Pulmonary arterial hypertension (PAH) is high blood pressure that occurs in the pulmonary arteries. The pulmonary arteries and capillaries carry blood from the right side of the heart through the lungs.

PAH begins when arteries become blocked, narrowed, or constricted. If the arteries are blocked in any way, blood cannot flow from your heart and through your lungs as easily as it should. In turn, your heart works harder to push blood beyond the blockages. This increased workload raises blood pressure in your arteries and in your heart. Over time, your heart muscle will grow weaker because of the additional work it’s having to do to compensate. Ultimately, this condition can lead to heart failure and eventually death.

PAH is serious and potentially life threatening, but treatment can help slow the disease’s progression and prevent some of the worst complications. A diagnosis may be scary, but you can prepare yourself to deal with it. The more you know about the condition and how it is treated, the greater your chances for living a happy life despite the diagnosis.

How Is Pulmonary Arterial Hypertension Treated?

Treatment for PAH isn’t as simple as a daily pill or a one-off procedure. Your doctor will have to work with you to find a treatment that addresses your symptoms and any causes. Eventually, a treatment may become ineffective, and you’ll have to try a different approach.

Oxygen

Breathing pure oxygen can help increase your blood-oxygen levels. This may help reduce symptoms of PAH that occur when blood-oxygen levels become depleted. To do this, your doctor will help you acquire a home oxygen tank and facemask. Once the tank is opened, oxygen flows through a tube into the mask where you will breathe it in normally.

Medications

Several types of medicines are prescribed to treat PAH.

Vasodilators
These blood vessel dilators open narrowed, restricted blood vessels. For most people with PAH, a once-a-day pill won’t be enough, however. Instead, you may have to wear a pump that continuously administers the medicine through an intravenous catheter. You might also be given an inhaler that allows you to breathe in the medicine at periodic intervals throughout the day.

Erectile Dysfunction Medicines
Drugs prescribed to treat erectile dysfunction can open the blood vessels in the lungs. This increases blood flow, which can increase oxygen levels. For some people, these ED meds may help relieve symptoms of PAH.

Endothelin Receptor Antagonists
These medicines work to reverse the damage caused by endothelin, a substance that builds up and narrows blood vessels .

High-Dose Calcium Channel Blockers
These medicines are designed to relax the muscles that line the blood vessels so blood flow can increase.

Surgeries

Though both types of surgery are typically reserved for severe PAH cases, doctors use the following two procedures to treat PAH.

Atrial Septostomy
During this procedure, a surgeon will relieve some of the pressure on the right side of the heart by creating an opening between the left and right chambers.

Transplantation
For certain people with PAH, a heart or lung transplant may be an option. Not every person with PAH will be a candidate for this procedure, and the risks and side effects of it make it difficult for some others. If your PAH is the result of a congenital heart disease, a combo heart-lung transplant may be necessary.

Treating the Underlying Condition

In the event your PAH is caused by another condition, such as scleroderma or a congenital heart defect, your doctor will have to treat the underlying condition first. Curing or treating the condition or disease that caused the PAH may not ease the hypertension, however. Your doctor will also need to treat the PAH separately.

Make Life Changes

There may be no better time to make dramatic life changes than when you’ve had a dramatic life change. A PAH diagnosis can be difficult to absorb. Your doctor will give you a lot of new instructions. Chief among them should be lifestyle changes. If you are a smoker, it’s time to kick the habit. You should monitor your diet, and reduce your sodium and saturated fat intake. Get regular exercise as your heart and lungs allow.

Though they won’t stop the progression of the disease, these lifestyle changes can help slow it. Being kind to your body is always a positive thing.

Stay on Top of Your Symptoms

Once you’ve been diagnosed, however, keep tabs on your symptoms for your doctor. Be aware of any changes, and report them to your doctor at your next appointment. PAH is progressive—there is no cure. That doesn’t mean you have to succumb to the eventual outcome entirely. Your doctor can help you find treatments for many of the conditions if you work together.