LYCOS RETRIEVER 
Primary Pulmonary Hypertension: Diseases
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In contrast to PPH, secondary pulmonary hypertension (SPH) means the cause is known. A common cause of SPH are the breathing disorders emphysema and bronchitis. Other less frequent causes are the inflammatory or collagen vascular diseases such as scleroderma, CREST syndrome or systemic lupus erythematosus. Because PPH cannot be diagnosed until all types of secondary pulmonary hypertension (of which there are many) have been excluded on clinical grounds, PPH is very difficult to diagnose.
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The mechanism by which the Ca2+-channel blockers provide benefit is primarily through vasodilatation, as represented by the fall in mean pulmonary artery pressure that occurs. They do not have positive inotropic effects; ... the increase in cardiac output that accompanies therapy likely relates to pressure unloading of the right ventricle. There are no clinical features that prospectively identify responsive patients (those with an early 20% fall in PVR), but such patients tend to have less advanced disease and a more recent onset of symptoms. It is estimated that these "responders" represent 20% of all patients with PPH.
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PAH related to other causes -- When pulmonary hypertension occurs as a result of exposure to toxins or the effects of other diseases, it is referred to as associated pulmonary hypertension. Causes include:
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