Pulmonary Hypertension (PH) vs Pulmonary Arterial Hypertension (PAH)

Pulmonary hypertension (PH) is a broad term used for any condition in which the blood vessels of the lungs have high pressure. There are many types of PH with many different causes.

The World Health Organization (WHO) is considered an international authority on health definitions and disease information. The goal of the WHO classification system for pulmonary hypertension (PH) is to classify the disease into different groups according to their cause and treatment method. The most recent update to this classification system was created during the 4th World Symposium on PH held in 2008 at Dana Point, California. PH is divided into 5 WHO groups depending on the cause.

The first group, or WHO Group I, is otherwise known as pulmonary arterial hypertension (PAH). PAH specifically affects the pulmonary arteries of the lungs, causing them to tighten and stiffen.

Types of pulmonary arterial hypertension (PAH), WHO Group I¹:

1.1	Idiopathic PAH (IPAH) This refers to PAH that occurs without an apparent cause (no family history or identified risk factor). IPAH used to be referred to as "primary pulmonary hypertension" or PPH, and some older information still uses this term.
1.2	Heritable PAH With this type of PAH, a faulty gene is passed on through the family, causing PAH to develop over time.
1.3	Drug-and toxin-induced PAH Certain drugs and toxins including aminorex, fenfluramine, dexfenfluramine, and toxic rapeseed oil have been associated with the development of PAH.
1.4	PAH associated with other diseases and conditions This category includes PAH associated with: Connective tissue disease (also called collagen vascular disease) This section includes diseases such as systemic scleroderma (SSc), CREST syndrome lupus (CSR), and others. HIV infection Portal hypertension Congenital heart disease

To learn more, visit the WHO website at www.WHO.int

Tracleer is approved for patients with pulmonary arterial hypertension (PAH) WHO Class II, III, IV.

IMPORTANT SAFETY INFORMATION
Because of the risks associated with Tracleer, this medication may be prescribed by your doctor only through the Tracleer Access Program.

Liver damage: Tracleer can cause serious liver damage, including in rare cases liver failure and, in situations with regular liver testing, unexplained liver cirrhosis. So you must have a blood test to check your liver function before you start Tracleer and each month after that. Call your doctor right away if you have any of these symptoms of liver problems: nausea, vomiting, fever, unusual tiredness, abdominal (stomach area) pain, or yellowing of the skin or the whites of your eyes (jaundice).

Serious birth defects: Tracleer is very likely to cause serious birth defects. It is important not to be or to become pregnant while taking Tracleer or one month after stopping treatment and to have a pregnancy test before you start the drug and each month thereafter. It is very important for you to use reliable forms of birth control while taking Tracleer. Hormonal contraceptives should not be used alone because they may not be effective in patients taking Tracleer.

Do not take Tracleer with glyburide because it may increase your chance of liver injury. Do not take Tracleer with cyclosporine-A because it may increase your chance of side effects.

Please see Additional Safety Information.
Please see full Prescribing Information, including BOXED WARNING and Medication Guide.

Learn more about how PAH is diagnosed

REFERENCE:

1. Adapted from Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:S43-S54.