LYCOS RETRIEVER 
Primary Pulmonary Hypertension: Patients
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Eighteen patients with primary pulmonary hypertension (17 adults and 1 child) were entered into this long-term study after informed consent was obtained. From the 25 patients enrolled in the preceding 8-week randomized study [19], the 10 surviving patients already receiving prostacyclin plus conventional therapy and 8 of the 11 survivors from the group receiving conventional therapy alone elected to enter this long-term study. The clinical and demographic characteristics are shown in Table 1. The mean age was 35.9 ±13.4 years. Twelve patients were female and 6 patients were male. Seventeen of the 18 patients were NYHA class III or IV despite conventional therapy, which consisted of vasodilators, oxygen, diuretic agents, cardiac glycosides, and anticoagulant agents as deemed necessary.
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Epoprostenol (Flolan), or prostacyclin, is the single most important advance in the treatment of primary pulmonary hypertension. This potent, short-acting vasodilator and inhibitor of platelet aggregation is produced by vascular endothelium. In one study,19 continuous intravenous infusion of epoprostenol improved exercise capacity, quality of life, hemodynamics and long-term survival in patients with class III or IV function (Table 2). Although the delivery system for continuous infusion is complex, most patients are able to learn how to prepare and infuse the drug.
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There are several known causes of pulmonary hypertension: chronic obstructive pulmonary disease, congenital heart disease, mitral stenosis, left ventricular dysfunction and recurrent pulmonary emboli. Primary Pulmonary Hypertension is a disorder of unknown etiology which is diagnosed only after the known causes of pulmonary hypertension have been eliminated. Prior to the PPH registry, little was known regarding the epidemiology, etiology, natural history or ultimate survival among patients with PPH. In 1973, the WHO met to review the current state of knowledge on PPH and proposed the establishment of a multicenter collaborative study. The NHLBI PPH registry enrolled patients in the registry from 1981 to 1985.
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The prognosis for patients with primary pulmonary hypertension can cover a wide spectrum. Many patients report that lifestyle changes allow them to perform many of their day-to-day affairs. The median period of survival is three years after diagnosis, although the survival rate is generally longer for those patients without heart failure and for those patients diagnosed after 40.
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PHA is primarily a volunteer support group for patients with pulmonary hypertension (PH) of any cause. The group is administered by PH patients and their families. They provide education for patients and physicians about the disease. They ... have a nationwide system of support groups and maintain a listing of physicians experienced with treating PPH.
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Researchers are studying oral treatments for primary pulmonary hypertension. Encysive Pharmaceuticals is recruiting patients for a phase III study comparing sitaxsentan sodium (Thelin) with placebo. For information, call PharmaTech Solutions at (866) 332-3275.
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