PAH is often not diagnosed early in the course of the disease. Often a diagnosis may be delayed for months or even years. Undiagnosed pulmonary arterial hypertension patients who do not receive treatment may experience more rapid worsening of symptoms or physical condition than patients who are on a doctor-prescribed treatment plan.
PAH is a rare disease that starts with symptoms that are easily confused with those of other conditions, such as asthma or other lung diseases. Thus, patients may be misdiagnosed and receive inappropriate treatment for their symptoms. That's why a series of tests may be performed to rule out other conditions and confirm PAH.
Once PAH symptoms are detected, there is an array of diagnostic testing tools that your healthcare team may want to use. For an accurate diagnosis to be made, certain tests may be performed either to rule out other conditions and/or to confirm PAH. If PAH is suspected, some of the tests your doctor may order are:
The definitive test to make a diagnosis is a right-heart catheterization. This test may also evaluate the severity of PAH by measuring pressure and blood flow changes in the pulmonary arteries and right side of the heart.3 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.3
Your healthcare provider might order other kinds of tests to learn more about your condition or to rule out other diseases.
Once a PAH diagnosis has been made, your healthcare provider may determine your Functional Class. The Functional Class system categorizes the severity of your disease according to your symptoms. Functional Class describes how symptoms, such as shortness of breath, chest pain, or lightheadedness, limit your physical activity.5
The 6-Minute Walk Test is a tool to evaluate exercise capacity. For the test, you walk as far as possible in 6 minutes. Before, during, and after the test, you will be asked to report how short of breath you are. Your healthcare provider may ask you to take the 6-MWT before you begin and several times during your treatment.
VENTAVIS® is a prescription medicine used to treat adults with certain kinds of severe pulmonary arterial hypertension (PAH), a condition in which blood pressure is too high in the blood vessels between the heart and the lungs. VENTAVIS® may improve your ability to exercise and your symptoms for a short time by lowering your blood pressure and opening up the blood vessels in your lungs.
VENTAVIS® has not been studied in children younger than 18 years old.
VENTAVIS® may not be right for you.
Before taking VENTAVIS®, tell your doctor about all of your medical conditions, including if you:
Tell your doctor about all the medicines you take, including prescription and nonprescription medicines, vitamins, and herbal supplements.
VENTAVIS® and other medicines may affect each other causing side effects. VENTAVIS® may affect the way other medicines work, and other medicines may affect how VENTAVIS® works.
Especially tell your doctor if you take:
Know the medicines you take. Keep a list of your medicines and show it to your doctor and pharmacist when you get a new medicine.
VENTAVIS® may cause side effects, including feeling dizzy, lightheaded and faint. If you have any of these side effects, you should stand up slowly when you get out of chairs or bed. Tell your doctor if your fainting gets worse during treatment with VENTAVIS®. Your doctor may need to change your dose or your treatment.
Do not drive a car or operate any tools or machines if dizziness or fainting from low blood pressure is a problem for you.
You may have trouble breathing after taking VENTAVIS® because it may cause the muscles around your airway to tighten (bronchospasm). Get emergency help right away if you have trouble breathing.
Other important side effects of VENTAVIS® include:
Talk to your doctor if you have any side effect that bothers you or that does not go away.
These are not all the possible side effects of VENTAVIS®. For more information, ask your doctor or pharmacist.
Call your healthcare provider for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.
Please see full Prescribing Information and Patient Information for VENTAVIS® and discuss any questions you have with your doctor.
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