Treatment of Pulmonary Hypertension Avoidance of activities that may exacerbate the condition (eg, cigarette smoking, high altitude, pregnancy, use of sympathomimetics) Idiopathic and familial pulmonary arterial hypertension: IV epoprostenol; inhaled, oral, subcutaneous, or IV prostacyclin analogs; oral endothelin-receptor antagonists; oral phosphodiesterase 5 inhibitors, oral soluble guanylate cyclase stimulators; oral prostacyclin (IP2) receptor agonists Secondary pulmonary arterial hypertension: Treatment of the underlying disorder Lung transplantation Adjunctive therapy: Supplemental oxygen, diuretics, and / or anticoagulants Pulmonary arterial hypertension, group 1 Treatment of pulmonary arterial hypertension (PAH) is rapidly evolving.