Congenital Heart Disease & Pulmonary Hypertension

Podcast Transcript

Announcer:

Welcome to Love Your Heart, brought to you by Cleveland Clinic's Sydell and Arnold Miller Family Heart, Vascular & 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 Joanna Ghobrial, and I specialize in interventional cardiology, as well as adult congenital heart disease. And with me here is Dr. Neal Chaisson, who is my colleague in the congenital pulmonary hypertension clinic. Neal, can you introduce yourself?

Dr. Neal Chaisson:

Yeah, thanks for having me. My name is Neal Chaisson. I am a pulmonary and critical care doctor and I specialize in pulmonary hypertension. And within that, I specialize in congenital heart disease, associated pulmonary hypertension. That's how we ended up working together.

Dr. Joanna Ghobrial:

Neal, you and I work in this very unique clinic that takes care of a small group of patients that are very complex and they have both congenital heart disease, as well as pulmonary hypertension. It's actually part of the American College of Cardiology and the American Heart Association to have this type of multidisciplinary care for this very unique group of patients. That's how we started our clinic and we started about four years ago. Correct?

Dr. Neal Chaisson:

Time flies. But it's something I've been dreaming of for a long time. Prior to you coming, it was something that just wasn't feasible. I think that the advantage of us having this combined clinic is that you can come, whether you live near or far, and you see both of us at the same time, which, in my opinion, fosters better communication, not only with the patients but with each other to make sure that the multidisciplinary plan is unified right at the time that you come and improved efficiency for you is probably a nice little benefit as well.

Dr. Joanna Ghobrial:

Like a two for one.

Dr. Neal Chaisson:

Two for one.

Dr. Joanna Ghobrial:

Up to 10% of patients that have congenital heart disease can develop pulmonary hypertension, and it can be anything from very mild to quite severe pulmonary hypertension. This is elevated pressures in the lung, which can obviously affect the heart as well. Obviously this kind of group of patients requires more care and more medical management and evaluation than normal. Can you tell us what are some of the symptoms that these patients often come to us with?

Dr. Neal Chaisson:

Sure. One of the things about congenital heart disease associated pulmonary hypertension is that congenital heart disease includes a whole constellation of different diseases and plumbing issues, for lack of a better word. But the symptoms in patients is most commonly increasing fatigue, in some cases, increasing swelling of the legs or increasing swelling in the abdomen. A lot of people think that they're gaining weight, but in fact they're absorbing fluid into their abdomen. Increasing shortness of breath. When you're walking even on flat ground or trying to climb stairs, sometimes you'll feel your heart is racing or skipping beats.

Dr. Neal Chaisson:

But in other patients with other types of congenital heart disease, the symptoms don't manifest that way. They occur as lower oxygen saturations. Especially patients who have one ventricle or single ventricle ventilation, they have lower oxygen. They may start to develop headaches in the morning as their blood tries to compensate for that. And that's often the first time that we meet people, those are the things that they tell us.

Dr. Joanna Ghobrial:

I think sometimes also we want to mention that patients with congenital heart defects may not have any symptoms. They may not know they're symptomatic because they've lived with it their whole life.

Dr. Neal Chaisson:

That's true.

Dr. Joanna Ghobrial:

They don't actually realize that until their friends or family will come and tell them, "Hey, you can't keep up with me as much as you used to."

Dr. Neal Chaisson:

I think it's actually one of the big reasons why regular follow-up with your cardiologist after your procedure or even longitudinally while you have congenital heart disease is so important. Because as you mentioned, patients who have congenital heart disease often don't recognize that they may be declining in their functional abilities and sometimes objective measures to show that there's something going wrong on the horizon can actually help you to get the necessary medicines or interventions that you need before it actually becomes an irreversible problem.

Dr. Joanna Ghobrial:

Very true. In clinic, Neal, you and I often will start from the very beginning. We start from scratch. We start figuring out what were they born with, what kind of surgeries or interventions they had when they were babies, what medications have they been on, how did they progress through that whole time. We look at their prior testing and imaging, but also we do kind of our basic type of evaluation to figure out where they're at now. Can you tell us a little bit about our usual congenital pH clinic evaluation?

