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Clinical guideline for treatment of pulmonary arterial hypertension (PAH) has been changing during the past 10 years with development of targeted therapy for PAH. Treatment of PAH should include general measures and supportive care, treatment of associated disease, targeted treatment for PAH, and lung transplantation in advanced cases. Targeted therapy for PAH can improve symptom, quality of life and time to clinical worsening in PAH patients. Regular follow-up to evaluate the treatment efficacy is essential and insufficient treatment efficacy should be modified with other class of medication or preparing lung transplantations. Research for new medication is actively performed and novel drug is expected to be improving survival of PAH patients.