Pulmonary Arterial Hypertension (PAH)
What is pulmonary arterial hypertension
High blood pressure in the lungs is called pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH). PAH is a chronic and life-changing disease that can lead to right heart failure if left untreated.1
Pulmonary arterial hypertension
Although PAH is a rare disease, with an estimated prevalence of 15-50 cases per million, the prevalence of PAH in certain at-risk groups is substantially higher.2
Meet Tina, an Accredo patient living with pulmonary arterial hypertension (PAH). See how her family, friends and Accredo support her and help her live her best life.
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1. https://phassociation.org/patients/aboutph accessed 7/12/2017
2. www.pah-info.com/How_common_is_PAH accesed 7/12/2017
What are the symptoms of pulmonary arterial hypertension
The symptoms for all types of pulmonary hypertension (PAH) may be similar, and symptoms are usually more severe as the disease progresses. Symptoms of PAH may include:
- Chest pain (also called angina pectoris)
- Fainting (also called syncope)
- Loss of energy (also called fatigue)
- Swelling of the arms, legs, ankles or abdomen (also called edema)
- Dry cough
- Raynaud’s phenomenon (chalky white or dusky blue fingers that may be painful and can sometimes be provoked by the cold)
In advanced stages of PAH, minimal activity may produce some or all of these symptoms. Patients in advanced stages may experience irregular heartbeat, a racing pulse, passing out and difficulty breathing at rest. Sometimes these symptoms mean you have another condition, but sometimes, these symptoms mean you have PAH.1
How is it diagnosed
PAH can be difficult to diagnose in a routine medical exam because the most common symptoms of PAH, such as breathlessness, fatigue and dizziness, are also associated with many other conditions. If your doctor suspects that you have PAH, he or she will want to review your medical and family history, perform a physical exam and perform one or more diagnostic tests.
To determine if you have PAH, and what type, your medical team will schedule specialized tests. If your medical team suspects PAH as a result of one or more of the following tests, they will go on to schedule a right-heart catheterization, which is required to confirm diagnosis.
Blood tests check the oxygen levels in the blood, they observe liver and kidney function, and they identify whether the patient has collagen vascular disease, thyroid problems, signs of infection or HIV antibodies. One test, the brain natriuretic peptide, helps to assess the strain on the heart and may also be used to monitor response to treatment.
Chest X-rays can reveal an enlarged right ventricle or pulmonary arteries. Chest X-rays can also show signs of emphysema or scarring (interstitial fibrosis) of the lungs.
An electrocardiogram checks the electrical impulses of the heart. Electrodes are attached to the patient’s skin, and a recording of these impulses is made. However, an ECG alone is not enough to indicate a PAH diagnosis. If your doctor performs an ECG, he or she will also perform one or more additional procedures to identify PAH.
In this procedure, electrodes are placed on the patient’s skin, and a sonogram of the heart is taken. This painless procedure is often used to make a preliminary diagnosis by estimating the pressures in the right heart and assessing how well the heart is functioning. Other heart conditions that produce symptoms similar to PAH may be diagnosed with an echocardiogram. In addition, an echocardiogram may be used to monitor a patient’s condition.
Pulmonary Function Tests
These tests measure how much air your lungs can hold, how much air moves in and out of them and the lungs’ ability to exchange oxygen. These tests may be performed to potentially identify its cause.
Exercise Tolerance Test (Six-Minute Walk Test)
During this test, a patient will be asked to perform an exercise, most commonly a six-minute walk. The purpose is to identify the patient’s exercise tolerance level.
Nuclear Scan (Ventilation/Perfusion Scan or V/Q Scan)
This diagnostic tool tests for blood clots in the lungs by producing a picture of air and blood flow to the lungs. A small dose of radioactive material is breathed in and another small dose is injected via a blood vessel into the lungs. The doctor will review the images that are produced to evaluate the health of the lungs.
