Pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare form of high blood pressure. It occurs in the pulmonary arteries, which flow from your heart and throughout your lungs.

Constricted and narrowed arteries prevent your heart from pumping adequate blood. When constriction occurs, the heart will need to work harder to compensate. This causes the blood pressure in the pulmonary arteries and in the heart to increase dramatically.

As the condition worsens and the pressure becomes greater, you may begin experiencing a variety of signs and symptoms.

It may take months, even years, before the constrictions and narrowing in the arteries become severe enough that noticeable pressure begins to build. For that reason, PAH may progress for several years before symptoms become noticeable.

The symptoms of PAH are also not immediately identifiable as being caused by PAH. In other words, many of the symptoms are common to other conditions. Even worse, you may easily dismiss them, as they typically grow worse gradually, rather than quickly. This makes proper diagnosis more difficult.

The first symptoms of PAH, especially shortness of breath and fatigue, may make you think you’re just out of shape. After all, it’s not uncommon to get out of breath after climbing several sets of stairs, even if you’re physically active every single day. For that reason, many people ignore the symptoms of PAH and let the disease progress without treatment. This makes the condition worse and potentially fatal.

One of the first symptoms of PAH you may notice is shortness of breath. The arteries and blood vessels that carry blood into and through the lungs make breathing possible. The inhale-exhale routine helps you quickly bring in oxygen-rich air and expel oxygen-depleted air. PAH can make that well-choreographed routine more difficult, even labored. Tasks that were once easy — climbing stairs, walking the block, cleaning the house — may become more difficult and leave you breathless quickly.

When your lungs can’t get enough blood to properly function, that means your body and your brain aren’t getting enough oxygen either. Your body needs oxygen to perform all of its tasks. Without it, you can’t carry on with your normal routine. Your legs will tire more quickly after a walk. Your brain and thought processing will seem slower, more labored. In general, you’ll feel tired earlier and more easily.

Lack of oxygen to the brain also increases your risk for dizziness or fainting (syncope).

PAH can cause swelling, or edema, in your ankles, legs, and feet. Swelling occurs when your kidneys are unable to properly flush waste from your body. Fluid retention becomes increasingly likely the longer you have PAH.

Your heart pumps oxygen-rich red blood cells through your body to help fuel all the activities and functions you need. When the amount of oxygen in your red blood cells is inadequate due to PAH, your body parts can’t get the oxygen they need. Low oxygen levels in your skin and lips can cause a bluish color. This condition is called cyanosis.

Increased pressure in the heart makes the heart’s muscles work harder than they should. Over time, these muscles grow weaker. A weakened heart can’t beat as well or as regularly as it once did. Ultimately, this may cause an erratic heartbeat, a racing pulse, or heart palpitations.

Increased blood pressure in the heart and arteries may cause chest pain or pressure. An overworked heart may also cause unusual chest pain or chest pressure.

Each person with PAH will experience a different assortment of symptoms. The severity of the symptoms will also differ from person to person. One person’s journey with having and treating PAH won’t necessarily be helpful to another person because the path with PAH and the treatment options are so individualized.

However, you can draw support from others who have PAH, learn from their experiences, and shape your approach to treating PAH accordingly. Read more about medications used to treat PAH.

Your doctor can help you identify the cause of your symptoms.

Your doctor will ask you to undergo a series of tests to eliminate some of the false positives mentioned above. You’ll likely start with a physical exam, chest X-ray, blood test, electrocardiogram (ECG), and echocardiogram. If they suspect PAH, then another series of tests will be given to accurately diagnose the condition.

Don’t wait if you’re experiencing symptoms of PAH. The longer you wait, the more serious these symptoms may become. Eventually, PAH can limit you from doing all physical activity. Additional symptoms become more likely as the disease progresses.

If you’re experiencing any of these symptoms or suspect you have PAH, make an appointment to see your doctor. Together, you can identify — and treat — this rare type of high blood pressure.