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Management of Primary Pulmonary Hypertension

  • Milton Packer
Part of the Developments in Cardiovascular Medicine book series (DICM, volume 83)

Abstract

Primary pulmonary hypertension represents a heterogenous group of disorders in which pulmonary artery pressure and pulmonary vascular resistance are markedly elevated in the absence of known causes {1–3}. Although the disease commonly afflicts young women, patients of any sex or age may be affected. Three distinct pathologic subtypes of primary pumonary hyper­tension are recognized by the World Health Organization {4}: plexogenic arteriopathy, microthromboembolism, and pulmonary veno­occlusive disease, but it remains unknown whether these categories represent distinct eti­ologies with a common final clinical picture or represent different pathologic manifestations of the same disease. The dilemma is made more complicated by the fact that these subtypes can­not be distinguished clinically with confidence, and attempts to establish a diagnosis by open lung biopsy risks the possibility that pathologic changes seen in a small specimen may not be present throughout the lung {5}.

Keywords

Pulmonary Hypertension Pulmonary Vascular Resistance Pulmonary Vasculature Primary Pulmonary Hypertension Vasodilator Therapy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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© Kluwer Academic Publishers 1988

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  • Milton Packer

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