Jean M Elwing, MD - phaware® interview 532

ICU & You: What Happens When Pulmonary Hypertension Turns Critical?

Pulmonary hypertension is already a complex condition, but what happens when it becomes life-threatening? Dr. Jean Elwing breaks down what patients and families can expect when someone with PH ends up in the ICU.

My name is Dr. Jean Elwing, and I'm a professor of medicine, and I'm the director of the Pulmonary Hypertension Program at the University of Cincinnati, and I've been there about two decades now, really focused the entire time on the care of patients with pulmonary hypertension and related illnesses. Over that time, we've built a large practice of pulmonary vascular disease-related conditions and are trying to do our best to improve the lives of people we take care of.

So today I want to talk to you about how we approach a patient with pulmonary hypertension that's in a critical situation in the intensive care unit and how we manage the medications that are very helpful to you as an outpatient, but how we do assess and how we use those medications inpatient when people are very sick.

So there's multiple situations that can bring a patient into the ICU. They could rapidly worsen in terms of their right heart function. If they would have an arrhythmia, irregular heartbeat, or a blood clot or something would change dramatically with their medications, or they could gradually be worsening over time, or they could be a new patient that had never been diagnosed before. Each one of those scenarios we treat a little bit differently, but the thing we can't forget is the origin of the problem is the elevated pulmonary pressures. So once we figure out why they're elevated and why that individual has right heart stress and strain and going into right heart failure, then we can intervene.

In patients who have progressive pulmonary arterial hypertension or patients who are newly diagnosed, the main focus should be addressing the pulmonary vascular changes and trying to unload or relieve the stress of the right heart. And we do that through our pulmonary vasodilators.

Each individual patient will be assessed based on their specific needs, but in general, we try our best to initiate medications that relieve that pulmonary vascular resistance as soon as we can. Oftentimes we start with things we can get to the patient very quickly like an inhaled therapy such as nitric oxide or inhaled epiprostenol. And then we take a few minutes, we look at our situation, we look at that individual patient and then decide is this a person that should be given infusion therapy, continuous IV medications to help reduce that stress on the right heart? And in most circumstances, that is what we do when patients are critically ill with pulmonary arterial hypertension and right heart failure.

We also ask the other members of our team to help us make sure we address all the issues that could be contributing and anything else that could be worsening the situation like low oxygen. We would give you oxygen in a way that would raise the levels to a point where it no longer is causing stress on the right heart. And sometimes that's through a nasal cannula, sometimes it's through a non-invasive machine like a CPAP, and sometimes it requires patients to have a tube in their throat to be intubated and put on mechanical ventilation. So we have to figure out what need that individual patient has and then address it the best way we know how to take care of them.

The other thing we'd look at is how much fluid they have in their body. Is it a low fluid state or a high fluid state? Most of the time when patients are in right heart failure, they have extra fluid and we try our best not to give additional fluid and then gradually try to help the person get rid of extra fluid through medicines called diuretics.

We also have members of the team that could help with mechanical support. That is in our most severe situations, we look to people that could help us offload the work of the right heart by a machine, and that's usually ECMO. So it's a circuit that helps circulate blood, give oxygen, and get blood to the right places in the body to give the body a chance to heal. Now, that's not an option for everyone, but in rare circumstances when the medications are working but not fast enough, we have to have a bridge to give the body enough time to heal and hopefully recover from that serious situation.

No one part of this equation will get the person better. It has to be a multiple intervention all at the same time working together as a team to get the body to be able to get back to the point where you can breathe independently without extra oxygen, without a machine, and get the medications up to a level where your body can heal enough to be able to go back home or go to a place where you'd have additional time for rehab.

So it is a very complicated situation. We try to avoid patients getting sick to the point where they need the ICU, but this is why we have an ICU. So we can rescue people in circumstances that are very dire. So of course we never want a patient to be faced with a situation where they're critically ill, but we always want you to be prepared. This is something that we talk to our patients about oftentimes when they're first diagnosed. Then as you're on medications that are complicated and sometimes infusion therapies for years, we may not revisit that, but we want to remind everyone who's involved, the caretaker, the patient, the support individuals that we want to be ready. So be prepared in terms of having medications and your go-pack ready if you would have a situation where you'd need to go to the hospital quickly.

And if you're away from your home or you're in a remote place where there's not experts in pulmonary hypertension, then make sure that doctor that sees you and the person that does the intake when you get to the emergency room knows you're special, you have special needs, and you can't be treated like everyone else. You have to be treated in a way that takes care of the underlying problem, maybe an injury, maybe appendicitis, but in the context of your pulmonary hypertension, we manage things very differently. We change how we treat you with different medications. We also change how much fluid we might give you. So those are really important things, I think, to remember and to have on your list to be ready for anything and everything because that's how life goes. The minute you think things are quiet, something comes up where you might need some extra attention.

