Pulmonary arterial hypertension (PAH) is a form of pulmonary hypertension that causes the small arteries in the lungs to thicken and narrow. This can lead to high blood pressure in the lungs.
While there is no cure for PAH, there are many treatment options that can help control symptoms. It’s important to work closely with your doctor to make sure your PAH treatment continues to work.
“Treatments range from drugs to transplants,” says Richard N. Channick, MD, a pulmonologist at UCLA. Your treatment plan may include:
Vasodilators. Blood vessel dilators, called vasodilators, help relax and open up narrowed blood vessels to promote blood flow. Your doctor may give you treatment by intravenous (IV) infusion, under the skin, in pill form, or by inhalation. With inhalation, you will breathe the medicine through a machine called a nebuliser.
Anticoagulant drugs. These medicines can help prevent blood clots from forming. The most common form is warfarin (Coumadin, Jantoven).
Diuretics. These are “water pills” that help get rid of excess fluid in the body.
Digoxin. This drug may help relieve symptoms, make the heart muscle contract more tightly, and slow the heart rate.
Oxygen treatment. With this therapy, you will inhale air that has a higher concentration of oxygen than normal air.
Surgery. In some cases, surgery may be required. There are several types, including pulmonary endarterectomy, pulmonary balloon angioplasty, atrial septostomy, and grafting.
There are other, less commonly used treatments for PAH as well.
“We have this long list of potential drugs that we can choose from. The drugs we choose and how we use them are also a very important topic,” says Channick.
The main goal of treatment is to relieve 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 do you know if your PAH treatment is working?
“It doesn’t matter what you look like on the first day; it’s really how you respond to therapies that will determine how you fare in the long run,” says Channick. There are several ways to measure the success of a person’s PAH treatment:
Functional class. Doctors may simply ask how you feel with your current form of treatment. They’ll have you rate your symptoms on a scale, which experts call functional class.
“The functional class ranges from one to four. One is the [person] it has no activity limits, four means they become symptomatic even at rest or with minimal effort, and two or three are somewhere in between,” says Channick. “Their functional class can help us determine how they will perform and whether they will need of further treatment”.
exercise capacity. “We can measure this using what we call the ‘6 minute walk’ test, or the distance a patient can walk down a corridor in 6 minutes. It’s a pretty solid measure of a patient’s condition,” she says.
More evidence. “Then we have things that we measure more directly, like blood tests, an echocardiogram to see how well the right ventricle is working, or even repeated cardiac catheterization in some cases,” says Channick.
Whatever method your healthcare team uses, it’s important to see your doctor to let them know how you’re doing. Every 3 or 4 months is ideal. Don’t wait until you think your condition has gotten worse. It’s easier for them to determine your level of risk with regular appointments and tests.
“It’s important that you come in regularly, whether you have symptoms or not,” says Channick. “We have many examples of [people] that they 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.
“Do you notice a decrease in your exercise tolerance? For example, things you could do a month ago, you’re not able to do now,” says Channick.
Weight changes are another potential warning sign.
“One of the problems with PAH that doesn’t respond to treatment is fluid retention. It may not always be obvious. People hide liquids in places they can’t even see,” says Channick. “Regular weight checks can help us prevent a real problem or even the need for hospitalization.”
The treatment may also not work well if you notice other symptoms, such as:
- Shortness of breath during normal activities (such as climbing stairs)
- Fatigue
- Dizziness
- Fainting
- Swelling of the ankles, stomach or legs
- Chest pain
- Bluish skin or lips
- A rapid heartbeat
- An irregular heartbeat
- Difficulty breathing even when you are doing nothing
What if PAH treatment doesn’t work?
If one treatment doesn’t control your symptoms, another is likely to.
“More [people] start with two different drugs. Then we do a risk profile, and if they’re not low risk and still have limitations, we often add a third drug to the regimen,” Channick says. “So they could end up with three different treatments for their pulmonary hypertension.”
How you respond helps determine if you’re a good candidate for infusions.
“It is widely believed that infusions can help even when pills or other drugs don’t work. Eventually, if none of that works, we’re looking at a lung transplant,” says Channick.
But this doesn’t happen often.
“Most patients benefit only from current treatments and don’t need an extreme approach,” says Channick. “Before these medical therapies, median survival was less than 3 years with this condition. We now have long-term survivors. We can still do better, but we have certainly come a long way.
