Caitlin Whyte (Host): Pulmonary hypertension is a condition that affects the blood vessels in the lungs, developing when the blood pressure in your lungs is higher than normal. With us today for a deeper dive into pulmonary hypertension is Dr. Karla Cruz Morel, a Board Certified Pulmonary Medicine Physician and Director of the Pulmonary and Critical Care Hypertension Program at PRISMA Health.

 This is Flourish, a podcast brought to you by PRISMA Health. I'm Caitlin White.

 Doctor, we did a brief description in the intro, but can you tell us more about what pulmonary hypertension is?

Karla Cruz Morel, MD: Pulmonary hypertension is a type of high blood pressure that affects the vessels of your lungs. It is very different from hypertension, which is usually what we hear the most, when patients have high blood pressure, they take a blood pressure cuff and they can can measure it at home and they take medications for that.

This is completely different. This is when the pressures are elevated in those arteries of the lungs. And it usually happens because the blood vessels in the lungs can narrow, making it very hard for the heart to pump through them, and so that increases the pressures in the lungs and put a lot of strain in the right side of the heart, leading to right heart failure if you leave it untreated.

As of the cause, there are many different causes of pulmonary hypertension, and management differs very differently depending on what type of pulmonary hypertension you have. Because this is a very complex disease, having an accurate diagnosis, is very necessary to appropriately treat your type of pulmonary hypertension.

So, a lot of diseases can lead to pulmonary hypertension, some of which, patients may be very familiar with. The most common type of pulmonary hypertension is caused by left heart disease. So patients with heart failure or patients that have problems with their valve, like mitral stenosis or aortic regurgitation, those types can lead to pulmonary hypertension as well. And the treatment is very different from those that are primarily affecting the vessels of the lungs. Other common diseases that may cause this are connective tissue disease, such as scleroderma, lupus, and those can lead to narrowing of the vessels and cause pulmonary hypertension as well. Patients with liver disease, like cirrhosis, They can have pulmonary hypertension that is very different from those with other types of pulmonary hypertension.

Also patients that have developed pulmonary embolism in the past, there's a small chances that they can develop chronic thromboembolic disease, which is a type of chronic clot. It's more of a scar tissue in the vessels of the lungs that can cause that narrowing. And so it can lead to pulmonary hypertension. The treatment for those patients is very different than the ones mentioned before, and they may require a certain type of surgery or intervention to be cured. And therefore, that is why it's very important to accurately diagnose this disease so that we can provide appropriate treatment for patients.

Host: Wonderful answer, Doctor. Well diving deeper into this, who would you say is at risk for pulmonary hypertension? Is it something that's inherited?

Karla Cruz Morel, MD: It can be. If you have a family history of pulmonary hypertension and you have undergone genetic testing and it has shown that there's genetic proof of that, it can be passed through family, but that is a very complex conversation that needs a genetic counseling in those cases. And that is very rare as well.

So I would say patients that are higher risk for pulmonary are those with connective tissue disease. Scleroderma, as I mentioned, is very high risk, so those patients need to be screened frequently to see if they are developing pulmonary hypertension. Other patients that are also at risk are those that have methamphetamine use and FENFEN use in the past. Those were considered diet pills, very famous in the 70s. And that's where the epidemic for pulmonary hypertension happened. And more awareness was given to the right side of the heart. And patients that have liver disease, lung disease like COPD, interstitial lung disease; those patients are at higher risk for pulmonary hypertension.

Host: Gotcha. And how do you know if you have pulmonary hypertension? Are there any signs?

Karla Cruz Morel, MD: Most patients may present with fatigue, shortness of breath when they walk, especially when they are going on inclines and when they're using mostly their upper body, like doing their hair or trying to find things in above cabinets, bending over.

And some other patients may have palpitations, dizziness, or feeling like fainting, but those signs when patients are fainting are very dangerous, and that means the disease is probably more advanced, so they need to look for help immediately. But again, because those symptoms and signs are so unspecific, most patients take a lot of time to be diagnosed because of the lack of awareness in the community of this disease, and they get diagnosed with other diseases like asthma

and other lung diseases before they get the right diagnosis. So that is why I think it's important for doctors in the community to be aware that pulmonary hypertension is one of the causes for dyspnea or palpitations, so that these patients can be diagnosed earlier. There are statistics that says that patients that have pulmonary hypertension takes about three years and about more than three different specialists before they get the right diagnosis.

