title: Etiology and pathogenesis of primary pulmonary hypertension: a perspective.
abstract: In recent years, considerable advances have been made in treating primary pulmonary hypertension (PPH). These have provided a series of therapeutic options, ranging from the oral administration of calcium channel blockers to the continuous infusion of prostacyclin and/or lung transplantation. These therapeutic advances have highlighted the need for the better understanding of etiology and pathogenesis. Among the key uncertainties, the following are defined as leading uncertainties: (1) the nature of the initiating lesion; (2) the shared pathogenetic mechanisms that culminate in the pathologic lesions of PPH; (3) the molecular genetic bases for familial PPH and for susceptibility to PPH; (4) understanding of the obliterative-proliferative occlusive process in the small muscular pulmonary arteries; and (5) redefinition of "primary" and "secondary," ie, a revised nomenclature of pulmonary hypertension. A revised classification based on etiology is presented.
authors.affiliation: Department of Rehabilitation Medicine, University of Pennsylvania, Philadelphia, USA.
authors.forename: A P
authors.initials: AP
authors.lastname: Fishman
mesh_terms: D000818:Animals; D002455:Cell Division / Q000502:physiology; D004730:Endothelium, Vascular / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D009131:Muscle, Smooth, Vascular / Q000503:physiopathology; D011379:Prognosis; D011651:Pulmonary Artery / Q000503:physiopathology; D012307:Risk Factors