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.
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.
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.
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.
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.
PPH used to be untreatable and always fatal, but current treatments seem to help people with this condition live longer.
Learn to live within the limits of your condition. The following guidelines may help: