Behind the Scenes: Pulmonary Arterial Hypertension Treatment, Research, and RT's Expanding Role

Pulmonary arterial hypertension (PAH) is a critical condition often overlooked in respiratory care. In the first in a series of podcasts about PAH, Brie Soldano, MBA, RRT, RRT-NPS, Respiratory Therapist‑Lead at Nemours Children's Hospital, and Lisa Fuchs, EdD, MHA, RRT, CHWC, FAARC, FNAP, AARC Director of Education, discuss the vital role respiratory therapists play in early detection and intervention. Brie highlights her experiences and actionable insights to improve patient outcomes while underscoring the importance of proactive care in managing PAH, especially in newborns with congenital heart defects. 

AARC thanks Linde and Mallinckrodt for their support of this episode of the AARC Perspectives Podcast. 

Thank you for listening! Learn more at aarc.org 

Transcript

Lisa Fuchs (00:01.125)

Welcome to today's episode of the AARC Perspectives podcast. I am Lisa Fuchs, AARC Director of Education, and have been a practicing RRT and educator for more than 20 years. I am joined by Bree Solondo, a cardiac ICU clinical lead at Nemours Children's Hospital, Delaware. Today, we will discuss pulmonary arterial hypertension (PAH). What is it and how is it treated? This is the first in a series of podcasts that ARC is producing on PAH with support from our corporate sponsors, Linde and Mallinckrodt. Thanks, Bree, for joining us. To begin, Bree, can you tell us a little bit about how your journey to being an RT began and why you have such an interest in the pulmonary arterial hypertension arena?

Brie Soldano (01:00.137)

Thanks for having me. Like many of us in this profession, I thought I would be a nurse. I volunteered at a local hospital. I did all of the prereqs. My first day volunteering involved dropping my brand-new phone in the toilet, watching a patient escape a nurse by throwing his walker quite forcefully at her. And it ended with being invited to assist in perennial care, which is not my cup of tea. I think we can all agree on that.

Following that, I went to Salisbury University. I did all of the prereqs for nursing and I found myself at this kind of impasse of where do I go from here? And I believe it was my nursing advisor who introduced me to Dr. Inslee, which anybody who's gone through that program knows quite well. He is a force of nature, and he walked me through what this profession is, what it does, why it's important, and why it matters.

And he was such an inspiration to me. I made it through their program, graduated, and moved to Phoenix, Arizona, because of adventure. I began my career at Banner  where I had my first NICU pulmonary hypertension baby. And it dawned on me during the care of this patient, just how important it is to know when your patient has pulmonary hypertension and what to do. Because like any baby with pulmonary hypertension, she could change her color rapidly, terrifyingly, sats in the 30s to 20s. And you would bag and deal with 100 % oxygen, and nothing would happen. You had to be patient, and I didn't know that. So, to categorize myself as terrified would be generous, I think. I don't think I handled it well, but my preceptor was amazing and walked me through it, and she was okay. Disclaimer: The patient was fine.

And from there, my career has kind of grown. went to Hershey Medical Center, where I worked across all of its critical care units at the Children's Hospital. Lots of pulmonary hypertension is happening there. It's a level four NICU. And now I work here at Nemours. Again, a level four NICU. The Cardiac Center is well known. They have a fantastic program, and I've really enjoyed working here, learning with all these wonderful people. Our cardiologists are absolutely brilliant. And again,

Brie Soldano (03:23.126)

Pulmonary arterial hypertension is kind of paramount in this unit. And I'm one of the first lines of defense in treating that, especially when they go into crisis mode. That, you're kind.

Lisa Fuchs (03:33.329)

Well, that's incredible. It's incredible, as a respiratory therapist, to be on the front line. And with that, what is PAH, and what types of patients are most often affected?

