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Although primary pulmonary hypertension is rare, its incidence has increased with use of dexfenfluramine-derived anorexigens. Therefore, physicians should be alert for signs of the condition, which usually progresses rapidly and leads to right ventricular failure within a few years. In this article, the authors discuss triggers and presentation of primary pulmonary hypertension. They also provide a diagnostic algorithm and a summary of therapeutic methods that can improve quality of life and extend survival. Part 1 of this article, which described normal pulmonary vascular physiology and secondary pulmonary hypertension, appeared in the February 1999 issue.

Original Publication Date: March 1999

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