Management of Patients with Pulmonary Valve Disease

Podcast Transcript

Announcer:

Welcome to Cleveland Clinic Cardiac Consult brought to you by the Sydell and Arnold Miller Family Heart, Vascular and Thoracic Institute at Cleveland Clinic.

Joanna Ghobrial, MD:

Hello everyone. My name is Joanna Ghobrial. I'm the director of Adult Congenital Heart Disease, and I specialize in interventional cardiology and adult congenital heart disease, and I want to talk to you today about pulmonary valve disease. Most of the time pulmonary valve disease is going to be a congenital heart defect. There are times where pulmonary valve regurgitation or stenosis can be acquired, such as in the case of rheumatic heart disease, which is quite rare, or in the case of endocarditis. With congenital heart defects, one of the most common ones that you will see in your clinic will be Tetralogy of Fallot. Another one is congenital pulmonary valve stenosis. These are one of the two most common ones. In addition, patients that have different congenital heart defects, post-surgical repair, may have a replaced pulmonary valve, such as in the case, for example, of congenital aortic stenosis in those patients that underwent what's called a Ross procedure, where they replaced the pulmonary valve in the aortic position, and then placed a homograft in the pulmonary position. So now they have prosthetic pulmonary valve disease. So a lot of patients will also present with prosthetic pulmonary valve stenosis or regurgitation.

Joanna Ghobrial, MD:

It's important to note that in patients with congenital heart defects, they often may not know they are symptomatic or limited, and that's why just getting the subjective history from them may be insufficient quite often. And that's also one of the main reasons why we do regular metabolic stress testing on these patients, to have an objective evaluation of how limited they truly are. If you're born with a defect, you may not know what normal is, and that's why the stress test kind of brings out such symptoms and limitations.

Joanna Ghobrial, MD:

With pulmonary valve disease, it's important to actually focus on that on the echocardiogram, which is going to be your very first test to actually see what is the congenital heart defect or what is the acquired pulmonary valve defect that they may have. Pulmonary regurgitation of pulmonary stenosis has a specific way obviously of evaluation on echocardiogram. Sometimes if the echo windows are insufficient or inadequate, you can proceed with MRI or CT. It's also critical that in patients with complex congenital heart disease, that they get referred to an adult congenital heart disease center. Some centers have all the facilities capable of taking care of complex congenital heart disease patients.

Joanna Ghobrial, MD:

These centers also have what is called an ACHA accreditation. So that's the Adult Congenital Heart Association that actually comes in and makes sure that such center has all the capacity and the facility that can manage a complex patient with congenital heart disease. When you refer the patient to an ACHD specialist, we oftentimes will start with the basics, which is EKG and echo, as well as the imaging studies, as well as the stress testing. And then we may proceed to things like cardiac catheterization to evaluate the pulmonary valve a little bit more closely. This can include angiography or just hemodynamics.

Joanna Ghobrial, MD:

And then the next step is to evaluate whether they do need an intervention or not. Sometimes it's a watchful waiting. We just keep an eye on the right ventricle and see, is it hypertrophied? Is it dilated? Is it dysfunctional? Is it time to proceed with an intervention, whether that would be transcatheter or surgical or not. Some of the signs and symptoms that patients would have with pulmonary valve disease can be subtle. Some of them may have shortness of breath, dyspnea on exertion, palpitations, fatigue, as well as chest discomfort. And then sometimes, as I said, they can be completely asymptomatic. And that's why the testing is quite critical to diagnose that.

Joanna Ghobrial, MD:

As far as repair of pulmonary valve disease, there are several ways of actually fixing the pulmonary valve. It depends on the underlying congenital pathology or acquired pathology. For example, in congenital pulmonary valve stenosis, all you'll need to do is balloon valvuloplasty, and that can last quite a bit of time. In other patients that have pulmonary regurgitation, where it has caused at this point, marked RV dilation, or dysfunction, or sometimes LV dysfunction or arrhythmia such as nonsustained VT, then it's definitely time to evaluate what kind of valve replacement can they get. We always try and see if they are a candidate for transcatheter pulmonary valve replacement, and the reason for that is oftentimes these patients have had multiple open heart surgeries and we try and minimize the number of median sternotomies.

Joanna Ghobrial, MD:

We usually will get a specific type of CT that gives us the anatomy of the pulmonary artery and the right ventricular outflow tract to figure out if they are a candidate for transcatheter pulmonary valve replacement. And this can range in size anywhere from 18 to 38 millimeters, which is excellent because now we have the latest technology, ranging from Melody valve to Sapien valve, to Harmony valve and the Alterra stent that allows us to treat such a broad range and broad sizes of pulmonary arteries and RVOTs, thereby limiting the number of median sternotomies these patients get. And then if they are not a candidate for transcatheter pulmonary valve replacement, then we are one of the biggest centers and highest volume for surgical pulmonary valve replacement as well. And that is a very low risk surgery in the hands of the right surgeons, obviously, and we can do surgical pulmonary valve replacement here as well.

Joanna Ghobrial, MD:

After your pulmonary valve replacement, whether transcatheter or surgical, these patients will require regular monitoring, usually by echocardiograms if they have good images on echo. If not, they may require also congenital MRIs. The right ventricle tends to remodel quite well, and we usually see a decrease in size at the six months mark, and we do usually want to repeat imaging at that point in time. But, on a long term basis, these patients will require echocardiography or CMRI or any kind of imaging modality on a yearly basis. They will obviously need infective endocarditis prophylaxis, so antibiotics every time they go see a dentist, and they usually will need aspirin long-term, lifelong as well.

In some patients that get transcatheter pulmonary valve replacement, we may add Plavix for a short period of time, but that is not lifelong or long-term. We are more than happy to help collaborate and co-manage your patient with any pulmonary valve disease, whether adult congenital or acquired pulmonary valve dysfunction. Thank you.

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