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Understanding pulmonary arterial hypertension (PAH)

 
Illustration explaining how PAH is a disease where there is high blood pressure in the arteries of your lungs, which causes the heart to work harder, becoming enlarged and weakened
 

What are the signs and symptoms of PAH?

PAH can lead to a wide range of symptoms that may develop slowly over time. Most people with PAH begin to notice symptoms as the condition progresses.

Symptoms of PAH include:

Symptoms of PAH include Tiredness

Tiredness

Symptoms of PAH include Shortness of Breath

Shortness of Breath

Symptoms of PAH include Chest Pain or Pressure

Chest Pain or Pressure

Symptoms of PAH include Fainting

Fainting

Symptoms of PAH include Dizziness

Dizziness

Symptoms of PAH include Rapid Heartbeat

Rapid Heartbeat

Symptoms of PAH include Swollen Abdomen

Swollen Abdomen

Symptoms of PAH include Swollen Legs and Ankles

Swollen Legs and Ankles

 

How is PAH diagnosed?

Your doctor may perform several tests to see what's happening inside your body:

  • Electrocardiogram (EKG or ECG) icon

    Electrocardiogram (EKG or ECG) to measure the electrical activity of your heart

  • Pulmonary function test (PFT) icon

    Pulmonary function test (PFT) to see how well your lungs are working

  • 6-minute walk test (6MWT) icon

    6-minute walk test (6MWT) to see how far you can walk in 6 minutes

  • Echocardiogram (echo) icon

    Echocardiogram (echo) to check the size and function of your heart

  • Chest x-ray and ventilation perfusion scan (VQ scan) icon

    Chest x-ray and ventilation perfusion scan (VQ scan) to get images of your heart, lungs, and blood vessels

  • Right heart catheterization (RHC) icon

    Right heart catheterization (RHC) to measure the pressure inside your heart and the blood vessels of your lungs

 

How are the effects of PAH on everyday activities measured?

A functional class assessment can help your doctor understand how much PAH affects your everyday activities.

The functional classes are defined as follows:

NYHA Functional Class I No limitations on physical activity. Ordinary physical activity doesn't cause shortness of breath, fatigue, chest pain, or near fainting.
NYHA Functional Class II Some limitations on physical activity. Although comfortable at rest, ordinary activity causes shortness of breath, fatigue, chest pain, or near fainting.
NYHA Functional Class III Clear limitations on physical activity. Although comfortable at rest, even less than ordinary activity causes shortness of breath, fatigue, chest pain, or near fainting.
NYHA Functional Class IV Any level of physical activity is uncomfortable. There may be signs of heart failure, and shortness of breath and/or fatigue may be present even when resting.

NYHA = New York Heart Association

 

How PAH therapies work

Doctors treat PAH using medicines that aim to restore balance among 1 or more of 3 substances that are produced by your lungs: prostacyclin, nitric oxide, and endothelin.
The right amount of each substance helps keep pulmonary arteries open and blood flowing through the lungs.

1 OR MORE OF THE 3 SUBSTANCES ARE OUT OF BALANCE, CAUSING PULMONARY ARTERIES TO NARROW:

Too little prostacyclin
Too little nitric oxide
Too much endothelin

PAH THERAPIES HELP OPEN PULMONARY ARTERIES BY:

Increasing prostacyclin
Increasing nitric oxide
Decreasing endothelin

*Two types of therapies, called phosphodiesterase-5 inhibitors and soluble guanylate cyclase agonists, work in a similar way to nitric oxide to open blood vessels.