Tuesday, December 8, 2009

Pulmonary Hypertension - PPH

Pulmonary Hypertension
Pulmonary Hypertension is a very serious lung and heart disorder related to the blood vessels in the lungs. When a person has Pulmonary Hypertension these blood vessels constrict, increasing pressure in the pulmonary artery (connecting the heart to the lungs) and creating a life-threatening situation. Pulmonary Hypertension causes the tiny blood vessels in the lungs to change and close off, preventing blood from flowing freely. Victims of Pulmonary Hypertension suffer additional stress to the right ventricle of the heart, which has to work harder and harder to circulate enough blood through the lungs.
Symptoms of Pulmonary Hypertension include shortness of breath (also called dyspnea), which can come about even with little or no physical exertion. Pulmonary Hypertension also causes fatigue, chest pain, dizziness and fainting spells. Most persons diagnosed with Pulmonary Hypertension die within three years of diagnosis, although new treatments and early diagnosis are extending that time somewhat. Currently, Pulmonary Hypertension victims may be able to survive for ten years or more following their diagnosis.
There are two types of Pulmonary Hypertension: Primary and Secondary. Both forms of this disorder are similar in all but causes: Secondary Pulmonary Hypertension can be attributed to a specific cause, while Primary Pulmonary Hypertension cannot be linked to a specific cause. Ironically, when the disease is classified as Primary Pulmonary Hypertension, it is often connection to diet drug use, although the causal relationship has not been clearly established, since doctors do not yet fully understand the mechanics of that link.
Pulmonary Hypertension is a rare disease, only occurring in around two persons per million annually. Because it is so rare and the symptoms are so common to other diseases, Pulmonary Hypertension is often diagnosed only in the very late stages. In fact, the average length of time from the onset of symptoms to a Pulmonary Hypertension diagnosis is two years. This affects treatment options, increasing the chances of mortality.
Treating Pulmonary Hypertension usually focuses on extending the life span of the patient. There are few drugs approved for the treatment of Pulmonary Hypertension, and they are extremely expensive. The most effective way to treat Pulmonary Hypertension is through a single or double lung transplant, but these procedures are also very expensive, and the wait for a lung transplant is too long for many Pulmonary Hypertension victims. In rare cases, a heart transplant may also be required, but the heart strain is generally reversed naturally following a lung transplant.
Because Primary Pulmonary Hypertension has been linked to the use of diet drugs such as Fen-Phen, victims may be entitled to compensation from the drug manufacturers. Primary Pulmonary Hypertension was known to have developed in weight loss pill users in Europe, yet American companies continued to market diet pills as safe weight loss options. Primary Pulmonary Hypertension victims are able to bring claims for
compensation, including the cost of medical treatment, lost wages, and pain and suffering. Awards in the millions of dollars for Primary Pulmonary Hypertension sufferers (or their surviving loved ones) are not uncommon. To learn more about Primary Pulmonary Hypertension and your legal rights, contact an attorney who has experience in working with victims of diet drugs.


PPH Explained
What is PPH?
Primary pulmonary hypertension, also known as PPH, is a rare disease that has no known cause and results in the progressive narrowing of the blood vessels of the lungs. There are no known cures for PPH, but treatment for the cardiovascular condition is improving. The lungs contain millions of tiny blood vessels called capillaries. These capillaries are extremely small, just wide enough for blood cells to move through. The capillaries are lined with what's known as endothelial cells that prevent blood from leaking out of the vessel. On the outside of the blood vessels are muscle cells that expand and contract as blood moves through from the heart into the lungs.

PPH occurs when the vessels gradually stop functioning as they are supposed to. This happens when the cells within the blood vessels weaken and allow blood to leak. This, in turn, causes the exterior muscle cells to constrict. The constriction gradually worsens, eventually driving up the pulmonary arterial pressure. The exact cause of PPH is unknown. However, a prevalent theory holds that the blood vessels react strongly to certain internal and external factors, causing them to constrict when exposed to these factors. Aside from fen-phen and Redux, the causes of PPH include principally congenital heart defects, portal hypertension, HIV infection, Collagen Vascular Disease, and intravenous drug use.

PPH began surfacing through the 1970s because of the latency period found to exist between drug use and PPH diagnosis of 4-6 years. Currently cases of PPH are still being diagnosed. Often times with PPH diagnosis it not immediate because early PPH symptoms are easily mistaken for other causes. This delay in diagnosis results in the disease progressing to late stages without proper treatment.

Secondary pulmonary hypertension (SPH) is the result of other types of lung disease, abnormal breathing processes or heart disease.
What are Heart Valve Defects?
When these valves are impaired, they can cause blood to be regurgitated into the ventricle and left atrium. The symptoms of heart valve disease may be subtle---existent in the presence of shortness of breath and chest pain. Treatment can include medication, a valvoplasty, or a repair of the damaged heart valves and/or heart valve replacement surgery. This condition often requires long term heart monitoring and medications.

Dr. Praveen Sharma
9212943002


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