While there’s no cure for pulmonary arterial hypertension (PAH), many types of medicines and procedures can ease your symptoms and make your day-to-day life better. Everyone’s condition is different, so your doctor will help you figure out the treatments that will work best for you.
The first step of any plan is to treat the cause of your PAH. For example, if chronic obstructive pulmonary disease (COPD) is the reason you have it, treatment for that problem will improve pulmonary hypertension, too. But some people need more help to improve their breathing and lower the blood pressure in their lungs.
To help you choose a treatment plan, your doctor will see how severe your symptoms are and how much your disease keeps you from being active.
Medicines for PAH
These work in a few ways. Some let blood flow more easily through the arteries in your lungs, and others help your heart and lungs work better. You can take them as pills, medicines that you breathe in, or drugs you get through an IV.
Calcium channel blockers are drugs that lower your blood pressure by relaxing the muscles that control the arteries. Before your doctor prescribes these pills, they’ll see how well they work on your blood vessels during a procedure called a vasodilator test. If it seems that they can help you, you’ll take your first doses in the hospital so your care team can figure out a safe dose.
Digoxin is a daily pill that helps the heart beat more strongly, which can help if the cause of your PAH is heart failure or an irregular heart rhythm. The challenge of this drug is to get the right dose. If you have too much, you could have side effects like nausea, changes in vision, and irregular heartbeats.
Warfarin (Coumadin) thins your blood to keep clots from building up, which can make PAH worse. But it can also make you bruise and bleed easily.
Medicines that keep excess fluid out of the body, called diuretics, can make the heart and lungs work better and ease the symptoms of PAH. You usually take these pills once or twice a day. You may need regular blood tests if you take diuretics to prevent dehydration, low blood pressure, chemical imbalances in the blood, and kidney problems.
Other PAH pills lower blood pressure by keeping blood vessels open or preventing them from narrowing. These include:
- Ambrisentan (Letairis)
- Bosentan (Tracleer)
- Macitentan (Opsumit)
- Sildenafil (Revatio, Viagra)
- Tadalafil (Adcirca, Cialis)
Drugs called vasodilators open up blood vessels, but some have an effect on the body for only a few minutes. To take the most common one, epoprostenol (Flolan, Veletri), you need a small battery-powered pump so you can get a steady dose. The pump delivers medicine through a thin IV tube, and you wear it in a pack on your belt or shoulder. You can expect to spend a few days in the hospital to get the pump set up and to learn how to use it.
Other vasodilators are medicines that you inhale, like iloprost (Ventavis) and treprostinil (Tyvaso). They go straight to your lungs and can quickly relieve shortness of breath. To take them, you’ll get a nebulizer, a machine that vaporizes these medications and lets you breathe them in. Iloprost can be given six to nine times a day. Treprostinil can be given four times a day and can also be given orally or by injection.
Some people with PAH need oxygen therapy to get enough oxygen in their blood. You breathe it through a face mask or prongs that fit in your nose. It’s especially helpful for people who also have sleep apnea or who live at high altitudes. Some people with PAH eventually need oxygen therapy around the clock.
If you have severe PAH or medications don’t help your symptoms, your doctor may recommend one of two types of operations:
Atrial septostomy: A surgeon makes an opening between the upper chambers of the left and right sides of your heart to lower the pressure on one side. This procedure can have serious side effects, so doctors don’t often recommend it.
Lung and heart transplants: Doctors recommend them for people who have tried drugs to treat their disease without success and whose condition is getting worse. The people who get lung or heart-lung transplants most often are those who have a severe lung disease that’s causing their PAH.
A transplant can improve your life, but it’s serious surgery. It can take several months to recover completely. Though you may not need PAH medications, you’ll need to take drugs to keep your body from rejecting the new organs, probably for the rest of your life. You’ll want to talk to your doctor about the balance of benefits and risks before you go ahead with this treatment.