Pulmonary arterial hypertension (PAH) is high blood pressure that results from the blood vessels in your lungs becoming narrow.

Blood delivers oxygen throughout the body. To get that oxygen, the heart must first pump the blood through the lungs. Then, the oxygen-rich blood delivers that oxygen to cells in the body.

When the blood vessels in the lungs become narrow due to PAH, the heart works harder to push the blood through the lungs for oxygen. This extra strain weakens the heart over time.

Several factors can cause or contribute to PAH. Although it can be passed down from a family member, it can also develop in a person for no known reason

Although the cause of PAH is unknown, the disease has been known to be passed down from one family member to another.

The term idiopathic isn’t specific to PAH. The word is used to describe any disease that develops for an unknown reason.

Idiopathic PAH refers to cases when the disease develops in patients without prior family history.

Without a lung transplant, yes, it can be. But with early diagnosis and treatment, people with PAH may be able to improve their risk status, which may improve their life expectancy. How quickly PAH progresses will vary based on each individual and how their condition is treated.

That’s why you should tell your healthcare provider about your PAH symptoms, no matter how minor, or if your symptoms change over time. You might be able to take steps to improve how you feel and possibly improve your risk status.

Every case of PAH is unique, and many variables can impact PAH life expectancy. Experts recommend conducting a risk assessment as the best way to estimate life expectancy. Your risk status helps your doctor determine your prognosis and estimate your life expectancy.

If your healthcare provider suspects you have PAH, they may perform many tests to determine if you potentially have PAH or rule it out. To confirm a diagnosis of PAH, you must have a right heart catheterization.

 There are 4 pathways that currently have medications available to treat PAH. Each type of medication works on 1 of these pathways, helping balance a natural substance that promotes healthy blood vessels.

Unfortunately, there currently is no known cure for PAH.

However, PAH studies are continually being conducted, and PAH research is an ongoing endeavor for many dedicated PAH specialists. The PAH community is learning more about the disease and how to better manage and treat it all the time.

PAH symptoms include:

• Fatigue
• Shortness of breath
• Chest pain
• Dizziness
• Lightheadedness, fainting
• Swollen abdomen
• Swollen ankles
• Irregular heartbeat

In most PAH patients, PH gets worse if left untreated. In some PH patients, PAH may remain stable over many months or years.

Whether PAH gets worse in an individual patient depends on many factors.

These include:

• The cause of PAH

• The severity of PAH

• The age of a patient

• The general health of a patient

• The presence of other conditions of the heart, lungs, or body

• What PAH treatment is available to a patient

• How a patient responds to PAH treatment.

Right after diagnosis it is hard to imagine what life with pulmonary hypertension will be like. Over time, many people develop strategies to cope with the challenges of living with PH and lead full, happy lives.
 The key is to listen to your body, respect your limits, and rest when needed. Making small adjustments to your home, routine, and daily activities will allow you to conserve energy while remaining active. Support from other people who understand what it means to live with pulmonary hypertension and who have developed strategies to cope with the disease can also be crucial.

The tests performed to diagnose you will allow your doctor to determine which type of pulmonary hypertension you have and the severity of your illness. They will consider factors such as your functional class (a classification based on how pulmonary hypertension impacts your everyday life), the pressures in your lungs, and how well your heart is functioning to determine which treatment is best suited to your needs.

Your medical team will work with you to establish a personalized treatment plan and adjust it as your symptoms and lung pressures change.

Pulmonary hypertension is a relatively rare disease; most physicians have little or no experience with its diagnosis and treatment. For this reason, referral to a centre specialized in treating PH is encouraged to confirm a diagnosis and ensure that patients receive the best care.

 There are centres specializing in treating pulmonary hypertension (adult and pediatric) and chronic thromboembolic pulmonary hypertension (CTEPH) across Canada. Their experienced medical teams include PH nurses and specialists such as cardiologists, respirologists, and rheumatologists. Some provinces require patients to be followed by a specialist in order to receive coverage for pulmonary hypertension -specific medications.