Host: Mm, but that leads me into my next question. Why is pulmonary hypertension dangerous?

Karla Cruz Morel, MD: First of all, it's not a curable disease and it's progressive. The second is and most important is that it can lead to right heart failure and death from right heart failure. The way I put it to my patients is, when the blood has been used by the body, it goes to the right side of the heart.

The right side of the heart has the function of pumping blood through the lungs, so that it can get oxygen and once it has gotten oxygen, it goes to the left side of the heart and gets pumped to the body. Imagine, that the right side of the heart pumps the blood through these pipes that are called pulmonary vessels, and if those are narrow, when you have a pump and pipes, and the pipes get narrow, you have a lot of pressure, and the pump can fail.

Right, so if you're not pumping enough blood through your lungs, if things don't go forward, it will go backwards and patients will start having swelling of the legs. They may start feeling fullness in their abdomen. And so if you're not pumping enough blood through the lungs to get enough oxygen, then you're not going to be able to perform the activities that you usually do because of shortness of breath.

You're not getting enough blood through the body with this disease.

Host: Well, how is pulmonary hypertension then treated? And we touched on this already, but what happens if it goes untreated, then?

Karla Cruz Morel, MD: So pulmonary hypertension, regardless of the cause, have a very poor prognosis if not treated. When you have pulmonary hypertension and it is related to narrowing of the vessels, the treatment may vary depending on the specific type of pulmonary hypertension you have, as well as the severity of the disease, and the individual patient.

For pulmonary arterial hypertension in general, we use medications that are called vasodilator, and most of the time we prefer a combination of therapy that can target different pathways so that we can provide better response for the patients. And those medications, what they do is that they open up the vessels of the lungs, they relax those vessels and they improve the flow of blood through the lungs so that the patient can have more blood going through the lungs and the body and can help with the shortness of breath and their cardiac function, right?

Now, patients that have pulmonary hypertension due to chronic thromboembolic disease; those are caused by organized blood clots in the pulmonary vessels. Those may require a surgical intervention to remove those clots and restore the normal blood flow of the lungs. The management is different for patients, even in patients with chronic thrombolic disease, there's different type of surgery that can restore that blood flow, so it is very important to characterize those patients well and refer them as, as soon as possible.

Other treatments that we use for pulmonary hypertension are very commonly used in other disease such as heart failure and they are diuretics to reduce the fluid retentions and relieve the symptoms of swelling and shortness of breath. Oxygen is very important for these patients as well because low oxygen can increase the pressures in the lungs.

And so I always tell my patients, think of oxygen if you need it, as medicine, especially with all the stigma patients may have around the use of oxygen. I think it's very important for them to know that if you need it for pulmonary hypertension, it is a medication.

And then pulmonary rehabilitation. Most patients that are suffering from a lung disease and heart disease, they get deconditioned. They stop doing what they used to do. It is very reasonable for these to be afraid because they are having significant symptoms, but as this happens and they stop doing things, they get very deconditioned, and so pulmonary rehabilitation and exercise is a big part of their recovery and trying to get them to where they want to be.

 So, I just want to close with the management of pulmonary hypertension should be very individualized depending on the type of PH, the severity of the patient's symptoms, and the other patient's overall health status. So we need to collaborate very closely with the patients and other healthcare providers to develop a very comprehensive and individualized plan for each patient so that we can get them to where they need to be.

Host: Wonderful. Well, Doctor, is there anything we can do to reverse pulmonary hypertension naturally then?

Karla Cruz Morel, MD: Unfortunately not. This is not a curable disease, although there's a lot of research going on trying to find a cure. It is a progressive disease. Now newer medications are being developed. And we have hoped that patients can live longer with these medications, but ultimately if the patient is not doing well on these therapies that we use, and they are progressive, the options would be either lung transplant, or, you know, if they're not a candidate for lung transplant, then considering other palliative measures, but there's no natural treatment.

