Pulmonary arterial hypertension (PAH) is a form of pulmonary hypertension that causes the small arteries of your lungs to thicken and narrow. This can lead to high blood pressure in your lungs.
While there’s no cure for PAH, there are many treatment options that can help control your symptoms. It’s important to work closely with the doctor to ensure your PAH treatment continues to work.
“Treatments range from medication all the way up to transplantation,” says Richard N. Channick, MD, a pulmonologist at UCLA. Your treatment plan might include:
Vasodilators. Blood vessel dilators, called vasodilators, help relax and open your narrowed blood vessels to help blood flow. Your doctor may give you treatment through an intravenous (IV) infusion, under the skin, as a pill, or through inhalation. With inhalation, you’ll breathe in the medication through a machine called a nebulizer.
Anticoagulant medications. These drugs can help prevent blood clots. The most common form is warfarin (Coumadin, Jantoven).
Diuretics. These are “water pills” that help get rid of extra fluid in your body.
Digoxin. This medication can help ease your symptoms, strengthen your heart muscle contractions, and slow down your heart rate.
Oxygen treatment. With this therapy, you’ll inhale air that has a higher concentration of oxygen than normal air.
Surgery. In some cases, you may need surgery. There are a few different types, including pulmonary endarterectomy, balloon pulmonary angioplasty, atrial septostomy, and transplant.
There are other treatments less commonly used for PAH as well.
“We have this big list of potential medications that we can choose from. Which medications we choose and how we use them is also a very important topic,” Channick says.
The main goal of treatment is to ease symptoms and slow the progression of your condition. If your PAH seems to be getting worse, you may need to explore new treatment options.
How Can You Tell if Your PAH Treatment Is Effective?
“It doesn’t matter as much how you are at day one; it’s really how you’re responding to therapies that will determine how you’ll do long term,” Channick says. There are a few different ways to measure the success of someone’s PAH treatment:
Functional class. Doctors may simply ask how you’re feeling with the current form of treatment. They’ll have you rate your symptoms on a scale, which experts refer to as a functional class.
“The functional class ranges from one to four. One being the [person] has no limitations to activity, four means they get symptomatic even at rest or with minimal exertion, and two or three being in between,” Channick says. “Their functional class can help us determine how they’re going to do and whether they need additional therapy.”
Exercise capacity. “We can measure that using what we call the ‘6-minute walk’ test, or how far a patient can walk up and down a hallway in 6 minutes. It’s a pretty strong measure of how a patient is doing,” he says.
Other tests. “Then we have things that we measure more directly, such as blood tests, an echocardiogram to look at how the right ventricle is functioning, or in some cases, even doing a repeat heart catheterization,” says Channick.
No matter which method your care team uses, it’s important to check in with your doctor to let them know how you’re doing. Every 3 to 4 months is ideal. Don’t wait until you think your condition has gotten worse. It’s easier for them to determine your risk level with regular appointments and tests.
“It’s important that you come in regularly, regardless of the presence or absence of symptoms,” says Channick. “We have many examples of [people] who felt like they were doing pretty well, but maybe they weren’t doing as well as they thought.”
Symptoms don’t always tell the whole story, but it’s still important to pay attention to how you’re feeling.
“Are you noticing a decrease in you exercise tolerance? For example, things you could do a month ago, you’re now no longer able to do,” Channick says.
Weight changes are another potential warning sign.
“One of the problems with PAH that isn’t responding to treatment is fluid retention. It may not always be apparent. People hide fluid in places they can’t even see,” Channick says. “Getting regular weight checks may help us prevent a real problem or even a need for hospitalization.”
Your treatment may also not be working well if you notice other symptoms, like:
- Shortness of breath with normal activities (like going up the stairs)
- Swelling in your ankles, belly, or legs
- Chest pain
- Bluish skin or lips
- A racing heartbeat
- An irregular heartbeat
- Trouble breathing even when you’re not doing anything
What Happens if Your PAH Treatment Doesn’t Work?
If one treatment doesn’t control your symptoms, it’s likely something else will.
“Most [people] start on two different medications. Then, we do risk profiling, and if they’re not at a low risk, and they still have limitations, then we’ll often add a third drug to the regimen,” Channick says. “So they may end up on three different treatments for their pulmonary hypertension.”
How you respond helps determine whether you’re a good fit for infusions.
“It’s generally thought that the infusions can help even when the pills or the other medicines aren’t working. Ultimately, if none of that is working, then we consider lung transplantation,” Channick says.
But that doesn’t happen often.
“The majority of patients benefit from just the current therapies and don’t need an extreme approach,” Channick says. “Before these medical therapies, the average survival was less than 3 years with this condition. Now, we have long-term survivors. We still can do better, but we’ve certainly come a long way.”