• COVID-19 can be particularly deadly for people with pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH), whose lung function is already compromised.
• Avoiding exposure to the coronavirus by physical distancing is the best way to stay safe.
• Some PH treatments may also be used to treat COVID-19, but it is unknown whether they protect those with PH from the coronavirus.
• Unlike PH flare-ups, COVID-19 usually causes coughing and fevers, as well as fatigue.

Members of myPHteam have voiced concerns about their risks of experiencing serious complications if they contract COVID-19, the disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). In addition, people with pulmonary hypertension or pulmonary arterial hypertension may wonder how to tell the difference between a typical PH flare-up and the symptoms of COVID-19.

To get answers to members’ questions about COVID-19, myPHteam conducted an interview with Dr. Vinicio de Jesus Perez, a staff physician at the Stanford Adult PH Clinic and an associate professor of pulmonary, allergy, and critical care medicine at Stanford University School of Medicine.

Why Is COVID-19 a Serious Concern for People With PH?

According to the U.S. Centers for Disease Control and Prevention (CDC), people of any age with serious underlying health conditions have a higher risk for developing severe illness from coronavirus infections. According to Dr. de Jesus Perez, those with chronic lung disease — including high blood pressure in the pulmonary arteries — may be especially at risk.

“It's no secret that COVID-19 is an infectious disease that has a strong affinity for the respiratory system,” said Dr. de Jesus Perez. ”Patients with chronic lung disorders are already a vulnerable population. Many of our patients live at the edge of maximum reserve of what their heart and lungs can deal with.”

Dr. de Jesus Perez went on to explain how the coronavirus enters the respiratory system, resulting in an inflammatory response that disrupts the body’s access to oxygen. “So, if you put that now in the context of somebody who's got a chronic lung disorder — be it IDF [idiopathic pulmonary fibrosis], COPD [chronic obstructive pulmonary disease], pulmonary hypertension — these patients are already living on the edge, because their systems are already working at max capacity to keep the vital processes going. Well, it's a recipe for disaster. We're very, very anxious about our patients. Because if they're exposed to this, there's a very high proportion of patients that will die.”

Avoiding Exposure to COVID-19

According to an April 29 article in Pulmonary Circulation, “Currently, the most challenging aspect of ongoing care of PAH patients is considering ‘exposure risk’ for patients coming to the clinic or hospital for follow-up and new appointments, including routine laboratory tests.” The goal is to keep people safe when they come in for doctors’ appointments or treatments.

When possible, doctors are rescheduling appointments, procedures, and diagnostic tests. Fortunately, some members of myPHteam have been able to push out testing and procedures to avoid exposure risk. One member reported, “I have a virtual appointment with my PH doctor this Wednesday. We will be rescheduling my heart cath. It had been cancelled due to COVID-19.” Another said, “I saw the pulmonologist yesterday. Due to COVID-19, he's unable to do the tests he wants. He scheduled it three months out.”

Dr. de Jesus Perez emphasized the importance of following physical distancing guidelines. “We are very, very aggressive about encouraging our patients to stay indoors, protect themselves with masks and hand sanitation. Most importantly, stay sheltered in place until things normalize.” He acknowledged that it’s impossible to predict how long the situation will last. “We just don't know when that time is going to be, so [staying home] is our strongest recommendation right now.”

Members of myPHteam understand the need for social distancing from others. As one member said, “I have stayed at home for three months per my doctor's orders. I have been out a couple of times in the last two weeks, but I wear my mask and stay 6 feet away from people.” Another member recognized that it is not easy to stay quarantined, but that it’s worth it. He wrote, “Quarantine is hard sometimes, but the alternative is deadly.”

Dr. de Jesus Perez pointed out how seriously the PH community has taken COVID-19 and met the challenges of physical distancing. “I think the major reason why we haven't seen many PH patients walk through the doors [with COVID-19] is because they are taking our advice. They're staying indoors and observing all the precautions that are in place to keep people from getting the disease.”

Is It a PH Flare-Up or COVID-19?

Researchers have identified a growing list of COVID-19 symptoms in what was initially believed to be primarily a respiratory illness. Some of the symptoms the CDC initially listed as COVID-19 symptoms (particularly shortness of breath) could easily be mistaken for symptoms of PH flare-ups. How can people with PH tell the difference between a flare-up and COVID-19?

Dr. de Jesus Perez described the difference between coronavirus symptoms and PH flare-ups. “I think one of the telltale signs [of COVID-19] is that they are likely to have a significant cough. They're going to have acute fatigue. They can have fevers, and they can feel chest tightness.” In this scenario, he noted, “It is really not subtle, and many patients will immediately call us, get screened, or come to the hospital to be seen acutely.”

In summary, Dr. de Jesus Perez described the situation that will trigger a doctor to suspect coronavirus in someone with PH: “The biggest red flag will be, ‘I was feeling fine. Now I am coughing, I feel this overwhelming fatigue, and I have this fever.’ In the context of the COVID epidemic, that [situation is considered] COVID until proven otherwise.”

Doctors may want to test to confirm the diagnosis. Members of myPHteam have experienced their doctors’ cautious approach firsthand. One member reported, “I had to get tested for COVID-19 because I'm still having breathing issues. ... Doctors want me tested just to be sure. I feel good and sincerely doubt I have it. I also had a chest X-ray to help find out what's causing being unable to breathe during a shower or walking just half a block.”

PH Medications and COVID-19

One question people with PH may have is whether common medications prescribed for pulmonary hypertension might provide protection against the impact of COVID-19. Experts do not yet have enough information about this, and won’t know more until they have had a chance to study COVID-19 and PH in depth and over time.

It is true that certain medications sometimes prescribed for PH or PAH are being studied for use in the treatment of COVID-19. Brigham and Women’s Hospital lists Veletri (Epoprostenol) as one of the potential treatments for hypoxemia (low levels of oxygen) in cases of COVID-19. On May 6, STAT published an article about the use of anticoagulants such as Coumadin (Warfarin). Researchers reported better outcomes when providers added anticoagulants to treatment options for COVID-19 patients.

According to Dr. de Jesus Perez, “There's been discussion in the medical community that some [PH] medications may actually have some benefit since they work as vasodilators, which means they can potentially keep vessels open and help drive blood to areas of the lungs that are still getting oxygenation. On the other hand, this can also be damaging, because you may be driving blood to areas of the lung that are actually not working appropriately. We are very careful to make any assumptions that our patients are being protected by medications.”

Stay Up-to-Date and Connected

Advice from the CDC is changing on a daily or weekly basis. Check the latest updates regularly for changes.

It’s important to stay connected. Speaking on the phone or video chat may not replace hugs and in-person visits, but connecting with friends or loved ones can help minimize feelings of isolation. As always, myPHteam offers a support group more than 27,000 strong, where other people facing PH or PAH are available online.

Do you have more questions about how COVID-19 might impact you and others living with pulmonary hypertension? Ask your questions in the comments below.