Disorder overview
ATX is the only national network of pharmacies dedicated to the treatment of pulmonary arterial hypertension.

We’re the only national provider of all FDA-approved therapies for PAH. (Through an exclusive relationship with GlaxoSmithKline, we’re also the only dispenser of Flolan. No other specialty pharmacy has access to this front-line therapy.)

Infused

• Epoprostenol sodium (generic)
• Flolan
• Remodulin

Inhaled

• Ventavis

Oral

• Letairis
• Revatio
• Tracleer

Patients and physicians receive services customized to their needs.

• Experienced clinicians
• An extensive network of pharmacies
• A national customer support center
• Specific support programs
• Reimbursement specialists

Contact Us: 866.FIGHT.PH (866-344-4874)

What is PAH?
Pulmonary arterial hypertension is a rare disorder of the blood vessels in the lungs. The pressure in the pulmonary artery rises above normal levels. Put simply, PAH is high blood pressure in the lungs. It’s a disease that affects people of all ages and backgrounds.

The pulmonary artery carries blood from your heart to the lung. Once enriched with oxygen, this blood flows back to the heart, then on throughout the body, providing oxygen and nutrients to the various tissues and organs. The term pulmonary hypertension means that the pulmonary artery has narrowed, making it difficult for blood to pass from the heart to the lungs. This makes the heart work harder.

Over time, scarring (fibrosis) of the vessels makes them stiffer and thicker, and some may become completely blocked. This extra stress causes the heart to enlarge and become less flexible. Less and less blood is able to circulate from the heart to the lungs and throughout the body.

PAH often escapes early detection. The disease can progress to a late stage before doctors even become aware of it. For this reason, survival rates have generally been low. New treatments are offering promise, however, and many patients now live with their condition for 20 years or more.

The disease takes several forms. When the pulmonary arteries themselves are at fault, the condition is known as category pulmonary arterial hypertension (PAH), or precapillary PH. Often the exact cause can’t be determined and the disease is considered idiopathic (IPAH), meaning unexplained. Since more and more causes are being discovered, the percentage of IPAH cases is actually declining.

PAH can be triggered genetically, and you can inherit a predisposition for it from your parents. If you do, you’ll have about a one in five chance of actually developing the disease. When two or more members of a family have it, the condition is considered familial (FPAH). Inherited causes account for up to 10 percent of all cases.

Genetic mutations aren’t always inherited. Errors in DNA coding can occur outside the womb, often due to environmental factors. When PAH is triggered this way, it’s termed sporadic PAH because it appears seemingly at random and without an identifiable cause.

The list of possible environmental causes is extensive. Although not all researchers agree, antidepressants, birth control pills, estrogen therapy, methamphetamines, cocaine, and various chemotherapy drugs have all been linked to PAH. Tobacco smoking doesn’t seem to be a direct cause, but emphysema caused by smoking is. Culpability has been more firmly established for aminorex, fenfluramine, dexfenfluramine, toxic rapeseed oil, amphetamines, and contamination of L-tryptophan.

PAH is often associated with other conditions, such as HIV, liver disease, collagen vascular disease, and congenital diseases of the heart and lung. The exact relationship between such conditions and PAH isn’t always understood.