The Gold Standard for PAH Diagnosis
If the results of initial tests point to PAH, your doctor will schedule a right-heart catheterization (commonly referred to as a “right heart cath”). Right-heart catheterization is one of the most accurate and useful tests to get a definitive diagnosis for PAH. This is the only test that directly measures the pressure inside the pulmonary arteries, and it should be done in all patients at least once to confirm a patient’s diagnosis of PAH. During the test, doctors insert a catheter (a thin rubber tube) through a large vein in the patient’s groin or neck. They then pass the catheter up into the patient’s heart to measure the blood pressure in the right side of the heart and lungs.
Vasodilator Study (Acute Vasodilator Challenge)
This test is used for patients who have already been diagnosed with PAH to determine how much their pulmonary blood vessels can relax over a brief period of time. Its main purpose is to screen for patients who might respond favorably to calcium channel blockers, a form of medication. The test can also help determine the patient’s prognosis. With a right heart catheter in place, the patient is given drugs that relax the pulmonary arteries. The test drug is given to the patient in higher and higher doses, pausing at each dose to see how the patient reacts. Once a significant response occurs or the side effects become intolerable, the test is considered complete. 2
1. https://phassociation.org/patients/aboutph/symptoms-of-ph/ accessed 7/12/2017
2. Nhttps://phassociation.org/patients/diagnosis/ accessed 7/12/2017
Pulmonary Arterial Hypertension Medications
While there is currently no cure for PAH, different treatment options are available to help you manage your disease and feel better day-to-day depending on your type of PAH. Your doctor may prescribe medication as part of your treatment. The following specialty medications are available at Accredo for the treatment of PAH.
|Adcirca® (tadalafil)||United Therapeutics|
|Adempas® (riociguat)||Bayer Pharmaceuticals|
|Letairis® (ambrisentan)||Gilead Sciences|
|Opsumit® (macitentan)||Actelion Pharmaceuticals US, Inc.|
|Orenitram® (oral treprostinil)||United Therapeutics|
|Remodulin® (intravenous treprostinil)||United Therapeutics|
|Revatio® (sildenafil)||Pfizer Inc.|
|Tracleer® (bosentan)||Actelion Pharmaceuticals US, Inc.|
|Tyvaso® (inhaled treprostinil)||United Therapeutics|
|Uptravi® (selexipag)||Actelion Pharmaceuticals US, Inc.|
|Veletri® (epoprostenol)||Actelion Pharmaceuticals US, Inc.|
|Ventavis® (iloprost)||Actelion Pharmaceuticals US, Inc.|
Financing Your Care
Financial assistance coordination may be available to support your drug costs including manufacturer and community programs. Accredo representatives are available to help you find a program that might work for you 866-344-4874.
Community financial resources
- Caring Voice Coalition (CVC)
- Good Days
- PSI-Patient Services, Inc.
- Pan Foundation (IPF only, not PH)
- Flolan (epoprostenol) GSK manufacture assistance
- Adcirca (tadalafil) United Therapeutics manufacturer assistance
- Adempas (riociguat) Bayer manufacturer assistance
- Letairis (ambrisentan) Gilead manufacturer assistance
- Opsumit (macitentan) Actelion manufacturer assistance
- Orenitram (oral treprostinil) United Therapeutics manufacturer assistance
- Remodulin (intravenous treprostinil) United Therapeutics manufacturer assistance
- Revatio (sildenafil) Pfizer manufacturer assistance
- Tracleer (bosentan) Actelion Pathways
- Tyvaso (inhaled treprostinil) United Therapeutics manufacturer assistance
- Uptravi (selexipag) Actelion Pathways
- Veletri (epoprostenol) Actelion Pathways
- Ventavis (iloprost) Actelion Pathways
Life-saving specialty medication can be expensive. Learn how the Accredo teams help individuals find ways to afford the medication they need to survive in this video.
There are many organizations that support research and advocacy for pulmonary arterial hypertension. See below for a few of those organizations.
- Pulmonary Hypertension Association
- National Heart, Lung, and Blood Institute (NHLBI)
- National Organization of Rare Disorders (NORD)
Meet the Team
Accredo’s PAH care team is dedicated to serving you and we understand the complexity of your condition. Our specialty-trained clinicians are available 24 hours a day, seven days a week, to answer any questions.
Why We Do It
Accredo supports patients with chronic and complex conditions and helps them live their best life. Watch our video to learn why we do what we do for our patients.