Management of Primary Pulmonary Hypertension

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


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}.


Pulmonary Hypertension Pulmonary Vascular Resistance Pulmonary Vasculature Primary Pulmonary Hypertension Vasodilator Therapy 
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© Kluwer Academic Publishers 1988

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  • Milton Packer

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