What is pulmonary hypertension?

Pulmonary hypertension (PH) is high blood pressure in your lungs. The blood vessels in your lungs get thicker and stiffer, which makes your heart work harder to pump blood through your lungs. This raises the blood pressure in these blood vessels. The heart muscle gets thicker and stronger. As the disease gets worse, the right ventricle can’t get any stronger and you start having symptoms because not enough blood is being pumped through the lungs to pick up oxygen for your body to use.

What is the cause?

The cause of PH is not always known, but it can happen because of another disease. Possible causes include:

• Heart problems that you are born with
• Blood clots in your lung
• Lung diseases such as COPD or pulmonary fibrosis, which causes scar tissue in your lungs
• Heart failure or heart valve problems
• Sleep apnea, which is when you stop breathing for more than 10 seconds at a time many times during your sleep
• Some drugs, such as diet drugs
• Diseases that attack your immune system, such as HIV infection or rheumatoid arthritis that may damage the lungs

It is more likely to affect young and middle-aged women. It seems to run in families.

What are the symptoms?

The first symptoms are usually shortness of breath, fast heart rate, or feeling lightheaded with activity. Over time, you may have symptoms with only mild activity or while resting, such as:

• Chest pain
• Dizziness or fainting
• Feeling tired or weak
• Swelling in your legs

How is it diagnosed?

Your healthcare provider will ask about your symptoms and medical history and examine you. Tests may include:

• Blood tests
• Chest X-ray
• CT scan, which uses X-rays and a computer to show detailed pictures of the chest
• An ECG (also called an EKG or electrocardiogram), which measures and records your heartbeat.
• Lung scan, which uses a small amount of radioactive material injected into your blood or inhaled to make detailed pictures of your lungs
• Pulmonary angiogram, which is a series of X-rays taken after your healthcare provider places a long, thin, flexible tube (catheter) into a blood vessel in your groin and up to your heart and injects a special dye into your blood vessels to check how the blood is flowing through the arteries in your heart and lungs
• An echocardiogram, which uses sound waves (ultrasound) to see how well your heart is pumping
• Sometimes heart catheterization is needed to measure the pressure in the blood vessels in the lungs.

How is it treated?

There is no cure for PHT, but treatment can improve your symptoms. Treatment may include medicine to:

• Expand blood vessels in the lungs and improve blood flow
• Lower blood pressure
• Prevent blood clots that might further block blood flow through the lungs
• Help your heart pump

You may need oxygen therapy to help you breathe better.

A heart-lung transplant may be a possibility for some people.

How can I take care of myself?

Follow the full course of treatment prescribed by your healthcare provider. In addition:

• Carefully follow your provider’s instructions for taking medicines.
• Do not smoke.
• Follow your provider’s recommendations for physical activity. Exercise helps strengthen your heart and body and improves your blood flow and energy level. Avoid outdoor exercise if it is very hot, cold, or humid. Getting very hot or cold may cause your heart to work harder. Balance exercise with rest.
• Get enough rest, shorten your working hours if possible, and try to reduce the stress in your life. Anxiety and anger can increase your heart rate and blood pressure. If you need help with this, ask your healthcare provider.
• Ask your provider if you should avoid drinking alcohol. Alcohol can weaken your heart or may worsen heart failure. It may also interfere with medicines you are taking.
• Try not to get sick with a cold or the flu, which can be very serious if you have pulmonary hypertension. Stay away from people who are sick and get a flu shot every year. Ask your healthcare provider if you need a pneumococcal shot.
• Women with PH should avoid getting pregnant.

Ask your provider:

• How and when you will hear your test results
• If there are activities you should avoid and when you can return to your normal activities
• How to take care of yourself at home
• What symptoms or problems you should watch for and what to do if you have them

Make sure you know when you should come back for a checkup. Keep all appointments for provider visits or tests.