Primary Pulmonary Hypertension
What is Primary Pulmonary Hypertension?
Primary Pulmonary Hypertension (PPH) is a rare, progressive disorder characterized by high blood pressure (hypertension) of the main artery of the lungs (pulmonary artery). The pulmonary artery is a blood vessel carrying oxygen-poor blood from the right ventricle (one of the heart’s pumping chambers) to the lungs. In the lungs, the blood picks up oxygen, then flows to the heart’s left side, where the left ventricle pumps it to the rest of the body through the aorta (1).
The American Heart Association (www.americanheart.org) says, “Hypertension is a medical term for abnormally high blood pressure. This abnormally high pressure (pulmonary hypertension) is linked with changes in the small blood vessels in the lungs. These changes increase resistance to blood flowing through the vessels. This increased resistance puts a strain on the right ventricle, which now must work harder than usual to move enough blood through the lungs.”
PPH Statistics:
In the United States, an estimated 500 to 1,000 new cases of primary pulmonary hypertension are diagnosed each year.
The greatest number is reported in women between ages 20 and 40. However, men and women in all age ranges - as well as very young children - can develop PPH (2).
What are the symptoms of PPH?
The most common symptoms of primary pulmonary hypertension are related to difficulty breathing. They include:
- Progressive shortness of breath (especially with activity)
- Hyperventilation (breathing harder and faster)
- Swelling of ankles or feet (edema)
- Fatigue (tiring easily)
- Chest pain (angina)
- Progressive weakness
- Fainting spells
- Lightheadedness or dizziness
- Coughing up blood
- Progressive cyanosis (blue tinge to lips, hands and feet)
- The pumping action of your heart grows weaker
- Your energy decreases
- Are able to perform very little activity
- Have symptoms even when resting
- May become completely bedridden
PPH is still not totally understood by the medical community; while doctors and physicians are unable to compile a definite list of dangerous drugs or environmental conditions resulting in the development of PPH, a study by the World Health Organization (www.who.int) suggests appetite suppressants such as “fen-phen” and amphetamines have a “very likely causative role in pulmonary hypertension (3).”
According to an article in the American Family Physician (www.aafp.org), “The use of appetite-suppressant drugs for more than three months is associated with a greater than 30 times increased risk of developing pulmonary hypertension. In the United States, the anorexic agents fenfluramine and dexfenfluramine were recalled in September 1997, only 18 months after they were released (4).”
The World Health Organization cited aminorex (the appetite suppressant aminorex fumarate is thought to have caused an epidemic of pulmonary hypertension in Europe in the 1960s), fenfluramine and dexfenfluramine (“fen-phen”) on the ‘risk factors for Primary Pulmonary Hypertension – definite causal relationship’ list at the World Symposium on Primary Pulmonary Hypertension conference.
What if I have been diagnosed with PPH?
Always consult your physician for medical advice. PPH therapy has advanced significantly in recent years, which has resulted in increased survival rates amongst people with this disease. Unfortunately, there is no cure for PPH. If you or someone you care about has been diagnosed with PPH, contact our law firm today for a free and immediate evaluation of your case.
Sources:
Pulmonary Hypertension, Primary, WebMD (www.webmd.com), June 11, 2004.
Primary or Unexplained Pulmonary Hypertension, American Heart Association (www.americanheart.org) accessed June 8, 2005.
Rich S, ed. Executive summary from the World Symposium on Primary Pulmonary Hypertension, 1998, Evian France, September 6-10, 1998, cosponsored by the World Health Organization.
Diagnosis and Treatment of Pulmonary Hypertension, Trenton D. Nauser, M.D. and Steven W. Sittes, M.D. May 1, 2001, Voulme 63, American Family Physician.
This article is for informational purposes only. It is not intended to constitute medical advice and should not be viewed as such. For medical advice, you should always consult with your physician.
This law firm is not affiliated with, sponsored by or associated with WebMD, the American Heart Association, the National Heart, Lung, and Blood Institute, the World Health Organization, or the American Family Physician publication.
