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What Causes PAH?

PAH Initiative Ambassadors are patients living with PAH or are caregivers, and they were compensated for their time.

What are some factors that can contribute to PAH?

Pulmonary arterial hypertension (PAH) can be caused by many different things—like a gene mutation that can cause PAH to develop within families. This is called “heritable PAH.” Other factors that can contribute to someone developing PAH include:

  • Heart defects
  • Autoimmune diseases (like lupus, scleroderma, or rheumatoid arthritis)
  • HIV
  • Drug use (including methamphetamine or cocaine)
  • Liver disease (like cirrhosis)

“People don’t understand this disease. I do not look sick. People I’ve known for years know I have PAH but forget how serious it is.” Thekla, PAH Initiative Patient Ambassador

Woman with long brown hair wearing glasses and a red shirt

The cause of PAH is not always known

In many cases, doctors may not be able to find the specific cause of a person’s PAH. This is called idiopathic PAH, which means that PAH has developed for unknown reasons.

How common is PAH?

PAH is rare, so specialized care is needed

PAH prevalence

Only about 1000 new cases of PAH are diagnosed every year in the United States. PAH is more common in women than in men.

PAH is chronic, and it gets worse over time. But life expectancy has been improving for people with PAH. In addition, treatment options and approaches to care, such as improving your risk status, continue to advance.

Because PAH is rare, not all heart specialists (cardiologists) and lung specialists (pulmonologists) are considered specialists in PAH. For this reason, people living with PAH should seek out a specialist.

Find a PAH Specialist
PAH prevalence

What’s the difference between PH and PAH?

You may hear healthcare providers say “PH” or “PAH.” What’s the difference?

Illustrated lungs

Pulmonary hypertension (PH)

PH is a general way to describe high blood pressure in the lungs. PH can have many causes and is associated with many different diseases. Different types of PH are categorized into 5 groups by cause or related condition. Group 1 is pulmonary arterial hypertension (PAH).

Illustrated lungs with highlighted heart

Pulmonary arterial hypertension (PAH)

PAH is a distinct type of PH. PAH refers to high blood pressure that happens for a very specific reason: the blood vessels in your lungs have become narrow. PAH is a serious condition, but with early diagnosis and appropriate treatment, you might be able to improve your outcomes. PAH is also referred to as WHO Group 1 pulmonary hypertension.

Is there a cure for PAH? What can help?

Although there is no cure for PAH, medications and lifestyle changes may help reduce your symptoms and slow down the progression of the disease so that you can do daily activities without as many symptoms.

Doctor discussing PAH with patient

PAH & your right heart

Learn how PAH causes changes in the right side of your heart, the impact this has on how you feel, and what you can do to catch the changes as early as possible.

PAH Changes in the Heart

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