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Idiopathic pulmonary arterial hypertension (previous primary pulmonary hypertension) was a progressive disease with
high mortality. Many patients with idiopathic pulmonary arterial hypertension did not show vasoreactivity, rapidly
resulted in marked disability, right heart failure and death. Recent advances of therapeutic modalities have revolutionized
the treatment of idiopathic pulmonary arterial hypertension. Irrespective of pulmonary arterial vasoreactivity, new
vasodilatng agents, such as epoprostenol, treprostinil, iloprost, bosentan, and sildenafil, significantly improved
hemodynamics, symptoms, exercise capacities, quality of life, and survival. The median survival of patients with
idiopathic pulmonary arterial hypertension has been prolonged from 2.8 years to more than 5 years. In a near future,
pulmonary arterial hypertension could be easily controlled like a systemic arterial hypertension.
Key Words : Pulmonary arterial hypertension, Vasoreactivity, Therapy |
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