Primary Pulmonary Hypertension

What is primary pulmonary hypertension?

Primary pulmonary hypertension (PPH) is a rare type of high blood pressure in the lungs. When there is increased blood pressure in the lungs, the right lower chamber of the heart (the right ventricle) must work harder to pump blood to the lungs. 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 your blood is not picking up enough oxygen from the lungs.

How does it occur?

The small blood vessels in the lungs get thicker and stiffer. Doctors do not know why this happens. High pressure is then needed to force blood through the thicker and stiffer blood vessels.

PPH is more likely to affect young and middle-aged women. It seems to run in families. Some drugs and viruses have been linked to PPH, but for a few cases.

What are the symptoms?

The main symptom is shortness of breath when you exert yourself. You are short of breath because the right ventricle cannot force enough blood through the lungs to give you enough oxygen. Chest pain often occurs. You may faint, especially during exercise. Your symptoms may get worse over time.

How is it diagnosed?

Your healthcare provider will ask about your symptoms and other things about your health, especially what medicines and drugs you have been taking. Your provider will examine you.

There are several tests you may have.

• You may have an electrocardiogram (ECG or EKG) to record the electrical activity of your heart.
• You may have a chest X-ray to look for decreased blood flow in the lungs and enlargement of the main blood vessels to the lungs.
• An echocardiogram, which is a test that uses ultrasound waves to create a picture of the heart, may show thickening of the right ventricle. The ultrasound can also help measure the blood pressure in the arteries of the lungs.
• Sometimes heart catheterization is needed to measure the pressure in the blood vessels in the lungs.
• Your healthcare provider will also need to test for other causes of high blood pressure in the lungs, such as blood clots in the lungs, diseases of the lung tissue, and liver disease.

How is it treated?

Your health care provider will try to find the treatment that works best for you. Some people are helped with drugs called calcium antagonists. These drugs improve symptoms but they are not a cure. They expand (dilate) blood vessels in the lungs and improve blood flow. Blood thinners (anticoagulants) may be prescribed to prevent small blood clots that might further block blood flow through the lungs. Prostacyclin, a normally occurring body hormone, may be given through a vein with the help of a special pump. Prostacyclin and a mixture of air and nitric oxide may improve symptoms temporarily. In unusual cases a heart-lung transplant may be a possibility.

How long will the effects last?

PPH used to be untreatable and always fatal, but current treatments seem to help people with this condition live longer.

How can I help take care of myself?

Learn to live within the limits of your condition. The following guidelines may help:

• Carefully follow your provider's instructions for taking your medicines. Make sure you don’t miss taking any medicines.
• Do not smoke.
• Eat a healthy diet.
• 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; consider indoor activities on these days. 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.
• Follow your healthcare provider's advice on how much liquid you should drink.
• Weigh yourself and write down your weight every day. Tell your healthcare provider as soon as possible if you gain 3 or more pounds in 1 day or 5 or more pounds in 1 week, or if you keep gaining weight over weeks to months. Weight gain may mean your body is having trouble getting rid of extra fluid.
• Avoid getting very hot or cold because it may cause your heart to work harder.
• Keep all medical appointments even when you are feeling well.