Advances in the treatment of pulmonary arterial hypertension: focus on endothelin receptor antagonism

The term pulmonary arterial hypertension (PAH) defines a variety of conditions with different aetiologies but similar clinical presentation; it is characterised by progressive increase of pulmonary vascular resistance leading to right ventricular failure and premature death. In recent years, a great deal of research has been carried out on the pathogenesis, epidemiology and treatment of PAH. Patients have benefited substantially, as a number of disease-specific drug treatments have been approved, that not only improve haemodynamics and clinical state, but also prolong survival. The present supplement summarises the key presentations and topics of the workshop 'New perspectives in the treatment of endothelin-related diseases' held at the 40th Annual Scientific Meeting of the European Society of Clinical Investigation (ESCI) in Prague, Czech Republic, in March 2006. It comprises a series of in-depth reviews, previously unpublished original articles and case reports, mostly focussing on the endothelin system, which plays a pivotal role in PAH, and pharmacological antagonism to this system.