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Etiology and Pathogenesis of Primary Pulmonary Hypertension : A Perspective FREE TO VIEW

Alfred P. Fishman
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From the Department of Rehabilitation Medicine, University of Pennsylvania, Philadelphia

Alfred P. Fishman, MD, FCCP, University of Pennsylvania School of Medicine, 1319 Blockley Hall, 418 Guardian Dr, Philadelphia, PA 19104-6021


1998 by the American College of Chest Physicians


Chest. 1998;114(3_Supplement):242S-247S. doi:10.1378/chest.114.3_Supplement.242S
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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.


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