Pulmonary Arterial Hypertension

What Is Pulmonary Arterial Hypertension?

Pulmonary hypertension is a life-threatening condition that gets worse over time, but treatments can help your symptoms so you can live better with the disease. It may take some planning, but plenty of people who have it find ways to do all the things they love, just as they did before they were diagnosed.

Having pulmonary arterial hypertension (PAH) means that you have high blood pressure in the arteries that go from your heart to your lungs . It's different from having regular high blood pressure.

With PAH, the tiny arteries in your lungs become narrow or blocked. It's harder for blood to flow through them, and that raises the blood pressure in your lungs. Your heart has to work harder to pump blood through those arteries, and after a while the heart muscle gets weak. Eventually, it can lead to heart failure.

Causes

Sometimes doctors can't find a reason for high blood pressure in the lungs. In that case, the condition is called idiopathic pulmonary hypertension. Genes may play a role in why some people get it.

In other cases, there is another condition that's causing the problem. Any of these illnesses can lead to high blood pressure in your lungs:

• Congestive heart failure
• Blood clots in the lungs
HIV
• Illegal drug use (like cocaine or methamphetamine)
• Liver disease (such as cirrhosis of the liver)
• Lupus, scleroderma, rheumatoid arthritis, and other autoimmune diseases
• A heart defect you're born with
• Lung diseases like emphysema, chronic bronchitis, or pulmonary fibrosis
• Sleep apnea

Symptoms

You may not notice any symptoms for a while. The main one is shortness of breath when you're active. It usually starts slowly and gets worse as time goes on. You may notice that you can't do some of the things you used to without getting winded.

Other symptoms include:

• Chest pain
• Fatigue
• Passing out
• Swelling in your ankles and legs

Getting a Diagnosis

If you have shortness of breath and see your doctor, they will ask you about your medical history. They may also ask you:

• Do you smoke?
• Does anyone in your family have heart or lung disease?
• When did your symptoms start?
• What makes your symptoms better or worse?
• Do your symptoms ever go away?

Your doctor may order tests, including:

Echocardiogram: This ultrasound picture of the beating heart can check blood pressure in the pulmonary arteries.

CT scan: This can show enlarged pulmonary arteries. A CT scan can also spot other problems in the lungs that could cause shortness of breath.

Ventilation-perfusion scan (V/Q scan): This test can help find blood clots that can cause high blood pressure in the lungs.

Electrocardiogram (EKG or ECG): An EKG traces the heart's activity and can show whether the right side of the heart is under strain. That's a warning sign of pulmonary hypertension.

Chest X-ray: An X-ray can show if your arteries or heart are enlarged. Chest X-rays can help find other lung or heart conditions that may be causing the problems.

Exercise testing: Your doctor may want you to run on a treadmill or ride a stationary bike while you are hooked up to a monitor, so they can see any changes in your oxygen levels, heart function, lung pressure, or other things.

Your doctor may also do blood tests to check for HIV and conditions like rheumatoid arthritis or lupus.

If these tests show that you might have pulmonary hypertension, your doctor will need to do a right heart catheterization to be sure. Here's what happens during that test:

• The doctor places a catheter into a large vein, most often the jugular vein in your neck or femoral vein in your leg, and then threads it into the right side of your heart.
• A monitor records the pressures in the right side of the heart and in the pulmonary arteries.
• The doctor may also inject medicines into the catheter to see if the pulmonary arteries are stiff. This is called a vasoreactivity test.

Right heart catheterization is safe. The doctor will give you a sedative and use local anesthesia. You can usually go home the same day, although you will need someone to drive you home.

Questions for Your Doctor

You may want to write down a list of questions before your appointment, so you can make sure you ask your doctor everything you want to. It can also help to have a friend or family member with you to help you get the answers you want.

Some possible questions are:

• What's the best treatment for me?
• How often should I see a doctor for my condition?
• Do I need to see a specialist?
• When should I go to the emergency room?
• Do I need to limit the salt or fluids in my diet?
• What kind of exercise can I do?
• Are there any activities I should stay away from?
• Should I get a pneumonia vaccine and a flu shot?

Treatment

Pulmonary hypertension varies from person to person, so your treatment plan will be specific to your needs. Ask your doctor what your options are and what to expect.

First, your doctor will treat the cause of your condition. For example, if emphysema is causing the problem, you'll need to treat that to improve your pulmonary hypertension.

Most people also get treatment to improve their breathing, which makes it easier to be active and do daily tasks. Oxygen therapy, when you breathe pure oxygen through prongs that fit in your nose, will help if you’re short of breath and have low oxygen levels in your blood. It helps you live longer when you have pulmonary hypertension. If you are at risk for blood clots your doctor will recommend blood thinners. Other medicines improve how well your heart works and keep fluid from building up in your body.

If you have severe pulmonary hypertension, your doctor may prescribe medications called calcium channel blockers. These medicines lower blood pressure in the lungs and the rest of the body.

If calcium channel blockers aren’t enough, your doctor may refer you to a specialized treatment center. You may need more targeted therapies that can open up your narrowed blood vessels. They may be pills, medicines you breathe in, or drugs that are given through an IV. Options include:

• Pills: ambrisentan (Letairis), bocentan (Tracleer), macitentan (Opsumit), riociguat (Adempas), selexipag (Uptravi), sildenafil (Revatio), tadalafil (Adcirca), treprostinil (Orenitram)
• Inhalers: Iloprost tromethamine (Ventavis), treprostinil (Tyvaso)
• IV drugs: epoprostenol sodium (Flolan, Veletri), treprostinil

In more severe cases, or if medicines don't help, your doctor may recommend a lung transplant or a procedure called atrial septostomy. A surgeon creates an opening between the right and left sides of the heart. This surgery can have serious side effects.

Taking Care of Yourself

One of the best things you can do for yourself is to stay active, even if you have shortness of breath. Regular exercise, like taking a walk, will help you breathe better and live better. Talk to your doctor first to find out what kind of exercise is best for you, and how much you should do. Some people may need to use oxygen when they exercise.

Get plenty of rest, too. Pulmonary hypertension makes you tired, so get a good night's sleep and take naps when you need to.

Just like anyone else, it's good for you to eat a healthy diet with lots of fruits, vegetables, and whole grains. That's important for your overall health.

What to Expect

A lot depends on what's causing your pulmonary hypertension. Treating an underlying condition will help you feel better. There's no cure for pulmonary hypertension, but the earlier it's diagnosed, the easier it is to live with.

If you have idiopathic pulmonary hypertension -- the kind where doctors can't find a cause -- your symptoms may get worse over time. But treatment can slow down the progress of the disease and help you live longer.

Remember that each person is different, and there are good treatments available. Work with your doctor to find what's right for you.

Where to Find More Information

The Pulmonary Hypertension Association offers in-depth information on everything from medications to tips on making daily tasks easier. It also has an active online support community.

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