Editorial
Progress in, and future prospects for, the treatment of primary pulmonary hypertension
| The first 150 words of the full text of this article appear below. |
This is an exciting time to be in pulmonary hypertension research. The past few years have witnessed a steady flow of publications documenting the beneficial effects of prostacyclin treatment in primary pulmonary hypertension (PPH) and other forms of pulmonary arterial hypertension and the emergence of new drugs with therapeutic possibilities in these conditions. More recently, genetic analysis of familial PPH has identified a molecular mechanism that opens up new opportunities for pharmacological intervention in this disease.
Untreated PPH has an appalling prognosis, with a median survival of 2.5 years. Both warfarin and calcium antagonists (for those who can
tolerate them) improve survival but the most significant development in
the treatment of PPH to date has been the use of intravenous
prostacyclin and prostacylin analogues. Introduced initially as a
bridge to transplantation, the shortage of suitable donors meant that
many patients remained on treatment for several years. Controlled
clinical trials have demonstrated
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