LYCOS RETRIEVER 
Primary Pulmonary Hypertension: Patients
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Doctors have developed functional classifications of Primary Pulmonary Hypertension based on the level of the conditions and symptoms involved. The general treatments for PPH include: Calcium Channel blockers that can be used to lower pressure; oxygen enables the patient to breath easier and lowers pressure; Coumadin (warfarin) thins the blood and prevents clotting; diuretics or "water pills" relieve fluid that collects in the ankles and belly area, digoxin slows the heart rate to improve muscle force. These new treatments for PPH include use of Tracleer, Flolan, Reodulin (UT-15) and Bosentan. The most prescibed and so far the most effective PPH treatments have been with the use of Tracleer and Flolan.
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Primary pulmonary hypertension (PPH) is a rare disease that is characterized by a progressive elevation of pulmonary artery pressure (PAP) and vascular resistance without a demonstrable cause. It is considered to be a progressive, incurable, and fatal disease.1 2 3 4 5 6 7 8 9 PPH can start at any age, but it strikes mostly during the third and fourth decades of life. The ratio of women to men with PPH is 1.7:1. Estimates of the incidence of PPH range from one to two cases per million people in the general population,2 and the number seems to be rising.9 Diagnosis is difficult to achieve and often is established only late in the course of the disease.2 3 Data from the US National Institutes of Health (NIH) registry on PPH2 3 show that PPH is a fatal disease in which most of the patients die within 2 to 3 years from the diagnosis and the 1-year survival rate is 68%, the 2-year survival rate is 48%, and the 5-year survival rate is 34%.
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Primary pulmonary hypertension has no cure. If left untreated, the right side of the heart becomes overworked and eventually fails, leading to death. One study showed that 30% of untreated PPH patients died within 3 years of diagnosis. New treatments for PPH, developed during the 1990's, have helped to relieve the symptoms and improve the quality of life of PPH patients.
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When primary pulmonary hypertension was diagnosed, a multidisciplinary team comprising a cardiologist, obstetrician, intensive-care anaesthetist, paediatrician and midwife was assembled to manage the patient. Since she had reached the 26th week of gestation and was stable with no suggestion of right heart failure, pregnancy was cautiously continued with close monitoring of the maternal and fetal condition. At the 28th week she was transferred to the intensive-care unit. A pulmonary artery flotation catheter was inserted and PA pressure was 97/43 mmHg (systemic blood pressure 140/65 mmHg). Nebulized Iloprost 15 µg lowered the PA pressure to 72/33 mmHg, an effect lasting 3 hours; systemic blood pressure was unaffected. The patient was then transferred to theatre where a lumbar epidural catheter was inserted.
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A nationwide Class Action Settlement has been reached with manufacturer American Home Products that resolves the claims of former Pondimin and Redux patients suffering serious and fatal heart conditions, including primary pulmonary hypertension (PPH). On January 11, 2002, the Fen Phen settlement became final, entitling the estimated 6 to 7 million Americans that have taken Fen Phen eligible for compensation. Under the Settlement it allows former Redux and Pondimin patients to receive compensation if they have developed the deadly and serious conditions that have been linked to the once popular diet drugs. If you have used the diet drug Fen Phen, including Redux and Pondimin, you are eligible to file a claim by August 2002.
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Pulmonary thromboendarterectomy (PTE) is a surgical procedure that is used for chronic thromboembolic pulmonary hypertension. It is the surgical removal of an organized thrombus (clot) along with the lining of the pulmonary artery; it is a very difficult, major procedure that is currently performed in a few select centers. Case series show remarkable success in most patients.
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