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Abstract In the following study, pulmonary hypertension is defined as an elevation of pulmonary artery pressure above normal, and is classified into five categories according to etiologies; the most important type is pulmonary arterial hypertension which may be idiopathic or associated with other diseases. According to pathophysiology different mechanisms are involved in development of the disease, and according to histopathology the main vascular lesions are described. Different experimental animal models are used to study the disease with various stimuli. Different lines of treatments including classic, recent and drugs in the pipelines are discussed according to mechanism of action, adverse effects, drug interaction, advantages and disadvantages of each group, and finally best drug combination therapy according to guidelines for treatment of pulmonary hypertension 2009. |