Brie Soldano (03:47.884)

So, the definition of pulmonary hypertension is a mean pulmonary artery pressure exceeding 20 millimeters of mercury and or a pulmonary vascular resistance greater than or equal to three wood units. Kind of depends on what side of the heart is failing for what you're going to use. But that pulmonary arterial pressure is usually the marker. In my world, patients that are most often affected are persistent pulmonary hypertension of newborns because we do get newborns in the cardiac unit because they also come with cardiac defects a lot of the time, and for me, it's a lot of pulmonary hypertension due to left heart failure and just pulmonary arterial hypertension. Again, it's usually directly linked to congenital heart defects, a lot of trisomy 18s, a lot of VSDs; that kind of patient population is primarily what I deal with.

Lisa Fuchs (04:46.181)

So how do you diagnose this?

Brie Soldano (04:51.133)

Well, thankfully, I'm not in charge of diagnosing it, but it is diagnosed formally with cardiac catheterization. However, signs that we look for to be like, hmm, could be a pulmonary arterial hypertension patient would be cyanosis or blueness, low oxygen saturations that don't respond to oxygen. Again, because this is a blood flow driven issue, not necessarily oxygen deprivation. Tachycardia, fast heart rate.

Again, a number one sign of hypoxia, tachypnea or fast and or labored breathing. And again, especially in children, that's the number one way they try to compensate for the lack of oxygen traveling systemically. But thankfully, I don't have to cardiac cath anybody.

Lisa Fuchs (05:42.971)

So how, what role, this sounds like it's an interdisciplinary role. So what role does the RT play in that interdisciplinary care of the patient?

Brie Soldano (05:56.305)

So first and foremost is awareness, pulmonary hypertension patient. The first thing we're going to know what to do in a crisis is to give them 100 % oxygen, which is not the norm across the CICU and or the NICU and also to have nitric at the ready in our cardiac center, we keep it. Front and center. It is right where I sit because when we need it, we need it yesterday. I usually have it somewhat pre-dressed so I can just throw it in line and get it running because it is the fastest and most direct treatment for pulmonary hypertension crisis. So, I would say knowing how to give them oxygen because it's again, not the norm here in the cardiac center, knowing nitric and knowing it well and just having the awareness really is the biggest thing because a lot of our kids aren't supposed to have a lot of oxygen. It's a little bit of a confusing factor when you're like,

This one's supposed to get 100 % and I push this button? Yes, they are.

Lisa Fuchs (06:59.308)

Is ROP addressed with this high oxygen use?

Brie Soldano (07:03.943)

Yes. So, we really see that in the NICU a lot. Thankfully, we are not getting a ton of newborns in the cardiac center. It's not a completely foreign concept, but in NICU, it becomes this weighing of, is ROP going to be worse than having a pulmonary hypertension crisis? So, we give them what they need, and we try to limit it. So once their SATs are acceptable, backing off oxygen as quickly as we can is definitely one strategy for combating retinopathy of prematurity, but sometimes it's like, you know what, we can do laser eye surgery. It's not the best or the most favorable outcome, but it certainly beats coding a patient. When we're weighing big picture items, it's like, you know what, I'll just, take that chance, I guess.

Lisa Fuchs (07:54.801)

So, is there any other treatment involved? Maybe not from the RT, but in general, how do they treat these babies?

Brie Soldano (08:02.922)

Well, so depending on how it's driven and in the unit for us they can be on diuretics like Lasix again, we're doing a fluid balance because this is a very big plumbing issue. They can get cardiac surgery. Repairing a lot of these kids are VSD kids and the right heart circuit, the pulmonary circuit is lower resistance. A lot of the blood chooses to go there. Sometimes it floods the pulmonary circuit. We have pulmonary hypertension due to too much blood, and then that blood isn't going systemically. So they desat on the pulse ox on one side of their body and not the other, depending on where the shunt is. And you have to balance that. So sometimes we do pulmonary artery banding. Sometimes it's VSD repair. The pulmonary artery band seems counterintuitive. Why would I restrict something that can go into pulmonary hypertensive crisis?

And the answer to that is again, balancing that blood flow. So, you'll get a PA band on a pulmonary hypertension baby and be like, well, this is really bizarre. You must work around it to the best of your ability. And they do, if they need a hundred percent, you're going to give it to them and you're going to try your best not to flood the pulmonary circuit, but they are very, very tricky patients. There are those kids that are like drop of a hat. You go into your day like, please, please, please, please behave for me today. I love you.

