What is PAH?
Pulmonary arterial hypertension (PAH) is a rare but serious condition. It causes the blood pressure in the arteries of your lungs to become too high. The blood pressure rises due to the constriction of your pulmonary arteries, which carry blood from your heart to your lungs to pick up oxygen.
Over time, PAH can lead to other problems. When your pulmonary arteries are constricted or narrowed, your heart must work harder to pump blood to your lungs. Over time, your heart can weaken. This can lead to heart failure.
Symptoms of PAH
The symptoms of PAH are similar to those of other heart and lung conditions. They often include:
- shortness of breath
- dizziness or fainting
- racing pulse
- heart palpitations
Your symptoms will likely get worse as the disease progresses. However, many symptoms are treatable.
Causes of PAH
Changes in the cells that line your pulmonary arteries can cause the artery walls to become stiff and thick. This can reduce or block the flow of blood through your pulmonary arteries. As a result, the blood pressure in those arteries rises.
The underlying cause of PAH varies from one person to another. For example, it can be caused by:
- a genetic mutation
- heart disease or failure of your lower left heart chamber (left ventricle)
- chronic obstructive pulmonary disease (COPD), pulmonary fibrosis, or other lung diseases
- connective tissue disorders: systemic sclerosis, rheumatoid arthritis, and systemic lupus erythematosus (SLE)
- chronic liver diseases
- schistosomiasis, a disease caused by a parasite worm
- blood diseases
- metabolic disorders
- long exposure to high altitudes
- certain drugs or toxins such as some appetite suppressants
In some cases, the cause of your PAH may be unknown.
Diagnosis of PAH
Many common symptoms of PAH are also associated with other health conditions. This can make PAH harder to diagnose. If your doctor suspects you may have PAH, they will likely take your personal and family medical history and conduct a physical exam.
Several diagnostic tests may be used to help confirm the presence, severity, and cause of PAH. These include:
- blood tests
- chest X-rays to examine your pulmonary arteries, lungs, and heart
- electrocardiogram (ECG) or echocardiogram to assess your heart function
- pulmonary function test to assess your lung function
- echocardiography (ultrasound of the heart)
The best and most accurate way to diagnose PAH is right-heart catheterization, in which a catheter is used to measure the blood pressure in your pulmonary arteries. Because this test is invasive, it is often done if the diagnosis of PAH is not determined after many of the tests listed above have been completed.
Treatments for PAH
There’s no known cure for PAH, but your doctor can prescribe treatments to help you manage your symptoms. Treatment options include both lifestyle changes and medical interventions. Your specific treatment plan will depend on the type and severity of PAH you have.
A number of lifestyle changes can help relieve the symptoms of PAH. For many people with PAH, the hardest symptom to manage is lack of energy. That can be difficult to deal with, especially if you previously led a more active lifestyle. To help you conserve your energy for things you love to do, there are some simple strategies.
Make a list of your priorities. The simple truth is that you probably don’t have enough energy to do everything you used to do. Make a list of the things you think should be done. Then prioritize that list and focus on your top priorities. You may find that you can let tasks at the bottom of your list go or enlist someone else to complete them.
Try to work smarter, not harder. When you’re performing a task at the top of your list, think of ways that you can streamline the process. For example, don’t walk back and forth across your home gathering dirty laundry. Instead you could carry a basket and make a single trip.
Don’t wander the grocery store aisles aimlessly. You could write your grocery list in order according to where items are stocked.
It may also help to avoid bending over too much throughout the day. Lots of tasks become easier when you’re able to do them about six inches higher. For example, store items you use frequently on shelves or countertops that are easy to reach. Install a raised toilet seat. Ask a friend or family member to raise your floor-level appliances or low tables by using bricks or cinder blocks. This tip is especially useful for your washer and dryer.
In addition to taking care of yourself physically, it’s also crucial to take care of your emotional health. It’s easy to give in to feelings of fatigue, loneliness, and sadness. To help boost your mood and mental health, save time and energy for activities you love, including social activities. If you don’t feel like going anywhere, invite friends or family to come to you.
Certain medications may also help you treat symptoms of PAH. Depending on your symptoms and the underlying cause of your condition, your doctor may prescribe the following:
- blood thinners, such as warfarin, to help prevent clots and keep your blood flowing as smoothly as possible.
- diuretics to help eliminate excess water from your body, including fluid accumulating in your lungs and lower extremities.
- blood pressure medications, such as calcium channel blockers, to help manage your blood pressure.
- endothelin receptor agonists (ERAs) to help prevent further narrowing of your arteries.
- prostacyclins or other medications to help treat and prevent shortness of breath.
In addition to medications, your doctor may recommend oxygen treatments. They can help to increase the amount of oxygen taken in by your lungs when the blood flow to your lungs is restricted. In certain situations, your doctor may even recommend a lung transplant.
Emergencies can happen to anyone. Being prepared is your first line of defense. Talk to your doctor about potential emergencies that may arise from your PAH. Ask what you should do if they occur.
Don’t be afraid to call 911 or your local emergency number if your symptoms of PAH suddenly worsen. Get medical help immediately if you have any of the following symptoms:
- shortness of breath that’s worse than usual
- coughing up blood or mucus
- chest pain
- black or tarry stool
- high fever
Outlook for PAH
PAH is a serious disease. There’s no known cure for the condition. But as long as you manage your symptoms properly, by making healthy lifestyle changes and following your doctor’s prescribed treatment plan, you can continue to lead a fulfilling life.