonary valve is replaced and then they end up with prosthetic pulmonary valve disease.

Now what are the symptoms that you can have that tell you “my pulmonary valve is too leaky or too tight”? Too leaky means that the blood that goes through the valve into the lungs actually regurgitates or leaks backwards into the heart. When it's too tight, the blood is having a lot of trouble actually getting into the valve to the lungs. And some people actually become completely asymptomatic. And that is why it is extremely important if you have congenital heart disease to follow up with your heart doctor, specifically if you have an adult congenital heart disease specialist because they understand that the symptoms can be quite subtle. But it's important to at least follow up with your primary care physician or your local heart doctor. And when it's time to be referred to an adult congenital heart disease specialist, you can then be referred to a specialized center such as a ACHA accredited center. So that means that the center is very, very special for your type of heart disease, which is congenital heart disease, and has all the equipment necessary to take care of your heart.

But some of the symptoms other than being asymptomatic, some symptoms can be just fatigue, having shortness of breath with minimal exertion or very little exercise, palpitations, feeling faint, or having a lack of ability to endure heat or standing up for too long. And other symptoms other than that is some chest discomfort or chest pain or chest heaviness. Any of these can be caused by pulmonary valve disease.

So how do you diagnose pulmonary valve disease? One of the first tests that you'll get done when you go see your heart doctor is going to be an EKG and then an echocardiogram. An EKG is just the electricity of the heart. An echocardiogram is an actual ultrasound of your heart that shows us how your heart is functioning. And that is usually the first step to see is your pulmonary valve leaky or is your pulmonary valve tight? And that will also assess importantly, how is your heart reacting to the pulmonary valve leakiness or tightness? Is the heart getting tired? Is it getting bigger? Is it getting thicker? Is it getting weaker? These are all extremely important things to note when we are looking at the ultrasound of your heart. And that tells us also whether it's time to do something about the leaky or tight valve or we can watch and wait for a little longer.

The second test that we may be able to do also in you is a congenital MRI. We can also do a cardiac CT. We can also do a stress test, which is sometimes actually very important in patients with congenital heart disease because one, a lot of you tend to be a bit like warriors and you just fight through it and don't even know that you may be limited or symptomatic. And we actually have to do that on what we call an objective evaluation, of are you limited relative to your peers because you may think you're asymptomatic, but this is a defect that you may have been born with, you've had it all along and you don't know what normal is. So actually doing that kind of stress testing brings out a lot of the limitations that you may not have even noted prior to the stress test.

In addition to that, we also do cardiac catheterization, and that is putting catheters through the arteries and veins to check the pressures in the heart and lungs. And that gives us a very good idea of how leaky or how tight your pulmonary valve is and when we need to intervene on it. It also gives us an idea of how we can intervene on it. And by intervene, I mean fix the valve. And fixing the valve can either be as simple as just ballooning it, and that's a minimally invasive transcatheter through little holes in the arteries and the veins, where we then a place a balloon in your pulmonary valve and then we inflate the balloon to open up a tight valve. It can also entail a transcatheter pulmonary valve replacement, so that way we are adding a catheter through the vein into the heart, and then we open up a brand new valve with brand new leaflets within your pulmonary artery because your initial valve was not functioning properly.

And then lastly, it tells us whether the transcatheter method, which is the one with minimally invasive through little holes in the veins, whether that would not work for you, rather you would need open heart surgery. And that is when we do a median sternotomy with open heart, and then we actually remove your old valve and replace it with brand new valve leaflets. We do not usually place mechanical heart valves in the pulmonary position just because of lower flow and high risk of clotting. So most of the valve replacements are going to be done with bioprosthetic or tissue valves. So either a pig or a cow pericardium, which is the covering of the heart of a pig or cow leaflets. Most people do not have any allergies to that, and most people function quite well, and these valves are fairly long lasting. They can last you up to 20 years, as minimum as 5, and the usual average is 10 to 15.

Both your transcatheter valves as well as your surgical valve that are placed are fairly the same. Again, they are what we call bovine or porcine pericardium, which is that covering of the heart of the cow or covering of the heart of a pig. And you have options of sizes, that depends on your anatomy. That tells us how big a valve we can put and we usually try to place a bigger valve in there for you.

So one of the important options in transcatheter pulmonary valve replacement is that we are able to replace your valve from the size as small as 16 to 18 to as large as 38 millimeters. So we have multiple options of different valve platforms that we can use. So we have the melody valve, we have the Edwards SAPIEN valve, we have the harmony valve, and then we have the Alterra stent within which we can place a SAPIEN valve. And that gives us a very broad range of therapy. In congenital heart disease patients, you've had already probably at least one or two surgeries, and we try and minimize how many open heart surgeries you get through a lifetime. That's extremely important. And that's why the technology of performing transcatheter pulmonary valves is very critical because we then can spare you multiple median sternotomies or open heart surgeries along your lifetime.

The goal is to get you to be as old as possible and as happy and independent as possible. And the way to do that is to be able to give you the valves that function over your lifetime, but limit how many open heart surgeries you get. So the transcatheter technology is very important for us to focus on and be able to have the latest technology available here at Cleveland Clinic.

After you have your valve replaced, first off, if it's transcatheter, you need probably about a week off. If it's surgical, you need a little bit more, usually about anywhere between eight weeks to three months off to kind of recover from that. Long term, you're going to have serial echocardiograms or MRIs or CTs, any kind of imaging modality to look at the heart valve, the pulmonary valve, and see how well it's functioning. You usually would want to be following up with your adult congenital heart disease specialist on a yearly basis if feasible. And echocardiograms specifically, which is kind of the easiest test here on a yearly basis to make sure the valve continues to function well. You do want to be on a medication called aspirin, which a lot of people know very well, and that helps the valve last you longer. It prevents the formation of little, tiny, tiny clots on the valve leaflets that can make the valve not function.

Another very important thing is that if you are going to be getting dental cleanings, you need to be using antibiotics every single time. When you have a bio-prosthetic valve, that's extremely important. So if you have any pulmonary valve disease, whether congenital or something you acquired as you grew up, and whether it's leaky or it's tight, at Cleveland Clinic, we are an ACHA accredited center and we have one of the highest volumes in both pulmonary valve surgeries or transcatheter pulmonary valve replacements. And we're able to take care of you from A to Z. We have all the facilities available to diagnose you, treat you, and make you feel better for sure.

Announcer:

Thank you for listening. We hope you enjoyed the podcast. We welcome your comments and feedback. Please contact us at heart@ccf.org. Like what you heard? Subscribe wherever you get your podcasts, or listen at clevelandclinic.org/loveyourheartpodcast.