Korean J Med.  2006 Jul;71(1):4-9.

Diagnosis and treatment of idiopathic pulmonary arterial hypertension

Affiliations
  • 1Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Abstract

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.

Keyword

Pulmonary arterial hypertension; Vasoreactivity; Therapy

MeSH Terms

Diagnosis*
Epoprostenol
Heart Failure
Hemodynamics
Humans
Hypertension*
Iloprost
Mortality
Quality of Life
Sildenafil Citrate
Epoprostenol
Iloprost
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