Patient education: Pulmonary hypertension (The Basics)

What is pulmonary hypertension? — 

Pulmonary hypertension ("PH") is 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? — 

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 your other health problems.

First, you might get an echocardiogram ("echo"). This uses sound waves to create pictures of the 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 – These create pictures of the inside of the body.

●Electrocardiogram ("ECG") – This measures the electrical activity of the heart (figure 1).

●Blood tests

●Tests to check how well your lungs are working

●Sleep study – This checks your breathing, oxygen level, and other body functions while you sleep. The study is usually done in a sleep lab, but sometimes can be done at home.

To confirm you have PH, your doctor might do:

●Pulmonary artery catheterization – This 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 help control swelling in the legs and feet. An example is furosemide (brand name: Lasix).

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

●Anticoagulants – These are also called "anti-clotting" medicines or "blood thinners." They help prevent blood clots in the lungs. They are only needed if your PH is related to blood clots. Children might take aspirin instead.

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 you are up to date on all your vaccines.

In addition to taking medicine, you might also need to do pulmonary rehabilitation ("rehab"). This is a special program designed to help you understand your condition, and improve your health and 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 – The doctor replaces the diseased organ with a healthy one from another person. You might have a lung transplant, or a heart and lung transplant.

What can I do on my own to feel better? — 

You should:

●Quit smoking, if you smoke – Smoking makes PH symptoms much worse and is bad for your lungs and overall health. If you are having trouble quitting, your doctor or nurse can help.

●Get regular physical activity – You might feel too tired to be very active. But if you can, walking or other light exercise can help. Check with your doctor before you start 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 during pregnancy. 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, tell 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 cannot 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 need to see your doctor often to check if you need to change any doses.

What problems should I watch for? — 

If you or your child has PH, there are certain problems to 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 a blood clot has traveled to the lungs. This 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:

•Feeling like you're getting less air than usual

•Needing to lean forward when sitting to breathe

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

•Blue fingertips, fingernails, or lips

●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: Echocardiogram (The Basics)
Patient education: ECG and stress test (The Basics)
Patient education: What is a sleep study? (The Basics)
Patient education: Pulmonary artery catheterization (The Basics)
Patient education: Oxygen therapy at home (The Basics)
Patient education: Anticoagulant medicines – Uses and kinds (The Basics)
Patient education: Pulmonary rehabilitation (The Basics)
Patient education: Heart transplant (The Basics)

Patient education: Heart failure (Beyond the Basics)
Patient education: Heart transplantation (Beyond the Basics)