Primary Pulmonary Hypertension (PPH) is a disease that affects the pulmonary artery. This disease results in constriction of the pulmonary artery and the blood vessels in the lungs. The constriction of the pulmonary artery and the blood vessels in the lungs causes an individual's blood pressure to increase. This increase in blood pressure influences only the pulmonary artery, leaving the rest of the body alone. When this disease goes untreated, failure of the right side of the heart can occur. Even when the disease is treated, there is still not a cure. While there is not a cure, there are options that will increase the quality of life of those who have been diagnosed and that will extend life's duration. It is also important to note that research studies are being conducted all the time. These are being used to find a cure.
When an individual is first diagnosed with PPH, a doctor will first prescribe a low salt diet and diuretics. This may do a lot to help a person lessen the chest pains and other symptoms associated with the disease. If these do not do enough to improve a person's life, there are prescription medications that are available to help. An anticoagulant, or blood thinner, can help relieve pressure in the pulmonary artery. A blood thinner helps by making it easier for the heart to pump the thinner blood. The drug Flolan has been the most important discovery in the treatment of Primary Pulmonary Hypertension. This medication has been shown to increase an individual's ability to exercise, their quality of life, and their lifespan. This is just one of a few drugs that have been developed.
The problem with treatment is that there is no way to predict how an individual's body will respond to the drugs before they have been administered. What works for some people may not work for everyone. Because this is true, a doctor will commonly prescribe a series of treatments for a person in an effort to determine what will work best.
Primary Pulmonary Hypertension is a progressive disorder that is often fatal. Because of this, the best option for an individual might be a lung transplant. It is a treatment option that is available to people who are less than 65 years of age who not only have PPH but their PPH doesn't respond to prescription drug treatment. When a transplant is performed, 73% of patients survive one year past their transplant. 55% survive 3 years and 45% survive to 5 years after the transplant is performed.
A side benefit of the transplant is that there is an immediate reduction of pressure in the pulmonary artery. This reduction of pressure allows for an increased function on the right side of the heart. Primary Pulmonary Hypertension has not been reported in people once they have had the lung transplant.
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