If that's what it takes, I'll do it. Keeping them calm. All the things. I'll do anything.

Lisa Fuchs (09:39.079)

They're fine one minute and could rapidly deteriorate in the next, right?

Brie Soldano (09:43.398)

And that's what makes them so scary.

Lisa Fuchs (09:47.015)

So what types of education? We can't educate a small baby, right? But we have those parents that are very nervous. They may be new parents about their baby and everything that's going on. How do you educate the parents or the caregiver of a baby with PAH as an RT?

Brie Soldano (10:11.707)

Right, great distinction because cardiology does a lot of that education. We have, for our cardiac kids, we have diagrams of what their heart defect is. We'll go into detail about what those defects mean for this kid. And if it's pulmonary hypertension as part of it, they're usually going to go home on oxygen. They'll also usually go home on sidenafil, which is a common drug that treats pulmonary hypertension. And sometimes they go home with Lasix. Nursing and RT work together to kind of balance out these are the drugs you're going to give. This is the therapy you're going to have at home. So, a lot of my pulmonary hypertension patients require education on O2 therapy. Here's a nasal cannula goes on this way. Here's an oxygen tank. This is when you're going to use it. Oxygen concentrators are becoming more common at home as well. The most recent one I had went home on CPAP. So we did a lot of education on here's the interface, here's the device, this is how it works, put it together, take it apart, your battery life, things to look out for. So again, those signs of cyanosis, low SATs, tachycardia, tachypnea, labored breathing, and these buddies do come back. And I like to think that part of it is because we've done the education for families. This is the sign that we're going downhill, please come back. And they don't play around. Most of our families are like, yep, we came back.

Brie Soldano (11:35.496)

They were doing weird things and sometimes they're fine and I love it. And sometimes they're not. So, we recently had a pulmonary hypertension baby who also had severe, um, map because which is just weird pulmonary circuitry happening. Um, and transport maybe didn't appreciate how bad his pulmonary hypertension could get. I, it seemed maybe a little bit of an assumption. It seemed that he had screamed the entire way to the hospital. And by the time he got to us, he was in crisis.

He was grayish, he was bluish, we threw on oxygen, we threw on nitric, we ended up having to intubate because he was just spiraling out of control. So that's another one of the big things to stress for these little kids, they just can't handle it. But back to the root of the question, education really involves understanding what to look out for and how their home O2 therapy works and then staying on that regimented drug schedule, whatever it may be.

Lisa Fuchs (12:35.975)

Do they have any kind of like a care navigator? Just, there's a lot of things that they're dealing with and if they need some answers or help, do they have any kind of support?

Brie Soldano (12:49.882)

Yes, usually. So all of our patients and their families get a care coordinator who will walk them through all the things. They also help coordinate us, the caregivers, on giving them education. do, if they're going home with devices or meds that they're not maybe accustomed to, we also do extensive education. And then we do, like, pretend you're at home day, where they do all the meds.

They take apart and reassemble the ventilator, put together their oxygen tank, demonstrate to me that they can open it and close it and understand how to read if it's empty. And then outside of that, they have home care companies. So, we have one that comes into the hospital, brings their home equipment so they can practice while they're still here, and then supports them when they're at home. So, they can call them and say like, hey, this thing is happening. What do I do now? And they will walk them through troubleshooting any equipment stuff. As far as the meds go, they can reach out to their care coordinator and with questions, or sometimes they'll even call our cardiology department. We will chat with families at home, but, you know, it, it really is, it does fall a lot on the family to know their limitations and to be like, I don't think I'm doing this right. Or I don't think what's happening is supposed to be happening. And sometimes that divide is really.

It's a real challenge. It can be very frustrating for all parties involved. And they must learn so much. I teach them about the ventilator. Somebody else comes in and teaches them about the ventilator and maybe uses different verbiage. And therein lies another source of confusion. And they'll come back, and they're like; I took this thing out of line because it was alarming. And the home care company said I could. I'm like, please do not. Please do not do that. 

Lisa Fuchs (14:39.815)

Yeah. So what is the prognosis then for patients who develop PAH?

