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Primary Pulmonary Hypertension Causes, Causes of PPH

Primary pulmonary hypertension is a disease of unknown causes. It is often referred to as unexplained pulmonary hypertension or idiopathic pulmonary arterial hypertension (IPAH). It is thought that an insult to the endothelium lung tissue, whether due to a mechanical insult, such as major chest injury sustained during auto accidents, hormonal imbalance, or other, may open the door to increased vascular injury. Over time this leads to scarring of the vascular lung tissue, endothelial (vessel lining) dysfunction and the spreading of muscle tissue along the vessels. All of these activities can cause narrowing of the lung vessels (i.e. vasoconstriction) and increase the back pressure in the pulmonary artery. As the disease becomes more advanced, the vessels become even narrower and the pressure increases dramatically. Also, the narrowing of the vessels allows less blood into the lungs and thus, less oxygen into the blood and in the body.

Diet Drugs and Primary Pulmonary Hypertension

Treatment with diet drugs including Fen Phen, Redux (dexfenfluramine) and Pondimin (fenfluramine) have been shown to cause primary pulmonary hypertension. Studies show that treatment with these appetite suppressant drugs increases the risk of PPH by 28 times. The normal incidence of PPH in the population is 1 to 3 per million persons.

Family Ties to PPH

Studies show that at least 15 to 20% of patients with primary pulmonary hypertension have an inherited form of the disease. It is unclear whether it is a sporadic gene defect in these families.

Other PPH Causes

Other conditions associated with PPH include exposure to anorexigens or narcotic alpha adrenergic stimulants such as cocaine and amphetamines. HIV seropositivity is also associated with PPH. How these conditions cause primary pulmonary hypertension is unknown. If you or a loved one begins to display any of the Primary Pulmonary Hypertension symptoms, it is important that you consult a doctor immediately. PPH treatments, while unable to stop the progression of the disease, can help to improve the patient's quality of life. In addition, a doctor will help determine the exact cause of PPH. In cases whereby this condition is caused by dangerous diet drugs, the doctor might suggest that the patient seek counsel of a skilled personal injury attorney to determine if compensation might be available.

Last Revision: June 23, 2009

MediaFact is a noteworthy Internet resource about drug side effects connected with Stevens Johnson Syndrome and Primary Pulmonary Hypertension.

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