prototype of clinical pulmonary hypertension is primary pulmonary
hypertension (PPH), for which interesting new information is available.
PPH is a severe and progressive disease with a mean survival of < 3
years without treatment. The central feature of PPH is widespread
obstructive lesions of small pulmonary arteries. Despite dramatic
advances in many aspects of PPH during the past decade, including
improved diagnosis, new understanding of biological mediators, and the
development of the first effective therapy, the possibility of
prevention or cure seems elusive unless the central basic mechanisms
are identified and characterized.