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Pulmonary artery hypertension is a severely debilitating and fatal disease. Unfortunately, despite recent advances in treatment options for this disease, physicians and patients still face many difficulties in using treatment methods and dealing with the problems. During the preparation of this guideline, experienced experts in this field carefully examined the evidences from the reliable sources and an attempt was made to set the recommendations on evidence. Unfortunately, in many cases, there is no accurate evidence to answer the questions that arise in the face of these patients, which is mainly due to the small and heterogeneous number of patients in the studies. Therefore, in cases where there was strong and sufficient evidence based on controlled clinical trials, recommendations were made based on this evidence, and in cases where there was insufficient evidence, the recommendations were based on the collective wisdom of experienced experts in this field and case studies. This guideline is a guide for the treatment of patients with pulmonary artery hypertension (group I) and emphasizes that before starting treatment, the exact diagnosis of the disease and its cause must be determined by experienced specialists in this field

Keywords: Guidelines, Pulmonary artery hypertension, Lung

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