Abstract
Pulmonary arterial hypertension (PAH) is a severe and debilitating disease characterised by vascular proliferation and remodelling of the small pulmonary arteries, leading to a progressive increase in pulmonary vascular resistance, increased afterload on the right ventricle and, ultimately, right heart failure. Although there is no “cure” for PAH, the availability of targeted therapies over the past decade has led to major advances in the management of PAH, reflected in improvements in survival in the modern treatment era. However, despite this, disease progression is inevitable in the majority of patients with PAH and overall the long-term prognosis, although improved, remains poor. There is a clear and urgent need for new therapeutic options, either through the development of improved drugs that act on targets established by existing PAH-specific therapies, or of agents targeting novel pathogenic pathways not addressed by currently available therapies. A number of such new agents that have shown promise in experimental models and preliminary human studies are discussed in this article.
- Endothelin receptor antagonist
- nitric oxide
- prostacyclin
- pulmonary arterial hypertension
- serotonin receptor antagonist
- tyrosine kinase inhibitor
Footnotes
Provenance
Publication of this peer-reviewed article was supported by Actelion Pharmaceuticals Ltd, Switzerland (principal sponsor, European Respiratory Review issue 122).
Statement of Interest
V.V. McLaughlin has received speaking and/or consulting fees from Actelion, Bayer, Gilead, and United Therapeutics. The University of Michigan (Ann Arbor, MI, USA) has received research funding for multicentre trials from Actelion, Novartis, Pfizer and United Therapeutics.
- Received August 24, 2011.
- Accepted September 8, 2011.
- ©ERS 2011