PAH FAQ

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Answers to frequently asked questions (FAQ) about living with PAH

2. What are the different types of PAH?

The World Health Organization (WHO) recognizes five groups of pulmonary hypertension, the first of which is pulmonary arterial hypertension (PAH). WHO defines three types of PAH:

• Idiopathic pulmonary arterial hypertension (IPAH) – This refers to PAH which occurs at random, without a known cause.¹
• Familial pulmonary arterial hypertension (FPAH) – This type of PAH is passed on through the family, causing PAH to develop over time. It is estimated that the familial form accounts for 6 percent of PAH cases.¹
• Pulmonary arterial hypertension associated with other diseases or conditions (APAH) – Under this category are certain populations at risk for PAH, including those with:
  • Collagen vascular disease – also known as "connective tissue disease," this includes diseases such as scleroderma and lupus.
  • Congenital heart disease
  • Portal hypertension
  • HIV infection
  • History of drug and toxin usage
  • Others – such as thyroid disorders¹

The good news is that research is ongoing and scientists and physicians know more about PAH today than they did 10 years ago. A cure has not yet been found, but progress has been made.²

* The World Health Organization is considered the international authority on health definitions and disease information. To learn more, visit the WHO website at www.who.int.

3. What are some typical symptoms of PAH?*

Some of the typical symptoms of PAH are caused by lack of oxygen and increased stress on the heart. These symptoms may not be obvious at first, but over time they may become more limiting. Without treatment, most patients will continue to experience increasing breathlessness and fatigue.³

Some symptoms of PAH are:

• Dyspnea (breathlessness or shortness of breath) — most common symptom
• Chronic fatigue (feeling tired all the time)
• Dizziness, especially when climbing stairs or when standing up
• Fainting
• Edema (swollen ankles and legs)
• Chest pain, especially during physical activity

* Tracleer and Ventavis do not improve all of these symptoms. Individual results may vary. For more information, consult the full prescribing information or visit the Tracleer and Ventavis websites.

4. Does PAH run in families?

There are different causes for PAH. In Inherited or Familial PAH, the disease is passed down genetically from your parents. Most cases of PAH occur in individuals with no known family history of the disorder. In fact, it is ten times more likely to be diagnosed with PAH because you have an underlying disease or medical condition than it is because you have a family member with PAH.⁴

5. What are endothelin and prostacyclin receptors?

Endothelin and prostacyclin are naturally occurring chemicals in the body.

Normal levels of endothelin help keep arteries open and elastic. Patients with PAH have high levels of endothelin—suggesting that endothelin may play a role in PAH.³ Endothelin receptor antagonists are designed to even out the imbalance of endothelin.

Prostacyclin keeps arteries open and suppresses excess cell growth and clotting. In PAH, production of prostacyclin is impaired, so there is not enough to keep arteries open.³

References: 1. Farber H, et al. Mechanisms of disease: pulmonary arterial hypertension. New Engl J Med. 2004;351:1655-1665 2. Gibbs J. Making a Diagnosis in PAH. Eur Respir Rev. 2007;16:102,8-12. 3. McLaughlin V, McGoon MD. Pulmonary arterial hypertension. Circulation. 2006;114:1417-1431. 4. American Lung Association Web site. Available at: American Lung Association Web site.