Pulmonary arterial hypertension (PAH) is a rare type of high blood pressure that involves the right side of your heart and the arteries that supply blood to your lungs. These arteries are called the pulmonary arteries.
PAH occurs when your pulmonary arteries thicken or grow rigid and become narrowed inside where blood flows. This makes blood flow more difficult.
For this reason, your heart has to work harder to push blood through your pulmonary arteries. In turn, these arteries aren’t able to carry enough blood to your lungs for adequate air exchange.
When this happens, your body can’t get the oxygen it needs. As a result, you grow tired more easily.
Other symptoms can include:
A study conducted by the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) found that study participants with PAH had the following survival rates:
- 85 percent at 1 year
- 68 percent at 3 years
- 57 percent at 5 years
It’s important to note that survival rates are not universal. These types of statistics can’t predict your own outcome.
Everyone’s outlook is different and may vary widely, depending on the type of PAH you have, other conditions, and treatment choices.
Although PAH has no current cure, it can be treated. Treatment can relieve symptoms and may delay the progression of the condition.
To get the proper treatment, people with PAH are often referred to a specialized pulmonary hypertension center for evaluation and management.
In some cases, a lung transplant can be performed as a form of treatment. Although this doesn’t necessarily improve your outlook, a lung transplant may be beneficial for PAH that doesn’t respond to other types of therapies.
If you have PAH, your doctor will likely use a standard system to rank your “functional status.” This tells your doctor a lot about the PAH’s severity.
The progression of PAH is divided into
In this class, PAH doesn’t limit your usual activities. If you do ordinary physical activities, you don’t develop any symptoms of PAH.
In the second class, PAH only mildly affects your physical activities. You experience no symptoms of PAH at rest. But your usual physical activity may quickly cause symptoms, including breathing problems and chest pain.
The final two functional status classes indicate that PAH is growing progressively worse.
At this point, you have no discomfort when at rest. But it doesn’t take a lot of physical activity to cause symptoms and physical distress.
If you have class IV PAH, you can’t perform physical activities without experiencing severe symptoms. Breathing is labored, even at rest. You may grow tired easily. Small amounts of physical activity can make your symptoms worse.
If you’ve received a PAH diagnosis, it’s important that you remain as physically active as possible while you can.
However, strenuous activity can damage your body. Finding the right way to remain physically active with PAH can be challenging.
Your doctor may recommend supervised cardiopulmonary rehabilitative sessions to help you find the right balance.
Trained healthcare professionals can help you create a program that provides adequate exercise without pushing you beyond what your body can handle.
A PAH diagnosis means that you’ll face some restrictions. For example, most people with PAH shouldn’t lift anything that’s heavy. Heavy lifting can increase blood pressure, which could complicate and even accelerate symptoms.
A number of measures can help you manage pulmonary hypertension, including PAH:
- Attend all medical appointments and seek advice if new symptoms appear or symptoms get worse.
- Have vaccinations to prevent flu and pneumococcal disease.
- Ask about emotional and social support to help manage anxiety and depression.
- Do supervised exercises and remain as active as possible.
- Use supplemental oxygen during airplane flights or at high altitude.
- Avoid general anesthesia and epidurals, if possible.
- Avoid hot tubs and saunas, which may put strain on the lungs or heart.
- Eat a nutritious diet to boost overall health and well-being.
- Avoid smoke. If you smoke, talk with your doctor about setting up a quit plan.
While it’s true that advanced stages of PAH can grow worse with physical activity, having PAH doesn’t mean you should avoid activity entirely. Your doctor can help you understand your limitations and find solutions.
If you’re considering becoming pregnant, talk with your doctor first. Pregnancy may put additional strain on your lungs and heart.
As PAH progresses, daily living can become a challenge, whether due to pain, shortness of breath, concerns about the future, or other factors.
Supportive measures can help you maximize your quality of life at this time.
You may also need the following supportive therapy, depending on your symptoms:
- diuretics in the case of right ventricular failure
- treatment for anemia, iron deficiency, or both
- the use of medications from the endothelin receptor antagonist (ERA) class, such as ambrisentan
As PAH progresses, it will become appropriate to discuss end-of-life care plans with loved ones, caregivers, and healthcare providers. Your healthcare team can help you create the plan you want.
A combination of lifestyle changes, medications, and surgeries may alter the progression of PAH.
Although treatment can’t reverse PAH symptoms, most treatments can add years to your life.
Talk with your doctor about getting the proper treatment for your PAH. They can work with you to delay PAH progression and retain quality of life.