Pulmonary arterial hypertension (PAH) is a rare but debilitating disease, which if left
untreated rapidly progresses to right ventricular failure and eventually death. In the quest to
understand the pathogenesis of this disease differences in the profile, expression and action
of vasoactive substances released by the endothelium have been identified in patients with
PAH. Of these, endothelin-1 (ET-1) is of particular interest since it is known to be an
extremely powerful vasoconstrictor and also involved in vascular remodelling. Identification …