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What is pulmonary arterial hypertension (PAH)?

High blood pressure in the blood vessels of the lung

Pulmonary hypertension means high blood pressure in the blood vessels that carry blood from the heart to the lungs. Pulmonary arterial hypertension (PAH) is a type of pulmonary hypertension where the high blood pressure in the blood vessels connecting the heart to the lungs causes changes to the blood vessels that make it difficult for the heart to pump enough blood to the lungs. These changes produce a constant state of high blood pressure in the vessels of the lungs.

PAH causes changes in the pulmonary artery^1,2

• The healthy pulmonary artery is open and elastic, allowing blood to flow through easily.
• As PAH changes the pulmonary artery, resistance to blood flow increases.
• In advanced PAH, the pulmonary artery narrows and stiffens from blood vessel wall thickening, scar tissue, and clotting.

PAH causes narrowing of blood vessel walls^*

*not actual sizes

PAH also changes the heart and the way it works^1,2,4

• Over time, extra pressure causes the heart to enlarge and become less flexible.
• As this cycle continues, less blood flows out of the heart and into the lungs, decreasing the body’s supply of oxygen.

An imbalance of natural chemicals may contribute to PAH

3 key pathways may be involved³:

• Endothelin: excess endothelin may cause tightening, excess cell growth, and inflammation.^*3,5
• Prostacyclin: too little prostacyclin may cause tightening, excess cell growth, and blood clots.³
• Nitric oxide: too little nitric oxide may cause tightening of artery walls.³

*Statement is based on observations reported from in vitro or animal trial. The clinical significance in humans is unknown.

What could trigger PAH?

Some causes for the chemical imbalances:

• Genetic susceptibility⁴
• Chemical (fen-phen, cocaine, methamphetamine)⁴
• Illness (congenital heart disease, connective tissue diseases, HIV)^3,4
• Others and unknown³

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