We see each patient as an individual. Pulmonary hypertension has so many different faces and so many different levels of severity. So usually the first thing that happens when you come to the hospital and you're sick and you're seen in the emergency room, the ER staff is very aware of the complexities of pulmonary hypertension and they oftentimes call a consult to the pulmonary hypertension team, which may be a cardiologist in one center or a pulmonary in another center, and they get them involved and they look how the patient is doing.

If you're the individual patient being seen, they'll assess how your heart's doing with an echocardiogram. They may do labs to look how the kidneys are functioning and labs to look at stress and strain on the heart with an NT-proBNP or a BNP. They'll look at blood pressure, heart rate, and urine output and try to figure out what is exactly happening with the level of risk you're at for having worsening symptoms or for worsening right heart failure. And then we'll decide are we having a problem with medication? Was there an interruption in medication? Do we need to go up on medication or do we have to start something totally different? So really depends on what's happening.=

And then of course we go through all the things that could trigger the pulmonary hypertension to be less well-controlled, meaning we think about thyroid disease, we think about infection, we think about medication interruption or anything that could set off the fine balance that you as a patient are in on a routine basis.

And then once you're admitted to the hospital, you would be admitted to the location that can take care of patients with pulmonary arterial hypertension or pulmonary hypertension of other sorts. In that location we hope to be able to deliver care that's very sensitive to your needs and take that into account when we choose different therapies that we give you for whatever is triggering the episode of worsening pulmonary hypertension.

One very common thing that triggers pulmonary hypertension worsening is irregular heartbeats, fast heartbeats, or what we call arrhythmias. In that circumstance, we would give you medicine oftentimes to help control that heart rhythm, but we choose specific ones that fit the needs of pulmonary hypertension patients. We would interact with our cardiology colleagues that focus on arrhythmias so we can do our best to control that, because controlling that arrhythmia many times will help you to be able to recover from that worsening event.

Another thing that frequently gets patients in trouble with pulmonary hypertension is infection, and so we're very mindful of that. We try to treat infection early and treat it in a way that you tolerate, meaning not too much fluid and be very cautious about the oxygen changes that may occur in circumstances like pneumonias. So it's really complicated when you get sick and need to come to the hospital, but it really takes a team approach and to really hone in on the cause of the issue and make sure we support the pulmonary hypertension in the meantime.

Of course, we always want the happy ending to every hospitalization, but sometimes things don't go that way. And sometimes there's things that are not recoverable easily with our routine medications. And as I mentioned, sometimes we look to a mechanical support with ECMO to support patients, but not everybody's a candidate for that because of various reasons.

And so one thing we always have in the back of our mind is could you be a transplant candidate if you're really sick with pulmonary arterial hypertension and right heart failure in the ICU? And if so, that's a conversation we would have with you. The things we pay attention to very carefully when patients are sick in the ICU and we're thinking about transplant are a lot about your history and what might make you a optimal transplant candidate or some things may limit that option for patients. And so we look at things like social support, history of other medical illness, age, also weight plays a role, and if you're even interested in that.

And if you are and you're the right type of person for a transplant evaluation, we would contact our transplant team and colleagues and see if this is the right time to complete that evaluation. Sometimes they would like us to continue to support, try to get you better before you are seen in the transplant center. And sometimes they want to start the evaluation right away.

Some centers have a transplant team right there with them. In my case, I do not. I work with outside facilities for transfer, so we would work with another hospital system and then it would be a transfer to that location. So it really depends where you are, what your interests are, and what your history is to determine if transplant might be the next best step.

When pulmonary hypertension patients develop a severe illness and they require urgent evaluation, we require a higher level of care to be delivered to the patient because of the complexities of pulmonary hypertension, the right heart function, and the medications that patients are on with pulmonary hypertension. So this is a circumstance where we have to be very mindful. We have to make sure all the team members are aware of the history of pulmonary hypertension, the specific medications the patient is on, because any piece of the puzzle that's left out will result in the patient with pulmonary hypertension not doing as well as we would like them to.

So it is a very complicated situation. We want to take it very seriously. We want patients to be ready for these situations, and we want to work together with a team, including the ICU physicians, the ER physicians, the surgeons if we need them, the cardiologists and the pulmonologists to get the best outcome.

My name is Dr. Jean Elwing, and I'm aware that my patients are rare.

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