[Endothelin system and inhalation of iloprost in pulmonary hypertension].

The haemodynamics and vascular proliferation (remodelling) in pulmonary hypertension are unfavourably affected by a malfunction of the endothelial cells of the pulmonary vessels. A characteristic result is reduced formation of endothelial dilators and increased formation of vasoconstrictors. The favourable effects of a blockade of the endothelin (ET) system with receptor antagonists suggest that the increased formation and limited clearance of ET-1 reaches pathogenetic significance. The therapeutic effects of inhaled iloprost on right cardiac haemodynamics and clinical course, could in addition to the direct vasodilatory effect, also be considered to have a favourable influence on the ET system as a pathophysiologically effective mediator system.