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Pulmonary arterial hypertension: current therapeutic strategies

Nature Clinical Practice Cardiovascular Medicine volume 4, pages 319–329 (2007)Cite this article

Abstract

The treatment of pulmonary arterial hypertension—once a lethal condition—has evolved considerably over the past few years as the number of therapeutic options available to treat this disease has increased. In this Review we attempt to summarize the current knowledge of the pathogenesis of pulmonary hypertension, in relation to the therapies presently available and those that could become available in the near future. The use of prostacyclin and its analogs, calcium-channel blockers, endothelin-receptor antagonists and phosphodiesterase type 5 inhibitors is reviewed. Newer concepts, such as the use of combination therapy, and the potential for long-term disease amelioration and improvement of outcomes, are also discussed. The role of supportive care and medications not specific to pulmonary hypertension is also examined. In addition, we review the novel emerging therapies, such as imatinib, fasudil, simvastatin, ghrelin and vasoactive intestinal peptide, which hold therapeutic potential for disease modification as well as treatment of symptoms.

Key Points

  • Therapy for pulmonary hypertension has evolved significantly over the past few years with the availability of new prostanoids, endothelin-receptor antagonists and phosphodiesterase type 5 inhibitors

  • A response to vasodilator challenge should determine which patients might benefit from a period of calcium-channel blocker therapy

  • Continuous infusion therapy with intravenous epoprostenol should generally be reserved for those patients in whom oral therapies are failing, those with progression of disease, or those who present with evidence of significant right ventricular dysfunction

  • After appropriate work-up, the first-choice treatment for most patients at initial presentation is an oral agent, either an endothelin-receptor antagonist (such as bosentan) or a phosphodiesterase type 5 inhibitor (such as sildenafil)

  • Combination therapy is increasingly used and could be beneficial in some patients

  • Therapies that could become available in the foreseeable future will offer other therapeutic options for this patient group

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Acknowledgements

Charles P Vega, University of California-Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. A Puri is a Senior Research Associate in Cardiology at King George's Medical University, Lucknow, India.,

    Aniket Puri

  2. MD McGoon is Professor of Medicine and Director of the Pulmonary Hypertension Clinic at Mayo Clinic.,

    Michael D McGoon

  3. SS Kushwaha is an Associate Professor of Medicine and Consultant in Cardiology at Mayo Clinic, Rochester, MN, USA.,

    Sudhir S Kushwaha

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  1. Aniket Puri
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Correspondence to Sudhir S Kushwaha.

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A Puri declared he has no competing interests.

MD McGoon is a consultant for Medtronic, Actelion, CoTherix, Myogen and United Therapeutics. He has received research or grant support from Medtronic, CoTherix and Myogen.

SS Kushwaha was an investigator for Encysive for the Sitaxentan studies.

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Puri, A., McGoon, M. & Kushwaha, S. Pulmonary arterial hypertension: current therapeutic strategies. Nat Rev Cardiol 4, 319–329 (2007). https://doi.org/10.1038/ncpcardio0890

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