Pulmonary Hypertension

What is pulmonary arterial hypertension?

Pulmonary arterial hypertension (PAH) is a condition that increases pressure in your pulmonary artery. The pulmonary artery is the large blood vessel that brings blood from your heart to your lungs.

What causes pulmonary arterial hypertension?

PAH may be passed from a parent to a child. This is called familial PAH. PAH with no known cause is called idiopathic PAH. Associated PAH means another condition caused you to develop PAH. The following are common causes of associated PAH:

• Certain conditions, such as tumors pressing on the pulmonary artery.
• Problems in your heart, lungs, or blood vessels.
• Connective tissue diseases, such as scleroderma, lupus, or rheumatoid arthritis.
• Weight loss medicines or illegal drugs.

What are the signs and symptoms of pulmonary arterial hypertension?

• Weakness and tiredness.
• Weight gain or lack of appetite.
• Joint pain.
• Shortness of breath with exercise.
• Abdominal swelling.
• Chest pain or heart palpitations (strong, fast heartbeats).
• Dizziness or feeling faint.

How is pulmonary arterial hypertension diagnosed?

Your healthcare provider will ask about your medical history and examine you. You may need any of the following tests:

• Blood tests. Blood tests may be used to find the cause of your PAH.
• A blood vessel test. This test may be used to see if the small arteries in your lungs will widen (dilate) when you are given a medicine. The pressure in your pulmonary artery will be measured before and after the medicine is given.
• Electrocardiogram (EKG). An EKG may be used to check for damage or problems in your heart. A short period of electrical activity in your heart is recorded.
• X-rays, echocardiogram, CT, or MRI. These tests may be used to show the structure, movement, and blood vessels of your heart. You may be given contrast liquid to help healthcare providers see the pictures better. Tell your healthcare provider if you have ever had an allergic reaction to contrast liquid. Do not enter the MRI room with anything metal. Metal can cause serious injury. Tell your healthcare provider if you have any metal in or on your body.
• Cardiac catheterization. This procedure looks for or treats a heart condition. A catheter is inserted in your arm, neck, or groin and moves into your heart. Contrast liquid is injected into an artery and X-rays of your blood flow are taken. Tell your healthcare provider if you have ever had an allergic reaction to contrast liquid.
• Pulmonary function tests (PFTs). PFTs help healthcare providers learn how well your body uses oxygen. You breathe into a mouthpiece connected to a machine and the machine measures how much air you breathe in and out over a certain amount of time. PFTs help your healthcare providers decide the best treatment for you.
• A ventilation and perfusion (VQ) scan. This scan takes pictures of your lungs. During the perfusion part of the test, contrast liquid will be given through an IV. This liquid helps the blood flow in your lungs show up clearly on the monitor. During the ventilation part, you will breathe in a medical gas. Pictures will be taken to see how well your lungs take in oxygen. Tell your healthcare provider if you have ever had an allergic reaction to contrast liquid.

How is pulmonary arterial hypertension treated?

There is no known cure for PAH. The goal of treatment is to improve your condition and stop it from getting worse. To treat PAH, you may need any of the following:

• Medicine. Medicine may be given to improve blood flow, get rid of extra fluid, or prevent blood clots. Blood clot medicine may make you bruise or bleed more easily. Use a soft toothbrush and an electric shaver to prevent bleeding.
• Extra Oxygen. You may need extra oxygen if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. Ask your healthcare provider before you take off the oxygen mask tubing.
• Surgery. Surgery may be used to help blood flow from one part of the heart to another. You may need lung or heart transplant surgery if other treatments do not work and your condition is severe.

How can I manage my pulmonary arterial hypertension?

• Eat less sodium (salt). You may need to limit the amount of sodium you eat. Check labels to find low-sodium or no-salt-added foods. Some low-sodium foods use potassium salts for flavor. Too much potassium can also cause health problems. Ask your healthcare provider how much sodium and potassium salt are safe for you to eat each day.
• Limit liquids as directed. You may need to drink less liquid to help balance your fluid level. Ask your healthcare provider how much liquid you should drink each day.
• Exercise as directed. Exercise may help decrease your symptoms and improve your heart function. Exercise also helps with weight control. Do not start an exercise program before you talk with your healthcare provider.
• Avoid activities that raise your body temperature. Do not sit in a sauna, hot tub, or hot bath. This can lower your blood pressure and cause you to faint.
• If you are a woman, talk to your healthcare provider about pregnancy. Pregnancy may not be safe for you. You may need to change your birth control method if you currently use birth control pills. Birth control pills may increase your risk for blood clots. Your healthcare provider can help you choose other methods that work for you.
• Manage health conditions affecting your PAH. You many need treatment for sleep apnea, hypertension, or other medical conditions. Ask your healthcare provider for more information.
• Do not travel to high altitudes unless your healthcare provider says it is okay. You may need to bring extra oxygen if you are traveling to a high altitude or  flying.

When should I seek immediate care?

• Your legs or ankles are swollen.
• You are vomiting and cannot eat or drink.
• You are confused or feel like you are going to faint.

Call 911 for any of the following:

• You have chest pain or heart palpitations.
• You have shortness of breath at rest, especially when you lie down.

When should I contact my healthcare provider?

• You have a fever.
• Your symptoms keep you from doing your daily activities.
• Your joints are painful and swollen.
• Your fingers or toes are clubbed (the ends are round and thick).
• You have questions or concerns about your condition or care.