Dr. Neal Chaisson:

Sure. For every patient that comes into our clinic, Joanna and I meet prior to you coming in and make sure that we have reviewed all of your prior imaging, what your surgeries were. If it's necessary for us to reach out to your doctor about any lingering questions, we'll certainly do that ahead of the appointment. But when you come in, these objective measures that I mentioned before are important to get a baseline in. That is looking at your pulmonary function status. You'll do some breathing tests with us. We do a simple walking test just to see how far you can walk in a very strict set of guidelines so that we can use that as a baseline for whether you're improving or not.

Dr. Neal Chaisson:

We'll get some labs. We'll get an echocardiogram of your heart. And then in some cases with specialized diseases, we will order additional studies to try to help us get to the root of what the problem is so that we make sure that we are a one step ahead of the disease.

Dr. Joanna Ghobrial:

Some of the testing that we may require during the evaluation is a cardiac catheterization where we actually look at the pressures inside the heart and lungs to figure out where you're at, whether you need medications as simple as diuretics, which is the medications that kind of reduce that filling pressure in your heart and lungs, or more advanced medications that treat the pulmonary hypertension. Neal, can you kind of give a broad view of some of the big classes of pulmonary hypertension medications?

Dr. Neal Chaisson:

Depending on the type of congenital heart disease you have really relegates what kind of medicines may work for you. There's three broad classes of pulmonary hypertension medications. I'll say that these medications may not actually be beneficial for every single patient that comes in with pulmonary hypertension. We want to make sure that we match the right patient to the right medicine. But we look at your condition, we look at what the underlying physiology of your condition is, and then we try to match you with the right medicine.

Dr. Neal Chaisson:

The neat thing about pulmonary hypertension is it is a disease that has historically been considered an orphan disease or a rare disease. I really am honored to be a part of the pulmonary hypertension community because there are all kinds of new drugs that are coming out and they are actually probably going to be out in the next one to two years. These whole new pathways that for the last 20 years we just haven't had access to. I think that's a really cool thing about the future of pulmonary hypertension care.

Dr. Joanna Ghobrial:

Absolutely. Some of the congenital defects that we see, Neal and I, in clinic include things like an ASD or a hole in the upper chambers of the heart between the upper chambers of the heart, or a VSD or a ventricular septal defect, the hole and the lower chamber of the heart, as well as a PDA, a patent ductus arteriosus. We've seen patients who have transposition of the great arteries. We've seen patients that have a single ventricle or half of a heart. It's a very broad category of patients that we see in clinic. But one of the neat things is it's actually nice to work together on every patient.

Dr. Joanna Ghobrial:

Because sometimes, for example, with a patient that has an ASD or an atrial septal defect that has elevated pulmonary pressures, we will sometimes actually start with the medication to treat the pulmonary hypertension. And after a while, we can then evaluate whether we can actually close the hole and close that defect and they improve significantly afterwards. We've had several patients with that scenario.

Dr. Neal Chaisson:

Another scenario where I think this multidisciplinary intervention has been really excellent is in patients with Fontan physiology. As you're obviously aware, Fontan patients often develop pulmonary complications. I think that having a pulmonary hypertension specialist from the pulmonary side of things and a cardiologist from the cardiology side of things really helps patients to get holistic care for their entire disease and the interventions that have been done. We've had a lot of success in that area as well.

Dr. Joanna Ghobrial:

Absolutely. I think also I would love for you to share the story of why you actually wanted to specialize in congenital pulmonary hypertension.

Dr. Neal Chaisson:

Sure. Well, my story goes back. I have four kids. My youngest son, who's now eight, almost nine, was born and very unassuming birth. Everything went well until about 48 hours after he was born. Things weren't quite so good after that, and we weren't sure why. Ultimately, he ended up being diagnosed with congenital heart disease and had to have multiple surgeries after. It was really through that experience that I learned about congenital heart disease associated pulmonary hypertension. I had studied pulmonary hypertension, practiced it before, but I'd never had a lot of experience in congenital heart disease.

Dr. Neal Chaisson:

I just really felt passionate about what my son had brought to the table and the opportunities for kids like this and to be able to just serve that community. I credit my son a lot for what I've ended up doing for a lot of my career to this point.

Dr. Joanna Ghobrial:

I think if you come to the Congenital Pulmonary Hypertension Clinic you'll definitely feel that kind of passion that both of us have to take care of the type of patient that requires this multidisciplinary care for sure.

Dr. Neal Chaisson:

Sure. I always say, we'll treat you like I treat my own kid.

Dr. Joanna Ghobrial:

There you go. Thank you, everyone.

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