And this is a very good question because some patients that I have met in the past, they're usually very healthy before, these patients are young and they try to find natural treatments because they're not used to taking all these medications, so their lifestyle changes drastically.

 And this is a great question. No, there is no natural treatment for these patients.

Host: All right. Well, let's talk about diet and lifestyle here as we wrap up. What food should you avoid if you have pulmonary hypertension?

Karla Cruz Morel, MD: This is a very interesting question. I think I would say people should be very cognizant of try not to eat processed food and lean more towards vegetables and more natural foods. I would say the most common one that I tell people to stop eating is salt. Patients that have pulmonary hypertension, they tend to retain retain fluid.

And salt, if you ingest in a high quantity and that doesn't take long, okay, because most processed food and canned foods and even frozen foods, they have a lot of salt already, more so than what we need to consume on a daily basis. And so salt makes us retain more fluid. And that is not good if your body itself cannot handle it because you have heart problems.

So low salt and eating as natural food as possible, trying to avoid processed food, those are the most important thing.

Host: Wonderful. Well, Doctor, can you live a quote unquote normal life with pulmonary hypertension? How does that work?

Karla Cruz Morel, MD: That is a great question. It depends on how sick you are when you get diagnosed and the severity of the disease by the time of diagnosis. It would also depend on the stamina of the patients. If they're younger, if in their comorbidities, if they have other diseases that also is affecting their lifestyle, you know, patients with connective tissue disease, they may have other problems with their joints, can cause a lot of chronic pain and difficulty walking or, it really depends on those factors. I do have patients that are living a normal life after they get treated. Quote unquote normal, it's difficult because some of these therapies are given continuous through a port or continuous infusion so your life is going to change.

But I think what is most important for the patients is to have these conversations about what are your expectations? Redefine those expectations. Sometimes it's needed. I tell my patients when they come to me and they said, I want to run a marathon.

I said, well, have you ever run a marathon before?And if the answer is no, it's probably, I won't be able to get them there. But it is, very important to have the talk of what would make their life meaningful. And if I can get them there, I have done my job, right? So it might not be that they have a normal life where they are hiking and traveling all the time, but they may be able to paint or garden or take care of their grandkids and kids.

So again, it all depends, but we can get them to a better point in life where they can enjoy and find purpose.

Host: Well, on that note, let's talk about travel as well. Can you travel by plane if you have pulmonary hypertension?

Karla Cruz Morel, MD: Again, the answer is, it depends. It will depend on how much oxygen, the patients need. So, we know that high altitude can can exacerbate, the pulmonary pressures go up because the oxygens tend to go down, so if the patient is in very low oxygen demands and we can get them through that travel with oxygen, it is okay.

And if the destination is not a very high altitude destination, that is also a consideration to have. We don't want these patients to go into exacerbation of the pulmonary hypertension and heart failure because they are going to a destination that involves high altitude and that it will pose a risk for them. It all depends. So those are great questions to ask your provider.

Host: Of course, of course, always referring to our personal providers. Thank you for that. And my last question here today, Doctor, what do you want listeners to know about pulmonary hypertension that we haven't already talked about?

Karla Cruz Morel, MD: I think the most important thing is that if you have pulmonary hypertension or if your doctors are suspecting it because of a test that you have had that is suggestive of pulmonary hypertension; that you go to a pulmonary hypertension center of excellence. Because this is such a complex disease and as we have talked in this podcast, the management will really depend on what type you have and the severity of the illness.

So, because it is so complex and the management will differ, broadly, the current guidelines recommend that patients need to be treated through a pulmonary hypertension center of excellence. And PRISMA Health is the only pulmonary hypertension accredited center in the Midlands, one out of three in South Carolina.

I know that most people pulmonary or cardiology, there's a lot of questions about who should manage these patients, but a specificity on that is recommended in the guidance that you must go to a center of excellence. And we are very proud of providing that service here at PRISMA Health.

Host: Well, just a great, in depth, informative conversation today. Thank you so much for joining us, Doctor, on the show and for all the work that you do.

For more information and other podcasts just like this one, head on over to prismahealth.org/flourish. This has been Flourish, a podcast brought to you by PRISMA Health. I'm Caitlin White. Thank you for listening.