Pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a 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, and 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 include:

Life expectancy for people with PAH

PAH has no cure. It will continue to worsen over time, even with proper treatment. However, untreated PAH tends to worsen much faster.

According to a study conducted by the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL), the patients with PAH who were involved in this study had survival rates of:

  • 85 percent at one year
  • 68 percent at three years
  • 57 percent at five years

Survival rates for PAH may vary widely, depending on the type of PAH you have.

Functional status of PAH

If you have PAH, your doctor will likely use a standard system to rank your “functional status.” This tells your doctor a lot about how severe your PAH has become.

The progression of PAH is divided into four classes. The number assigned to your PAH explains how easily you’re able to perform daily tasks and how much the disease has limited your activities.

Class I

In this class, PAH doesn’t limit your usual activities. If you do ordinary physical activities, you don’t develop any symptoms of PAH.

Class II

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.

Class III

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.

Class IV

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, and even small amounts of physical activity can make your symptoms worse.

Cardiopulmonary rehabilitation programs

If you’ve been diagnosed with PAH, it’s important that you remain as physically active as possible while you can.

However, strenuous activity can be bad for 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.

How to be active with PAH

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 your blood pressure, which could complicate and even accelerate symptoms.

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 the limitations.

Life with PAH

A combination of lifestyle changes, medicines, and surgeries may alter the progression of the disease. Treatment can add years to your life, but it can’t reverse the symptoms from chronic damage caused by PAH.

Even with proper treatment, PAH will grow worse gradually, over time. Within a few years of developing the condition, everyday tasks, such as walking and showering, will likely be more difficult and eventually impossible.

Q & A

Q:

What are the most helpful lifestyle changes I can make to improve my prognosis if I have PAH?

A:

Being realistic about your new limitations is an important aspect to coping with and living with PAH. Your lungs aren’t going to allow you to have the same endurance as they used to, so you need to prioritize the tasks and activities you want and need to accomplish. Understanding how your medications work and when you’re supposed to take them is essential. You’ll also want to understand how foods can interact with your medicines and what limitations your doctor suggests for food and fluid intake. Make sure you don’t run out of your medications and that you store them properly so they remain effective. Compliance with exercise, diet, and lifestyle suggestions from your medical team will help you survive and improve the quality of your day-to-day life. It’s also important to seek mental health services to manage and cope with such a huge change to your life. Support groups, either online or in person, can be extremely beneficial. If you’re using illicit drugs, especially cocaine or other amphetamines, get help to stop. You also want to quit smoking.

Carissa Stephens, RN, CCRN, CPNAnswers represent the opinions of our medical experts. All content is strictly informational and should not be considered medical advice.

Answers represent the opinions of our medical experts. All content is strictly informational and should not be considered medical advice.