Learn About Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) is one form of a broader condition known as pulmonary hypertension, which means high blood pressure in the lungs. PAH occurs when the very small arteries throughout the lungs narrow in diameter, which increases the resistance to blood flow through the lungs. Over time, the increased blood pressure can damage the heart.
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- Pulmonary arterial hypertension (PAH) is a specific type of pulmonary hypertension where the tiny blood vessels in the lungs become scarred.
- The symptoms of PAH are common to other diseases which makes it more difficult to diagnose.
- PAH is a complex condition that will usually worsen without the right treatment.
What Is Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) is high blood pressure in the lungs. PAH can be present even if you have normal “regular” (systemic) blood pressure because the blood vessels in the lung are very different from the blood vessels in the rest of the body. PAH is a specific type of pulmonary hypertension that is caused by the development of scar tissue in the tiny blood vessels of the lung. This scar tissue blocks the blood flow through the lungs and causes the pressure in those blood vessels to increase.
How Pulmonary Arterial Hypertension Affects Your Body
The heart and the lungs work together to deliver nourishing oxygen-rich blood throughout the body. The left side of the heart receives blood with high oxygen content from the lungs, and pumps it through the body via arteries. Oxygen is taken up by cells of the muscles and organs, and the low oxygen content blood is collected in the veins and returned to the right side of the heart. The right side of the heart pumps this blood back to the lungs where it picks up oxygen again.
Normally, the pressure in the right side of the heart and the blood vessels of the lungs is lower than in the rest of the body. This allows the blood to collect as much oxygen as possible while in the lungs. In someone with PAH, the scarred and narrowed blood vessels in the lungs make it harder for the blood to get through. This forces the right side of the heart to pump harder, under greater pressure. If this increased pressure is not treated, it can cause the right side of the heart to become overworked and possibly fail. This problem can also reduce the ability of the blood to collect enough oxygen to keep the body functioning normally.
How Serious Is Pulmonary Arterial Hypertension?
Pulmonary arterial hypertension is a very serious disease. It is not the same as other, more common forms of pulmonary hypertension, which are usually caused by other underlying diseases of the heart, lungs, or other organs. PAH is a rare condition, affecting about 15-50 people per million in the US. Since patients with PAH are often not diagnosed until their symptoms are severe, they may only have a few years to live unless they get proper treatment. Fortunately, advances in therapies have led to impressive improvements for patients with pulmonary arterial hypertension.
Pulmonary Arterial Hypertension Symptoms, Causes and Risk Factors
What Are the Symptoms of Pulmonary Arterial Hypertension?
The symptoms of pulmonary arterial hypertension are similar to the symptoms often seen in more common diseases, such as asthma, emphysema or chronic obstructive pulmonary disease (COPD), and heart failure. Symptoms include:
- Shortness of breath. At first with movement, but then at rest as the disease worsens.
- Swelling of the feet, legs, belly and neck.
- Chest pain, pounding of the heart and fainting are less common but can occur in very advanced disease.
- Rarely, patients will cough up blood or have a change in their voice.
What Causes Pulmonary Arterial Hypertension?
PAH is characterized by progressive scarring of the tiny blood vessels going to the lungs. A number of diseases and conditions can cause this scarring including: connective tissue disorders like scleroderma and lupus; exposure to certain toxins and drugs, including methamphetamine and cocaine; infections, including HIV and schistosomiasis; cirrhosis of the liver; and congenital heart abnormalities. When the cause of PAH is unknown, it is called idiopathic pulmonary arterial hypertension.
What Are Risk Factors?
You are more likely to get pulmonary arterial hypertension if you are a young female, since the idiopathic form of the disease occurs more often in women of childbearing age than in men. There is an inherited form of PAH, so a family history of the disease may put you at increased risk. Patients with diseases like lupus, scleroderma, cirrhosis of the liver, and HIV infection can develop it as well. Methamphetamine and cocaine use increase the risk for developing this disease.
When to See Your Doctor
If you have shortness of breath that does not improve with therapy for common diseases such as asthma and COPD; fainting or chest pain with exertion; unexplained swelling in your legs or abdomen and profound fatigue; you should contact your health-care professional. If you have been diagnosed with PAH, you should find a specialist who deals with this disease.
Diagnosing and Treating Pulmonary Arterial Hypertension
The treatment for PAH is very complicated and depends on many factors.
What to Expect
The diagnosis of PAH requires numerous tests to determine the type and severity of pulmonary hypertension that you have and treatment is complicated and depends on many factors.
If PAH is suspected, you should expect several tests such as blood tests, breathing tests, X-rays, and possibly procedures to directly measure the blood pressure in the lungs.
How Pulmonary Arterial Hypertension Is Diagnosed
The real key to diagnosis is suspicion of the problem. Diagnosis often requires the help of both a lung and heart specialist (pulmonologist and cardiologist). Patients often have the disease for a while before it is diagnosed. Tests commonly used to diagnose all forms of pulmonary hypertension, including PAH, are:
- Blood tests: Include HIV, thyroid tests, autoimmune disease panels (test for systemic lupus erythematosus, scleroderma, rheumatoid arthritis), liver tests, and blood chemistry tests.
