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Official Journal of the Italian Society of Thoracic Endoscopy
Indexed/Abstracted in: EMBASE, Scopus
Online ISSN 1827-1723
Spiekerkoetter E., Hoeper M.
Pulmonary arterial hypertension is a severe disease leading to progessive obliteration of the pulmonary vasculature, increased pulmonary vascular resistance, right ventricular hypertrophy and eventually right heart failure. Untreated, the median survival after diagnosis was 2.8 years in the 1980s. With the introduction of continuous intravenous prostacyclin the prognosis of pulmonary arterial hypertension has improved substantially over recent years. Alternative applications of prostanoids such as inhaled Iloprost, subcutaneous Treprostinil or oral Beraprost have shown beneficial treatment effects in large multi-center trials. The orally available dual endothelin receptor antagonist Bosentan has broadened the therapeutic options even further. New agents such as phosphodiesterase inhibitors and NO donors such as L-Arginine deserve further evaluation. These less invasive therapies should be considered in selected patients before invasive and more risky therapeutic options such as intravenous prostacyclin, atrial septostomy or lung transplantation are initiated. The most effective strategy for pulmonary hypertension will probably be a combination therapy. Clinical trials examining the safety and efficacy of prostanoids plus endothelin antagonists or phosphodiesterase inhibitors are ongoing.