PAH Overview

The following is a simple explanation of pulmonary arterial hypertension (PAH) and how it may be affecting your health. Knowing this may help you to better understand prostacyclin therapy and how it works.

  • PAH is high blood pressure in the blood vessels in the lungs
  • In people who have PAH, the blood vessels in the lungs become too narrow
    • This makes it harder for blood that is being pumped to the lungs from the heart to pick up the important oxygen it needs and carry it to the rest of the body
    • It also puts extra stress on the heart

Effect of PAH on the Lungs

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In a healthy person’s lungs, the vessels are wide enough for the blood to flow freely through the lungs to collect oxygen for the rest of the body.

In a person with PAH, the vessels in the lungs become narrowed, making it much more difficult for the blood to flow through them.

Effect of PAH on the Heart

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The heart in a healthy person works efficiently to pump blood to the lungs and throughout the rest of the body.

PAH causes the heart to work harder. Over time, the right side of the heart can become larger and the walls of the heart thicker. These effects can lead to symptoms of PAH.

Symptoms of PAH

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Classifications of PAH

A well-known classification system was created by the New York Heart Association (NYHA) to measure disease severity in patients with PAH.* Patients with these symptoms could benefit from REMODULIN therapy.

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*The World Health Organization (WHO) developed a similar classification system that is also commonly used for patients with PAH.

NYHA Functional Classes

Class I: No symptoms with ordinary physical activity
Class II: Some symptoms with ordinary activity and slight limitation of physical activity
Class III: Symptoms with less than ordinary activity and increased limitation of physical activity
Class IV: Symptoms with any activity, possibly even while at rest

Blood Vessels of the Lungs: A Closer Look at the Effect of PAH

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Healthy Vessel: In healthy vessels, the blood flows normally, allowing oxygen to get to the body as needed.

Affected Vessel: In vessels affected by PAH, the blood flow is restricted, resulting in less oxygen getting to the body.

Severely Affected Vessel: In vessels severely affected by PAH, there is very little blood flow and much less oxygen gets to the body.

Common Pathways

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The levels of prostacyclin, endothelin, and nitric oxide in the body can become unbalanced. When this happens, it can cause the blood vessels to narrow. This can lead to PAH.