Patient education: Pulmonary hypertension (The Basics)

What is pulmonary hypertension? — Pulmonary hypertension ("PH") means high blood pressure in the blood vessels that carry blood to the lungs. When this happens, the heart has to work harder. This causes symptoms like trouble breathing and feeling very tired. It can happen to adults and children.

What are the symptoms of PH? — The early symptoms include:

●Trouble breathing when moving around

●Feeling very tired

Over time, you might get other symptoms, including:

●Swelling of the legs and feet

●Chest pain

●Fainting

●Lack of appetite

●Belly pain

●Ankle swelling

Rare symptoms include:

●Cough

●Spitting up blood

Will I need tests? — Yes. Which tests you get depend on your symptoms and any other health problems you might have. First, you might get an:

●Echocardiogram (also called an "echo") – This test uses sound waves to create pictures of your heart.

You might also need other tests to find what might be causing your PH. These include:

●Imaging tests, such as a chest X-ray or CT scan – Imaging tests create pictures of the inside of the body.

●Electrocardiogram ("ECG") – This test measures your heart's electrical activity (figure 1).

●Blood tests

●Tests to check how well your lungs are working

●Sleep study – This test checks your breathing, oxygen level, and other body functions while you are sleeping overnight. These tests can sometimes be done at home, but they are often done in a sleep lab.

To confirm that you have PH, your doctor might do:

●Pulmonary artery catheterization – This test measures the pressure in the blood vessels that go from your heart to your lungs. Your doctor puts a thin, flexible tube (called a "catheter") into a blood vessel in your groin, neck, or shoulder area. Then, they move the catheter into the blood vessels in your heart.

How is PH treated? — Treatment includes taking medicines to:

●Help with the symptoms of PH

●Treat the PH itself

Medicines to treat the symptoms of PH might include:

●Diuretics – These medicines help control swelling in the legs and feet. An example is furosemide (brand name: Lasix).

●Oxygen – Your doctor might have you use oxygen. It comes in a metal or plastic tank that you can take around with you. The oxygen flows from the tank into 2 plastic tubes that you put in your nostrils. Using oxygen makes it easier to breathe.

●Anti-clotting medicines – These are also called "anticoagulant" medicines. Some people call them "blood thinners." They help prevent blood clots in the lungs. For children, aspirin might be used instead. These are only needed if your PH is related to blood clots.

Medicines to treat the PH itself come as:

●Pills

●IV medicines – These are given through a thin tube that goes into a vein.

●Shots under the skin

Your doctor will also make sure that you are up-to-date on all of your vaccines.

In addition to taking medicine, you might also need to do something called "pulmonary rehabilitation." This is sometimes called "pulmonary rehab." It is a special program designed to help you understand your condition, improve your health, and improve your quality of life. You will do exercises to get stronger and make it easier to breathe. Pulmonary rehab usually lasts several weeks and includes education, exercises, and healthy lifestyle changes.

People with very serious symptoms might need surgery. There are different types of surgery:

●Right-to-left shunt surgery – Doctors can do a special type of heart surgery to make a hole in the heart. This lowers the pressure in the arteries leading from the heart to the lungs and makes it easier for the heart to pump.

●Transplant – With a transplant, the doctor replaces your diseased organ with a healthy one from another person. You might have a lung transplant, or a heart and lung transplant.

Is there anything I can do on my own to feel better? — Yes. You can:

●Stop smoking – Smoking or vaping is bad for your lungs and for your overall health. Doing these things can make PH symptoms much worse.

●Physical activity – Some people with PH are too tired to be very active. But if you can, walking or other light exercise can help. Check with your doctor before you begin doing physical activity.

What if I want to get pregnant? — Pregnancy is dangerous for people with PH. It can make your symptoms much worse, be dangerous for a developing fetus, and even lead to death. Some of the medicines used to treat PH are also unsafe for a fetus. Because of these risks, many people with PH choose to avoid pregnancy. Your doctor or nurse can talk to you about birth control options.

If you do want to get pregnant, talk to your doctor or nurse. They can talk to you about the risks and work with you to come up with a plan that is as safe as possible. You might also work with doctors that specialize in PH and pregnancy.

What will my life be like? — PH is a serious illness. Treatment can help, but PH can't be cured. For adults, the disease gets worse over time. Eventually, it will probably make it hard for you to do everyday things, like bathing and dressing.

For children, the disease might get better or worse over time. Talk to your doctor to find out what to expect with your type of PH.

Medicines might make you feel better and live longer. You will need to see your doctor often to check if you need to change the doses of the medicines you take.

What problems should I watch for? — If you or your child has PH, there are certain problems you should watch for.

Call for emergency help right away (in the US and Canada, call 9-1-1) if you or your child:

●Have sudden shortness of breath or sudden chest pain – These are signs that a blood clot has traveled to the lungs and is an emergency.

Call for advice if you or your child:

●Have signs of infection – These include a fever of 100.4°F (38°C) or higher, chills, or cough (with or without mucus).

●Are having trouble breathing – This includes:

•Finding it harder to breathe or feeling like you're getting less air

•Needing to lean forward when sitting to make breathing easier

•The skin below or between the ribs "caving in" when breathing

•Fingertips, fingernails, or lips looking blue

●Have headaches often

●Feel very sleepy or confused

●Pass out

●Have heart palpitations – These feel like the heart is pounding, beating hard or fast, or skipping beats.

More on this topic

Patient education: Heart failure (The Basics)
Patient education: ECG and stress test (The Basics)
Patient education: Echocardiogram (The Basics)
Patient education: Heart transplant (The Basics)
Patient education: Oxygen therapy at home (The Basics)