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5
Scottish Pulmonary
Vascular Unit, Western Infirmary, Glasgow G11 6NT, UK
Correspondence to: Dr A J Peacock
| The first 150 words of the full text of this article appear below. |
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Introduction |
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Primary pulmonary hypertension (PPH) is a rare disorder with an
incidence of one to two cases per million people per year in Western
populations. It is a progressive disease usually affecting the arterial
side of the pulmonary circulation and, if untreated, progresses to
severe pulmonary hypertension and, finally, right heart failure.
Characteristically, and because of the vague nature of the symptoms,
PPH may take up to 18 months to diagnose and, following diagnosis, the
median survival is under two years. The disease is most common in women
(ratio 1.7:1) and the mean age at the time of diagnosis is in the mid
30s. This bleak picture of PPH has been transformed over the last
decade, partly because of better diagnostic techniques allowing earlier
diagnosis and also because of new treatments, in particular the use of
continuous intravenous prostacyclin. It is now realised that
vasoconstriction of the pulmonary vascular bed is not
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