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The Familial Occurrence of Primary Pulmonary Hypertension

Ann Intern Med. 1966;65(4):672-684. doi:10.7326/0003-4819-65-4-672
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Primary pulmonary hypertension is a disease characterized by increased arterial pressure in the pulmonary circulation caused by changes which appear to be intrinsic to the pulmonary vasculature. The pathology of these changes has been described for some time and has been discussed by several authors (1-5). Although many theories about the cause of these changes have been proposed, none have been proved. Because of this lack of a proved pathogenic mechanism, some authors (6-8) have used the term "idiopathic," instead of primary pulmonary hypertension.

The clinical features of the disease have been so well described that some people feel the


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