Glossary of Terms

This information is provided for educational purposes only and is not meant to be a substitute for the advice of a physician or other healthcare professional. You should not use this information for diagnosing or treating a health problem or disease. You should always consult with your healthcare team for your personal medical needs.

(Quick links by letter)

A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

angina. A type of chest pain or discomfort caused by reduced blood flow to the heart muscle.

angiogram. An x-ray picture of blood vessels that's taken after a dye is injected into blood vessels to make them more visible.

anticoagulants. A class of medications used to prevent blood clotting in the body, particularly in the lungs of patients with PAH.

breathlessness. Short of breath. Also called dyspnea. This is the most common symptom of PAH.

cardiac output. The amount of blood pumped by the heart over a particular period of time, measured in liters per minute (l/min).

congenital heart disease. A problem with the heart's structure and function that a person is born with. One of the most common congenital heart defects is a hole in the wall between the right and left ventricles of the heart. Those with congenital heart disease have an increased risk of developing PAH.

connective tissue disease. Disorders affecting joints (muscles, bones, tendons, cartilage). There is often an autoimmune component. Types associated with PAH include SLE (lupus), scleroderma, and mixed connective tissue disease. Those with connective tissue disease have an increased risk of developing PAH.

digoxin. A drug that helps the heart pump more effectively and is often prescribed along with PAH medication (it's important to note that this drug has no effect on constricted blood vessels).

diuretics. A class of medications that can help reduce fluid in the body and reduce swelling in the feet and legs. Diuretics are often prescribed along with PAH medication.

dyspnea. Shortness of breath or breathlessness; labored breathing. This is the most common symptom of PAH.

Doppler echocardiogram. A test in which ultrasound (sound waves) is used to examine the heart. Doppler is a special part of the ultrasound examination that assesses the direction and velocity of blood flow as it makes its way through and out of the heart.

edema. Swelling caused by fluid retention. For PAH patients, edema is often present in the ankles and lower legs.

electrocardiogram (ECG). A test in which electrodes are placed on the body to record the electrical activity of the heart. ECGs are used to learn the condition of the heart and to diagnose heart disease.

embolism. A blood clot or foreign substance that can block or obstruct a blood vessel, restricting vital blood flow.

endothelin. A chemical made by the body which may cause the smooth muscles in blood vessel walls to tighten up. Excess amounts of endothelin have been found in the blood of PAH patients.

endothelin receptor antagonist (ERA). A medication that may help people with PAH by blocking the effects of the extra endothelin their bodies produce. Tracleer is the first oral endothelin receptor antagonist approved for FC II, III, IV PAH.

hypertension. Abnormally high blood pressure.

idiopathic PAH. This refers to pulmonary hypertension that cannot be attributed to any other cause.

lung scan. A diagnostic test that measures air and blood flow in the lungs and is used to rule out the presence of other lung diseases.

PAH Pathways^®. A patient service and support program that provides patients with access to Actelion's PAH medications, insurance coverage information, financial assistance for eligible patients and Sure Steps™.

portal hypertension. An increase in the pressure within the portal vein (the vein that carries blood from the digestive organs to the liver). Those with portal hypertension have an increased risk of developing PAH.

pulmonary arterial hypertension (PAH). A disease of the vessels that carry blood from the heart to the lungs. PAH is caused by changes in the small blood vessels in the lungs that make it harder for blood to flow through these vessels.

pulmonary artery. The large blood vessel that carries blood from the heart to the lungs.

pulmonary function tests. A group of tests that measure how well your lungs work. Your healthcare provider might order this test to learn more about your condition or to rule out other lung diseases.

pulmonary hypertension (PH). Broad term used for any condition in which the blood vessels of the lungs have high pressure. PH comes in many types with many different causes. Pulmonary Arterial Hypertension (PAH) is one such type.

right heart catheterization. A thin tube with a special tip is inserted into a vein in the neck or groin and threaded into the heart and the pulmonary artery, where it measures pressure. This is the only diagnostic test to confirm PAH.

six-minute walk test. How far a patient can walk in 6 minutes is a measure of exercise capacity that is usually a part of the diagnostic process for PAH. It is also used during follow-up doctor visits to determine progress.

Sure Steps. An education and support program provided by PAH Pathways for Tracleer patients and caregivers. Patients will receive educational materials, one-on-one phone support from Nurse Counselors and online access.

Sure Steps Connect™. An online conference series where Tracleer Sure Steps members can watch PAH experts discuss a variety of PAH- and Tracleer-related topics.

syncope. Fainting. This can be a symptom of PAH, and is often the symptom that gets people to see their healthcare team.

Tracleer Enrollment and Renewal form and HIPAA form. A form patients will complete with their healthcare team to enroll in the Tracleer Access Program (T.A.P.^®) in order to receive Tracleer. You can also enroll in voluntary patient education and support programs by completing the HIPAA form.

warfarin. A medication that makes the blood less likely to clot. Warfarin is more commonly known by the brand name Coumadin (see anticoagulants above).

WHO Functional Class. A measurement of a patient's ability to perform ordinary physical activities, such as walking, without feeling shortness of breath. PAH patients are put into one of four possible classes depending on the severity of their symptoms (the lower the WHO Functional Class, the less severe the PAH). WHO stands for World Health Organization.


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.