NEW YORK, New York — Pulmonary arterial hypertension (PAH) is an extremely rare disease, affecting anywhere between 2.0 people per million in the United States to 7.6 per million in Scotland, according to a 2019 review article in European Respiratory Review.
In fact, PAH is so rare, says Noah Greenspan, DPT, even many pulmonologists don’t know what to look for.
“This isn’t something that’s going to necessarily show up on routine testing,” he said, listing fatigue, shortness of breath, and chest discomfort as some of the most common symptoms of PAH. “Unless you’re looking for it, it’s not going to make itself known.”
He advised: “When a patient comes in reporting shortness of breath and their pulmonary function tests are abnormal, it’s very easy to say, ‘well, what is it most likely to be?’ Most likely COPD or emphysema or asthma or chronic bronchitis. But be open to the idea that those aren’t the only lung conditions, and that if it’s an ILD [interstitial lung disease], then time is really of the essence.”
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Dr. Greenspan, a cardiovascular, pulmonary, and complex medical physical therapist, is the founder and director of the Pulmonary Wellness and Rehabilitation Center in New York City, as well as the nonprofit Pulmonary Wellness Foundation.
He had to shut his midtown Manhattan clinic in March 2020 as the pandemic swept through New York City. The people he was treating there were already among the most vulnerable.
PAH mostly affects women aged between 30 and 60 years old. A diagnosis of PAH is made when average blood pressure leaving the arteries on the right side of the heart exceeds 25 mmHg during rest, and 30 mmHg during exercise.
Problems with pulmonary circulation could lead to other issues, Dr. Greenspan said in a recent interview in New York City. This is why a major goal of any PAH management program is to treat the initial, underlying condition while preventing secondary complications from occurring.
“The earlier we can get on board with this, the earlier we can be aware of what we’re dealing with and get people on optimal medical management from a pharmaceutical perspective and optimal behavioral management, meaning things like cardiopulmonary rehab, good nutrition, managing stress and anxiety, prevention of infection, trying to stave off exacerbations, and trying to keep people in remission indefinitely,” he said. “All these things contribute not only to longer life but to greater quality of life across the board.”
Dr. Greenspan, who has a passion for bonsai trees and colorful tattoos, said that when it comes to PAH, it’s not enough for a doctor to be curious and inquisitive.
“You have to be relentless in terms of finding an explanation for somebody’s symptoms and presentation,” he said. “Pulmonary hypertension is not something that people dabble in. It’s not something that we see every day.”
The good news is that treatments for PAH are getting better all the time, he said. “Whereas several years ago there was almost nothing, right now there are medications that significantly lower pressures and improve heart function.”
These include calcium channel blockers that lower blood pressure by relaxing the muscles that control the arteries. Likewise, blood thinners prevent clots, while diuretics ease the symptoms of PAH by keeping excess fluid out of the body. Other common medications for PAH include bosentan (Tracleer®), ambrisentan (Letairis®), selexipag (Uptravi®), sildenafil (Viagra®), and tadalafil (Cialis®).
As a result, the median survival time from the time of diagnosis used to be 2.5 years, but these days, most patients are living another 7-10 years, and some for as long as 20 years.
While the pandemic has made it much harder for Dr. Greenspan’s patients to focus on proper exercise, nutrition, and stress management, it has also spurred research into respiratory diseases, as well as those with ILDs in general. He added that while pre-existing medical conditions could very well exacerbate the symptoms of COVID-19, “I’ve known of some people with COPD, pulmonary fibrosis, or pulmonary hypertension that did get COVID and survived, and made it through OK.”
In the meantime, Dr. Greenspan, who runs an online “boot camp” for people with respiratory diseases, said that when he does reopen his New York City clinic, it will be with tighter precautions than before the pandemic.
“When we build a new center, we’re going to be taking into account a little bit more social distancing, meaning like spacing the machines a little further apart, maybe having less people at a time in the gym, circulation and ventilation, and purification of the air. But we’re getting there,” he said. “I would be there a lot more quickly if more people would get vaccinated.”
This story was updated on August 4, 2021.