Brie Soldano (14:50.051)

So, pulmonary arterial hypertension, just as its own subcategory, really depends on the severity. There's such a range. So you can go home and live for years just maintaining your Lasix, Sildenafil, whatever it may be, but it is progressive. So over time, you'll probably add oxygen, and you might add CPAP, and you might add another drug. If you're older and you have late-onset pulmonary arterial hypertension, the risk of morbidity goes up, right? I mean, that's also kind of how aging works, lucky us. But it does increase the risk of morbidity during any kind of procedure or stressful anything. It gives that right heart pulmonary circuit a very fine line to walk for the rest of your life. So, intubations get very scary, surgeries get very scary. That being said, early diagnosis is key. You can do a lot if you know what you're dealing with. Some people just don't. And obviously staying on top of what you're prescribed. Some people are like, well, I feel better. I'm going to stop taking it. And that's never the answer.

Lisa Fuchs (16:06.343)

And early diagnosis, from what you've said, early diagnosis really is multiple eyes and interprofessional education. You as an RT, as you, you know, are trying to apply oxygen that's not working. And it sounds like it's multiple eyes, but you as a frontline RT, you can put the pieces together and collaborate with the physicians, the nurses.

Brie Soldano (16:35.194)

We certainly try to.

Lisa Fuchs (16:35.281)

So, what new research is on the horizon, and does respiratory therapy have a part in that research?

Brie Soldano (16:46.507)

So, to my knowledge, the WHO has extensive research. Lucky for me, I'm not super familiar with it. I've been working here at Nemours with a multidisciplinary team, that we've kind of patched together from nursing, RT, cardiology, cardiac intensivists, and a couple of, I think we have a social worker on that group as well. And it's really just working to captain pulmonary hypertension pathways so that we know a patient has pulmonary hypertension and we can all, as a team, react and treat these patients as quickly as they need to be treated. Because again, there can be a lot of ignorance there. know, somebody shows up, they're like, great, I'm going to bag this patient. And if it's not working, sometimes they move straight to intubation, but sometimes all you have to do is add nitric or increase your FIO2 because in the cardiac center and in NICU, every resuscitation bag is on a blender with a changeable FIO2 and that can make all the difference in the world. So, we've all been kind of educating each other. I've been learning a ton. A lot of the diagrams are maybe over my head, but it's so fascinating, and it's so amazing to hear these people talk and just try to pick up as much as you can as you roll along. You know, this profession is, we're in everything. Our teas have all the tea all the time in every hospital.

So, you know, just to have the opportunity to pick up even more expertise is huge. And we're hoping to build pathways so that people don't have to question what to do next? Call the attending, attending's not answering. You have something to follow to get that kid back to where they need to be.

Lisa Fuchs (18:32.987)

Yeah, that's one thing I think about respiratory therapy. I would say as a profession, would you agree that we're continuous learners, and if we don't know something, we're always ready to step in and learn it.

Brie Soldano (18:46.966)

I certainly like to think so. I've had the honor of training a lot of newer RTs to this unit and they've all been exceptional. I get a lot of questions and then sometimes I don't know the answer, and we get to find it together. I, you know, and that's the other thing working with this team is I have not run into a single situation where somebody was like, wow, you must be really stupid. Everybody's been very gracious.

Brie Soldano (19:13.157)

Learning to read echoes, didn't think that would happen in this lifetime or the next, but here I am trying to differentiate a PDA from an aorta, which is maybe a mark of how smart I might be, but I do. I love this profession for that. And I've met a lot of amazing and interesting people who are all just very driven to do the best by their patients. And I think that's just so cool.

Lisa Fuchs (19:38.459)

Well, Bri, I want to thank you for joining us today for this episode of the AARC Perspectives podcast.

As a member, be sure to subscribe to the AARC perspectives whenever you download or listen to your podcast and keep an eye on your inbox for additional episodes on PAH coming later this year. You can also visit aarc.org and search for pulmonary artery hypertension for additional resources. The AARC thanks Linde and Mallinckrodt for their support of this podcast series.