- Electrocardiogram: Shows the electrical activity of the heart, and may help detect abnormalities. Doctors may also be able to detect signs of strain on the right side of the heart.
- Breathing tests (pulmonary function tests): Check for diseases like asthma or emphysema
- 6 minute walk test: Objectively measures how far you can walk and to see if your oxygen levels drop when you exert yourself.
- Echocardiogram: Ultrasound of the heart to check the size and condition of the chambers of the heart. It can also be used to estimate the blood pressure in the pulmonary arteries.
- Right Heart Catheterization: Because this test is invasive, it is not usually performed unless other tests cannot produce a firm diagnosis. It involves inserting a catheter (small tube) into a large vein in either the neck, arm, or groin, and threading it through the right side of the heart and into the pulmonary artery. This allows measurement of the blood pressure in the lungs.
How Pulmonary Arterial Hypertension Is Treated
The treatment of PAH has changed rapidly over the last 15 years. PAH-specific medications come in pill, inhaled, and intravenous (IV)/subcutaneous forms. The medications are often used in a variety of combinations. The treatment of pulmonary arterial hypertension is very dependent on the patient, the severity of the symptoms, the test findings, and even the support that the patient has at home. In addition to specific drugs, patients with PAH are also often treated with anticoagulants (blood thinners) and supplemental oxygen either at night, as needed or continuously. There are also patients with PAH who would benefit from a specific program of pulmonary rehabilitation. All of these potential options should be discussed between the patient and the treating physician. If the disease continues to progress in spite of treatment, your doctor may talk to you about lung transplantation.
Living with Pulmonary Arterial Hypertension
What to Expect
Patients with PAH may have very different lives after they are diagnosed with the disease. The therapies that exist for this disease range from pills that can be taken between one to three times a day to medications that have to be given continuously through an IV line using a pump that is similar to an insulin pump used by diabetic patients. How well a patient responds to medicine is somewhat unpredictable and depends on how advanced the disease is at diagnosis as well as the underlying cause. Patients without treatment usually quickly progress to death over the course of months to years. However, with treatment, survival has improved significantly.
Typically patients will be seen in the doctor’s office at least every 3-4 months at first. In many centers, there will be regular testing such as echocardiograms and 6 minute walk testing. Some centers will obtain yearly right heart catheterizations to assess the impact of therapies on pulmonary pressures and heart function.
Special considerations for patients with PAH need to be made. For example, pregnancy should be avoided at all costs as it represents significant risk to the female patient with PAH and to the fetus. Also, many of the medications used to treat PAH may harm the fetus as it develops. Because PAH is a rare and complex disease, good care for a patient with PAH involves a close relationship with an expert in PAH and support from family and friends.
Managing Pulmonary Arterial Hypertension
To best manage your PAH you should:
- Take your medication exactly as directed.
- Do not stop a medication without consulting with your doctor.
- Take care to never run out of medication.
- Avoid things that can put strain on the lungs and heart. This would include lifestyle choices such as smoking and the use of recreational drugs such as cocaine and amphetamines. There are also potential risks of worsening PAH during the course of normal pregnancy. These issues should be discussed between you and your doctor.
- Since PAH can lead to a form of heart failure, adhere to a low salt diet and limit fluid intake.
- Monitor your weight to recognize fluid retention. If your weight goes up, it is often helpful to let your physician or healthcare provider know, as an adjustment of medications may be needed.
- Develop strategies to help cope with fatigue and shortness of breath.
- Develop a careful exercise program with your healthcare providers.
- Get immunized against flu and pneumonia.
- Talk to your doctor about anxiety and depression, which are common in patients with PAH.
The Lung Association recommends patients and caregivers join our Living with Lung Disease Support Community to connect with others facing this disease. You can also call the Lung Association’s Lung Helpline at 1-800-LUNGUSA to talk to a trained respiratory professional who can help answer your questions and connect you with support.
Learn more at the Pulmonary Hypertension Association.
Questions to Ask Your Doctor about Pulmonary Arterial Hypertension
Making notes before your visit, as well as taking along a trusted family member or friend, can help you through the first appointment with your doctor.
- What is the cause of my PAH?
- How advanced is my disease?
- How often do you treat this problem?
- Do I need to be referred to a specialist in PAH?
- What medicine choices are available to me?
- Why did we choose this particular medication?
- Does this medication have any interactions with other medications that I currently take?
- Are there any types of medications that I should avoid?
- Is an exercise program appropriate for me?
- Would I benefit from pulmonary rehabilitation?
- Are there any new developments in exercise or treatment?
- Is it safe for me to travel?
- What are my options for avoiding pregnancy?
- Will I be able to go back to work?
- Is there a patient support group in this area?
- Where can I find additional reliable information on this disease?
Stephen Mathai, MD, MHS, FCCP
Victor Test, MD, FCCP
Date Last Reviewed